Neurology Flashcards

1
Q

Define Alzheimer’s Disease

A

Alzheimer’s disease is a chronic, neurodegenerative disorder characterized by the progressive accumulation of abnormal protein deposits, primarily amyloid plaques and tau tangles, in the brain.

This leads to the deterioration of cognitive function, memory loss, and various behavioural and psychological symptoms.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Define Dementia

Define MCI

A
  • A state characterised by impairment of 2 or more cognitive domains
  • presenting for more than 6 months
  • severe enough to impact on function
  • That is not primarily attributable to underlying condition

MCI: impairment of 1 or more cognitive domain that is not severe enough to impact function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the Epidemiology of Alzheimer’s Disease?

A

Increasing prevalence with age
Women more commonly affected than men
APOE Gene

Alzheimer’s is the most common cause of dementia (>50%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the pathophysiology of Alzheimer’s Disease?

A

Amyloid Plaques: The accumulation of beta-amyloid protein fragments outside nerve cells in the form of plaques is a hallmark feature. These abnormal protein deposits are believed to disrupt neuronal communication, trigger inflammation, and ultimately lead to cell death.

Tau Tangles: Inside nerve cells, abnormal tau protein accumulates, forming neurofibrillary tangles.

Neuronal Loss and Brain Atrophy: As the disease progresses, significant neuronal loss occurs, particularly in brain regions responsible for memory and cognitive function, such as the hippocampus and the cerebral cortex.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Give some risk factors for Alzheimer’s Disease

A

Age: Advanced age is the most significant risk factor >65yrs

Genetic Predisposition: apolipoprotein E (APOE) gene, Down’s syndrome

Family History: Having a first-degree relative with Alzheimer’s disease

Cardiovascular Risk Factors: Conditions like hypertension, diabetes, obesity, and hypercholesterolemia

Lifestyle Factors: Physical inactivity, smoking, and a diet high in saturated fats may contribute to increased risk.

Traumatic Brain Injury: A history of head injuries, particularly repeated concussions, has been linked to a higher risk of developing Alzheimer’s disease.

Low Educational Attainment: Lower levels of education may be associated with an increased risk.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are some clinical features of Alzheimer’s Disease?

A

Cognitive impairment

  • memory loss
    • generally affects recent events more than distant memories
    • difficulty learning new information
    • the person may defer to family members when answering questions,
    • vague with dates
  • problems with reasoning and communication
  • difficulty in making decisions/executive function
  • nominal dysphasia

Behavioural and Psychological Symptoms of Dementia (BPSD):

  • Aggitation
  • Depression
  • Psychosis
  • Apathy
  • Disinhibition
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Give some examples of behavioural changes you may see in Alzheimer’s Disease?

A

Agitation
Aggression
Apathy
Mood swings
Irritability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Give some examples of Psychological symptoms you may see in Alzheimer’s Disease?

A

Hallucinations
Delusions
Paranoia (in later stages)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are some differential Diagnoses for Alzheimer’s Disease?

A

Vascular Dementia
Lewy Body Dementia
Frontotemporal Dementia
Mild Cognitive Impairment (MCI)
Normal Age related Decline

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the first line investigations for Alzheimer’s Disease?

A

History - Cognitive decline questionnaire (MMSE)
Examination - Neurological Exam
Blood tests - Confusion Screen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the lab tests involved in a confusion screen?

A
  • FBC (e.g. infection, anaemia, malignancy)
  • U&Es (e.g. hyponatraemia, hypernatraemia)
  • LFTs (e.g. liver failure with secondary encephalopathy)
  • Coagulation/INR (e.g. intracranial bleeding)
  • TFTs (e.g. hypothyroidism)
    Calcium (e.g. hypercalcaemia)
  • B12 + folate/haematinics (e.g. B12/folate deficiency)
  • Glucose (e.g. hypoglycaemia/hyperglycaemia)
  • Blood cultures (e.g. sepsis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the diagnostic investigations for Alzheimer’s Disease?

A

Once reversible courses of confusion are eliminated and dementia is still suspected:

Brain imaging - MRI or PET scan
CSF Analysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the management of Alzheimer’s Disease?

A

Non-pharmacological:

  • Psychological Interventions
  • Cognitive stimulation therapy

Pharmacological:

  • Acetylcholinesterase inhibitors (Donepezil or Rivastigmine)
  • NDMA antagonists (memantine)
  • If they have BPSD - low dose anti-psychotic (risperidone)

There is no curative treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Define Parkinson’s Disease?

A

Parkinson’s disease is a chronic, progressive, degenerative neurological condition

There is a progressive reduction in dopamine in the basal ganglia which leads to disordered control of bodily movements.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the epidemiology of Parkinson’s disease?

A

The second most common neurodegenerative disorder after Alzheimer’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the aetiology of Parkinson’s disease?

A

Unknown however the pathophysiology is well established and correlated to lewy body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are some risk factors for developing Parkinson’s Disease?

A

Male (twice as common)

Increasing age (>65 years)

Family History - though it isn’t a genetic condition this can increase risk

Previous Head injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are some protective factors for Parkinson’s disease?

A

Smoking
Caffeine intake
Physical activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the characteristic triad of Parkinson’s disease?

A

Resting tremor
Rigidity (resisting passive movement)
Bradykinesia (slowness of movement)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the clinical features of Parkinson’s disease?

A

Motor Features:

  • Asymmetrical Pin-rolling Tremor - worse on one side
  • Rigidity - “Cogwheel Rigidity, Lead pipe arm”
  • Bradykinesia - Shuffling gait, Micrographia, Poverty of movement

Other Features:

  • REM Sleep Behaviour Disturbance and insomnia
  • Anosmia (loss of sense of smell)
  • Autonomic Dysfunction - Postural hypotension, constipation and ED.
  • Psychiatric features - Depression, Hallucinations, Anxiety
  • Postural instability (a Late feature of Parkinson’s)
  • Hypomimia (lack of facial expression)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are some different presentations of Bradykinesia in Parkinson’s disease?

A
  • Handwriting gets smaller and smaller (micrographia)
  • Small steps when walking (“shuffling” gait)
  • Rapid frequency of steps to compensate for the small steps and avoid falling (“festinating” gait)
  • Difficulty initiating movement (e.g., going from standing still to walking)
  • Difficulty in turning around when standing and having to take lots of little steps to turn
  • Reduced facial movements and facial expressions (hypomimia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Symmetry, Hertz, At Rest, Movement, Additional features, Alcohol

What are the features of the Parkinson’s Tremor?

A

Asymmetrical
4-6 Hertz
Worse at rest
Improves with intentional movement
Additional Features Present
No change with Alcohol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Symmetry, Hertz, At Rest, Movement, Additional features, Alcohol

What are the features of a Benign Essential Tremor?

A

Symmetrical
6-12 Hertz
Improves at rest
Worse with intentional movement
Additional Features Absent
Improves with Alcohol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are some Parkinson’s-Plus Syndromes?

What are the characteristic features?

A

Multiple System Atrophy

  • Presents with early autonomic dysfunction
  • Constipation, Postural hypotension, Sweating, Sexual dysfunction.
  • Postural instability compared to PD

Lewy Body Dementia

  • Early and prominent cognitive dysfunction before parkinsonism traits.
  • Visual Hallucinations

Progressive Supranuclear Palsy

  • Verticle Gaze Palsy
  • Postural instability

Corticobasal Degeneration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What are some differential Diagnoses for Parkinson's Disease?
* **Benign Essential Tremor** * **Multiple System Atrophy (MSA):** Very prominent autonomic dysfunction, early postural instability, poor response to levodopa * **Dementia with Lewy Bodies (DLB):** Early and prominent cognitive dysfunction, visual hallucinations, fluctuating cognition * **Progressive Supranuclear Palsy (PSP):** Early gait instability and falls, vertical gaze palsy, prominent axial rigidity * **Corticobasilar Degeneration:** May have predominant apraxia, aphasia and 'alien hand' syndrome * **Normal Pressure Hydrocephalus (NPH):** Presents with Magnetic gait, urinary incontinence and memory problems * **Wilson's Disease:** May be associated with signs of liver disease * **Dementia Pugilistica:** Secondary to repeated head trauma
26
What are some side effects of Levodopa?
LEVODOPA: **L**unacy - psychosis, hallucinations **E**nd dose weaning in**V**oluntary movements - dyskinesia, Chorea, Athetosis **O**rthostatic hypotension **D**ry mouth/drooling **O**n off phenomenon **P**alpitations **A**norexia
27
Give an example of a Catechol-o-methyltransferase (COMT) inhibitor?
Entacapone Tolcapone
28
Give an example of a Monoamine Oxidase B (MOA) inhibitor?
Selegiline Rasagiline
29
What are the investigations for Parkinson's Disease?
Parkinson's disease is **primarily a clinical diagnosis, supported by positive response to treatment** trials. An absolute failure to respond to 1-1.5g of levodopa daily almost excludes a diagnosis of idiopathic Parkinson's disease. Other investigations such as MRI Head or a Dopamine Transporter Scan (**DaT scan**) can be considered in atypical cases. **The NICE guidelines recommend that the diagnosis is made using the UK Parkinson's Disease Society Brain Bank Clinical Diagnostic Criteria**
30
What is the management of Parkinsons? Motor Sx Persistent Sx Non-responsive
**Motor Symptoms significantly affecting QoL** * First line: Levodopa (often in combination with decarboxylase inhibitors is first line) * Dopamine agonists tend to be added later down the line for most people. **Motor Symptoms not affecting QoL** * Dopamine Agonists (**non-ergot derived**) * Levodopa (+decarboxylase inhibitors) * Monoamine Oxidase Inhibitors **Patients have symptoms despite optimal Levodopa Treatment then use adjuncts:** * Dopamine Agonists * MOA inhibitors * COMT inhibitors * Amantadine (when motor symptoms persist and adjuncts haven't helped) **If people have fluctuating symptoms but tend to respond well to medications then you can use Deep Brain Stimulation to improve the length of time the medications work for**
31
Give Some examples of Dopamine Agonists and a key side effect?
**Non-Ergo:** * Rotigotine * Ropinirole * Apomorphine Non-ergo DAs are used in parkinsons disease **Ergot:** * Bromocriptine * Pergolide * Cabergoline **Side effect of Pulmonary Fibrosis in prolonged use of Ergot dopamine agonists hence no the main ones used are non-ergot** All can cause hallucinations and impulse disorders (Gambling)
32
Define Essential Tremor?
Benign essential tremor is a relatively common condition chronic neurological condition associated with older age. **Autosomal Dominant Inheritance in > 50% of cases** It is characterised by a fine tremor affecting all the voluntary muscles. It is most notable in the hands but can affect other areas, for example, causing a head tremor, jaw tremor and vocal tremor.
33
What is the Epidemiology of Essential Tremor?
ET is one of the most prevalent movement disorders, with the incidence increasing with age. The condition may manifest at any age, from childhood to adulthood. The age of onset tends to be earlier in those with a positive family history.
34
What is the Aetiology of Essential Tremor?
Aetiology is complex and not fully understood Multifactorial - Genetic and Environmental Factors 50% of cases - ET is inherited via an **Autosomal Dominant Trait**
35
What are some medications combined with levodopa? What are some medications for the autonomic features of Parkinson's?
Levodopa is always combined with Dopa-Decarboxylase inhibitors to prevent peripheral metabolism of L-Dopa **Levodopa** (combined with Decarboxylase inhibitors **Carbidopa / Benserazide**) * **Co-beneldopa** (levodopa and benserazide), with the trade name **Madopa** * **Co-careldopa** (levodopa and carbidopa), with the trade name **Sinemet** **Specific Autonomic Symptoms** * REM Sleep disorder: **Modafinil** * Orthostatic Hypotension: **Midodrine** * Drooling: **Glycopyrronium bromide** * Nausea: **Domperidone**
36
What are the clinical Features of Essential Tremor?
A postural or kinetic tremor, which predominantly affects the upper limbs distally. Additional symptoms may include: * Involvement of the head, lower limbs, voice, tongue, face, and the trunk, although less common. * **Increased tremor amplitude** over time, causing difficulty with tasks such as writing, eating, holding objects, dressing, and speaking. * Exacerbation of tremor during situations of anxiety, stress, and social interaction. * Potential development of severe psychosocial disability, including depression, particularly in patients with head and voice tremors. **Improved with Alcohol Consumption**
37
What are some differential diagnoses for Essential Tremor?
* **Parkinson's disease:** Resting tremor, bradykinesia, rigidity, postural instability. * **Hyperthyroidism-associated tremor:** Palpitations, heat intolerance, weight loss, anxiety. * **Dystonic tremor:** Abnormal posturing, muscle contractions, worsened by voluntary movement.
38
What are the investigations for Essential Tremor?
**Mainly a Clinical Diagnosis** Further investigations, such as blood tests or neuroimaging, may be warranted to rule out other potential causes of tremor.
39
What is the Management of Essential Tremor?
No definitive treatment for Essential Tremor Medications that may improve symptoms are: * **Propranolol** is first line * Primidone (a barbiturate anti-epileptic medication) * Topiramate
40
Define Motor Neurone Disease?
Motor neurone disease is a term that encompasses a variety of specific diseases affecting the motor nerves. Motor neurone disease is a progressive, eventually fatal condition where the motor neurones stop functioning. There is no effect on the sensory neurones. Sensory symptoms suggest an alternate diagnosis.
41
What is the epidemiology of MND?
2:1 Male predominance Mean age of Onset 50-60 yrs **90%** of cases are Sporadic with only 10% familial **Notable overlap with Frontotemporal Dementia**
42
What are some potential genetics of MND?
Associated with the misfolding of the **TDP-43 Protein** 2% of cases associated with a mutation in the **SOD-1 gene**
43
What are some risk factors for MND?
Increased age >60 yrs Male FHx Smoking RUGBY
44
What is the most common type of Motor neurone Disease?
Amyotrophic Lateral Sclerosis (ALS) **Accounts for 50% of cases**
45
What are the different types of MND?
Amyotrophic Lateral Sclerosis (ALS): UMN and LMN signs Progressive Muscular Atrophy (PMA): LMN signs only **(best prognosis)** Primary Lateral Sclerosis (PLS): UMN signs only Progressive Bulbar Palsy (PBP): CN9-12 affected. Speech and swallowing issues **(worst prognosis)**
46
What is the Pathophysiology of MND?
Degenerative condition selectively affecting motor neurons (cortical and bulbar tracts) mainly in the anterior horn cells, motor cortex or cranial nerve nuclei There is relentless and UNEXPLAINED destruction of UMN and anterior horn cells in the brain and spinal cord **Causes both UMN and LMN dysfunction** * UMN and LMN affected but no sensory or sphincter loss – distinguishes from MS * Never affects eye movements – distinguishable from myasthenia gravis
47
What are the signs of Upper Motor neuron lesions?
Hypertonia Rigidity + spasticity Hyperreflexia Babinski Reflex Positive - Big toe goes up when stroking foot Power: Arms - Flexors > Extensors Legs - Flexors < Extensors
48
What are the signs of Lower Motor neuron lesions?
Hypotonia Flaccidity + muscle wasting Hyporeflexia Fasciculations Babinski Reflex Negative - big toe goes down when stroking foot Generally loss of power
49
What is not affected in MND?
Eye muscles and Sphincters **generally spared** (affected in MS and Myasthenia Gravis) Sensory function (affected in MS and polyneuropathies)
50
Give a characteristic presentation of MND?
The typical patient is a late middle-aged (e.g., 60) man, possibly with an affected relative. There is an insidious, progressive weakness of the muscles throughout the body, affecting the limbs, trunk, face and speech. The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).
51
What are the clinical signs of MND?
* **Fasciculations** * Asymmetric limb weakness is common in ALS * Mixed UMN and LMN signs * Dysarthria / Dysphagia * **Split Hand Sign** - Disproportionate wasting of thenar muscles compared to hypothenar muscles.
52
What are some differential Diagnosis of MND?
* Multiple Sclerosis * Myasthenia Gravis * Thyrotoxicosis * Brainstem lesions (TIA/Stroke) * Cervical Spondylopathy * Paraproteinaemias
53
How is MND Diagnosed? What are some observed findings?
**The diagnosis needs to be made very carefully. It is based on the clinical presentation after excluding other conditions. It should only be made by a specialist when there is certainty. The diagnosis is often delayed, causing stress.** * **LMN/UMN signs in 3 regions** * **Nerve conduction studies** will show normal motor conduction and can help exclude a neuropathy. * **Electromyography** shows a reduced number of action potentials with increased amplitude. * **MRI** is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy
54
What are some investigations to rule out reversable differential diagnoses of MND?
TFTs: Rule out Thyrotoxicosis Protein Electrophoresis: Rule out Paraproteinemia's MRI brain and spinal cord: Assess for brainstem lesions or Cervical Spondyloarthropathy EMG and Nerve conduction studies: Rule out Myasthenia gravis or multifocal mononeuropathy.
55
What is the management of MND? Symptom control?
**No effective treatments for halting or preventing progression of MND** **Riluzole** can slow progression by a couple of months **NIV (non-invasive ventilation)** BIPAP can be used to support breathing **PEG** is the preferred way to support nutrition **Symptom Control:** * MDT input * **Baclofen** for muscle spasticity * Benzodiazepines for breathlessness due to anxiety
56
What is the prognosis of MND What are some management considerations to make due to the prognosis?
* PMA has the **best prognosis** * PBP has the **worst prognosis** * Average prognosis is 2-3 years after diagnosis **Considerations** * Breaking bad news effectively and supportively * Multidisciplinary team (MDT) input to support and maintain their quality of life * Symptom control (e.g., baclofen for muscle spasticity and antimuscarinic medical for excessive saliva) * Benzodiazepines may help breathlessness worsened by anxiety * **Advanced directives** to document their wishes as the disease progresses * **End-of-life care**
57
Define Multiple Sclerosis (MS)
Multiple sclerosis (MS) is a chronic and progressive autoimmune condition involving demyelination in the central nervous system. The immune system attacks the myelin sheath of the myelinated neurones.
58
What is the pathophysiology of MS?
**Multiple sclerosis affects the central nervous system (the oligodendrocytes)** * **Type IV Hypersensitivity Rxn** * T-cell mediated – T cells activate B cells to produce auto-antibodies against Basic myelin protein of oligodendrocytes * The Abs will bind to the basic myelin protein and target oligodendrocytes for destruction by macrophages * T Lymphocytes manage to cross the BBB, they can cause a cascade of destruction to the neuronal cells (oligodendrocytes) in the brain by recruiting other immune cells * This results in plaques of demyelination and inflammation and therefore conduction disruption along axons * In early disease, the myelin sheath can regenerate and symptoms somewhat resolve, however the new myelin is less efficient and temperature dependent * Therefore Symptoms are exacerbated by heat
59
What are some causes of MS?
The cause of the multiple sclerosis is unclear, but there is growing evidence that it may be influenced by: Multiple genes Epstein–Barr virus (EBV) Low vitamin D Smoking Obesity
60
What is the Epidemiology of MS?
* **3** times more common in women * most commonly diagnosed in people **aged 20-40 years** * much more common at higher latitudes (5 times more common than in **tropics**)
61
What is characteristic about the symptom onset in MS?
* Symptoms usually progress over more than 24 hours. * 75% have significant Lethargy * Symptoms tend to last days to weeks at the first presentation and then improve. * Later in the disease the symptoms become more permanent **There are many ways MS can present, depending on the location of the lesions.**
62
What is the most common presentation of MS?
**Optic Neuritis: demyelination of the optic nerve and presents with unilateral reduced vision, developing over hours to days** Key features include: * Central scotoma (an enlarged central blind spot) * Pain with eye movement * Impaired colour vision (Red Desaturation) * Relative afferent pupillary defect
63
What are some other causes of Optic Neuritis?
Sarcoidosis Systemic lupus erythematosus Syphilis Measles or mumps **Neuromyelitis optica** Lyme disease
64
What are the clinical manifestations of MS? Visual Sensory Motor Cerebellar Other
**Visual** * optic neuritis: common presenting feature * optic atrophy * **Uhthoff's phenomenon:** worsening of vision following rise in body temperature * internuclear ophthalmoplegia **Sensory** * pins/needles * numbness * trigeminal neuralgia **Lhermitte's syndrome:** paraesthesiae in limbs on neck flexion **Motor** * spastic weakness: most commonly seen in the legs **Cerebellar** * ataxia: more often seen during an acute relapse than as a presenting symptom tremor **Others** * urinary incontinence * sexual dysfunction * intellectual deterioration
65
What is Uhthoff's Phenomenon?
A **worsening of neurological symptoms related to a demyelinating disorder such as multiple sclerosis when the body overheated** in hot weather, exercise, fever, saunas, or hot tubs
66
What are the classifications of MS?
Multiple sclerosis may be divided into two groups: **Relapsing-remitting** (which may become secondarily progressive) **Primary progressive** * Relapsing remitting MS makes up 80% of disease at presentation, compared with primary progressive which is <10%. * The remaining 10% fall into a difficult to classify intermediate group of progressive-relapsing disease.
67
What investigations are done in MS? 2 main tests? Other?
**Clinical History and Examination** **MRI Scan** * high signal T2 lesions * periventricular plaques * **Dawson fingers:** often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum **Lumbar Puncture** * **IgG Oligoclonal Bands in CSF** **Visual Evoked Potentials** * Delayed but well preserved waveform
68
What diagnostic Criteria is used in MS?
McDonald Criteria: * 2 or more attacks + 1 Objective clinical Lesion OR * 1 attack + 2 Objective clinical Lesions Objective clinical lesions must be **disseminated in time and space demonstrated by MRI or positive CSF findings** For the purposes of the McDonald's criteria, there must be lesions in two of the **four regions** of the central nervous system which satisfy dissemination in space; **juxtacortical, subcortical, infratentorial and spinal cord.**
69
What is the Acute management for MS?
**Glucocorticoids** * 500mg orally daily for 5 days * 1g Methylprednisolone intravenously daily for 3–5 days (where oral treatment has previously failed or where relapses are severe)
70
What is the Chronic management of MS? Additional Management?
Disease Modifying therapies: **Biologics:** * **Natalizumab** is first line * Ocrelizumab, Alemtuzumab **Symptomatic Control:** * Exercise to maintain activity and strength * Fatigue may be managed with amantadine, modafinil or SSRIs * Neuropathic pain may be managed with medication (e.g., amitriptyline or gabapentin) * Depression may be managed with antidepressants, such as SSRIs * Urge incontinence may be managed with antimuscarinic medications (e.g., solifenacin) * Spasticity may be managed with baclofen or gabapentin * Oscillopsia may be managed with gabapentin or memantine
71
What may indicate a worse prognosis in MS?
Older age of Onset Male Gender Primary Progressive MS High relapse rate Smoking Comorbidities
72
Define Muscular Dystrophy
Muscular dystrophy refers to a group of inherited genetic disorders characterized by the progressive degeneration and weakening of the body's muscles. The disease is categorized into various types, the most common being Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy, distinguished primarily by the severity and onset age.
73
What is the Epidemiology of Muscular Dystrophy?
* The most common form of muscular dystrophy is Duchenne muscular dystrophy (**1 in 3,500-6,000 male births**) * Becker muscular dystrophy is less common, (**1 in 18,000-30,000 male births**) **These conditions are X-linked recessive disorders, males are predominantly affected while females are usually carriers.**
74
What is the aetiology of Muscular Dystrophy?
Mutations in the dystrophin gene, leading to reduced expression of dystrophin, a crucial protein involved in muscle contraction and stability. In Duchenne's, the protein is virtually absent In Becker's, the protein is expressed at lower levels or the protein is dysfunctional.
75
What are the clinical features of Duchenne's Muscular Dystrophy?
* Presents in early childhood with muscle wasting and weakness * Children usually become wheelchair-bound before puberty and often succumb to respiratory failure by their early twenties * May present with hypertrophic calves, as degenerated muscle is replaced by fat * Notable signs include a positive Gower's manoeuvre and difficulty in lifting the child due to proximal muscle weakness
76
What are the clinical features of Becker's Muscular Dystrophy?
* Presents later in childhood with muscle wasting and weakness * Patients commonly become wheelchair-bound in their teens and can survive into their thirties
77
What are some differential Diagnoses for Muscular Dystrophy?
Limb-Girdle Muscular Dystrophy: Characterized by weakness and wasting of the proximal muscles, specifically around the hips and shoulders. (**autosomal inheritance**) Spinal Muscular Atrophy Myopathies
78
What are the investigations for Muscular Dystrophy?
* Creatine Kinase Measurement - **First line screening as levels are significantly raised in muscular dystrophy** * **Genetic Testing is Gold standard for diagnosis** * Muscle Biopsy
79
What is the management of Muscular Dystrophy?
MDT input to maximise quality of life and minimise disease progression. * Glucocorticoids slow muscle degeneration * Physical Therapy to maintain mobility * Genetic Counselling
80
What is the prognosis of Muscular Dystophy?
* Patients with Duchenne's muscular dystrophy typically survive into their early twenties * Patients with Becker's muscular dystrophy can live into their thirties. * **The leading causes of mortality are respiratory complications and dilated cardiomyopathy.**
81
Define Huntington's Chorea?
Huntington’s disease (also called Huntington’s chorea) is an autosomal dominant genetic condition that causes progressive neurological dysfunction.
82
What is the epidemiology of Huntington's disease?
It is one of the most common hereditary neurodegenerative disorders 1 in 10,000-20,000
83
What are the Genetics of Huntington's disease?
It is a trinucleotide repeat disorder involving a genetic mutation in the **HTT gene on chromosome 4**, which codes for the huntingtin (HTT) protein. CAG > 38 repeats
84
Give some examples of other Trinucleotide repeat disorders?
Fragile X syndrome Spinocerebellar ataxia Myotonic dystrophy Friedrich ataxia
85
What is Anticipation in genetics?
Anticipation is a feature of trinucleotide repeat disorders, where successive generations have more repeats in the gene, resulting in: **Earlier age of onset Increased severity of disease**
86
What is the pathophysiology of Huntington's disease?
Faulty Huntingtin protein builds up in the striatum causing cell death and loss of cholinergic and GABA-nergic neurons 🡪 decreased ACH and GABA synthesis in striatum Less GABA causes less regulation of dopamine to striatum causing increased dopamine levels resulting in excessive thalamic stimulation and subsequently increased movement (chorea)
87
What is the presentation of Huntington's disease?
**Features typical develop after 35 years of age** * chorea * personality changes (e.g. irritability, apathy, depression) and intellectual impairment * dystonia * saccadic eye movements * Dysarthria/Dysphagia
88
What are the movement disorders associated with Huntington's disease?
**Chorea** (involuntary, random, irregular and abnormal body movements) **Dystonia** (abnormal muscle tone, leading to abnormal postures) **Rigidity** (increased resistance to the passive movement of a joint) **Eye movement disorders** **Dysarthria** (speech difficulties) **Dysphagia** (swallowing difficulties)
89
What are some differential Diagnosis for Huntington's disease?
**Parkinson's disease:** Characterized by bradykinesia, resting tremor, rigidity, and postural instability **Wilson's disease:** Presents with liver disease, Kayser-Fleischer rings in the eye, and neurological symptoms such as dystonia, tremor, and dysarthria **Huntington's disease-like disorders (HDL1, HDL2, HDL3, and HDL4):** Present with a similar clinical picture but have different genetic backgrounds **Neuroacanthocytosis syndromes:** Characterized by movement disorders and spiculated red blood cells (acanthocytes)
90
What are the investigations for Huntington's disease?
**Neuroimaging**: MRI and CT scans may show loss of striatal volume and an enlarged frontal horn of the lateral ventricles in severe disease stages. **Genetic Testing**: confirmatory and allows for predictive testing in at risk family members
91
What is the management of Huntington's disease?
**No curative or preventative treatments** * **Breaking bad news** effectively and supportively * **Genetic counselling** regarding relatives, pregnancy and children * Multidisciplinary team (MDT) input to support and maintain their quality of life * **Physiotherapy** to improve mobility, maintain joint function and prevent contractures * **Speech and language therapy** where there are speech and swallowing difficulties * **Tetrabenazine** may be used for chorea symptoms * Antidepressants (e.g., SSRIs) for depression * Advanced directives to document their wishes as the disease progresses * End-of-life care
92
What is the prognosis for a patient with Huntington's disease? What are the common complications?
The prognosis for Huntington's disease is poor, with an invariable decline in physical and cognitive abilities. Death usually occurs due to complications: **Aspiration pneumonia** **suicide is the second most common cause of death**
93
Define a Brain Abscess?
A brain abscess is a pus-filled swelling in the brain. It usually occurs when bacteria or fungi enter the brain tissue after an infection or severe head injury.
94
Causes of Brain Abscesses?
* Extension of sepsis from middle ear or sinuses, * Trauma or surgery to the scalp * Penetrating head injuries * Embolic events from endocarditis
95
What are the clinical features of Brain Abscesses?
The presenting symptoms will depend upon the site of the abscess (those in critical areas e.g. motor cortex) will present earlier. Abscesses have a considerable mass effect in the brain and raised intracranial pressure is common. * headache - often dull, persistent * fever - may be absent and usually not the swinging pyrexia seen with abscesses at other sites * focal neurology - e.g. oculomotor nerve palsy or abducens nerve palsy secondary to raised intracranial pressure **Other features consistent with raised intracranial pressure** * Nausea * Papilloedema * Seizures
96
What are the investigations for a Brain Abscess?
* FBC * ESR/CRP * **Neuroimagining:** MRI may show a ring enhancing lesion with surrounding oedema
97
What is the management for a Brain Abscess?
**Surgery:** a craniotomy is performed and the abscess cavity debrided **IV antibiotics:** IV 3rd-generation cephalosporin + metronidazole **Intracranial pressure management:** e.g. dexamethasone
98
Define Meningitis?
Inflammation of the meninges from both infective and non-infective causes. This is a notifiable condition to PHE
99
What are the different infective causes of Meningitis?
Viral: **Enterovirus (coxsackie) HSV2 VZV** Bacterial: **N. Meningitidis S. pneumonia** Fungal: Cryptococcus Neoformans (primary in the immunosuppressed population) Parasitic: Amoeba, Toxoplasma Gondii
100
What is the most common cause of meningitis in children and adults?
**Neisseria meningitidis Streptococcus pneumonia**
101
What is the most common cause of meningitis in neonates?
Group B Streptococcus - **Streptococcus Agalactiae** E.coli Strep. pneumonia Listeria
102
What are some non-infective causes of Meningitis?
* Malignancies such as leukemia, lymphoma, and other tumors * Chemical meningitis * Certain drugs, including NSAIDs and trimethoprim * Systemic inflammatory diseases such as sarcoidosis, * Systemic Lupus Erythematosus, Behcet's disease.
103
What is the most common cause of meningitis?
Viral infection (Most commonly **enteroviruses**) More common but less severe than bacterial causes.
104
What are the main risk factors for meningitis?
Extremes of age (Infant/elderly) Immunocompromised Pregnancy Travel Crowded environment - barracks/uni Non-vaccinated
105
What vaccines are available for meningitis coverage?
N. Meningitidis - Men B + Men C + Men ACWY S. pneumoniae - PCV Vaccine
106
What are the symptoms of Meningitis?
Meningism: Headache, Fever, Neck stiffness Non-Blanching Purpuric Rash Nausea + Vomiting Seizures Photophobia Purpuric Rash - Bacterial Meningitis Non-Blanching Purpuric Rash - Meningococcal Septicaemia
107
What does the Non-blanching Purpuric Rash indicate in Meningitis?
Bacterial Meningococcal Septicaemia: This rash indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages.
108
What is the presentation of Meningitis in Neonates?
Neonates and babies can present with very non-specific signs and symptoms: * Hypotonia * Poor feeding * Lethargy * Hypothermia * **Bulging Fontanelle.**
109
What are the clinical signs of meningitis?
**Kernig's Sign:** When the hip is flexed and the knee is at 90°, extension of the knee results in pain **Brudzinski Sign:** Severe neck stiffness causes the hips and knees to flex when the neck is flexed
110
What are the primary investigations in meningitis?
**1st Line: Bloods** FBC - raised WCC CRP - raised Blood glucose - compared with CSF Blood culture - to determine viral/bacterial CT Head - Look for Brain Lesions/Abscesses/CIs for LP **Lumbar Puncture (LP) + CSF Analysis (Gold Standard)**
111
What are some contraindications for a lumbar puncture?
Raised ICP GCS <9 Focal Neurological signs
112
In Paediatric cases of fever and general unwellness what does NICE recommend as an important investigation?
A Lumber Puncture in all children: * Under 1 month presenting with fever * 1 to 3 months with fever and are unwell * Under 1 year with unexplained fever and other features of serious illness
113
Where is a lumbar puncture usually taken from?
Between L3/L4 Since spinal cord ends L1/2
114
What are some differential diagnoses for Meningitis?
* Encephalitis * Subarachnoid Haemorrhage * Brain Abscess * Sinusitis * Migraine
115
What would the results of CSF analysis be in Bacterial, Viral and Fungal Meningitis?
Fungal: * Lymphocytosis * Increased Protein Concentration * Decreased Glucose Concentration
116
What is the management of Bacterial Meningitis in the community?
STAT dose of **IM Benzylpenicillin** and immediate transfer to hospital In True Penicillin allergy then immediate transfer to hospital
117
What is the management of Bacterial Meningitis in the Hospital?
**Ideally perform a blood culture and a lumbar puncture prior to starting Abx unless the patient is acutely unwell.** Broad Spec Abx - Cover All Likely Organisms: 1st Line - **Ceftriaxone or Cefotaxime** (as they get through the BBB) WTIH OR AFTER **IV Dexamethasone** - Prevent neurological sequelae 2nd Line: Chloramphenicol Once Blood cultures have been done then can tailor Abx: Eg. IV Benzylpenicillin for N.Meningitidis
118
What antibiotics should be used to treat bacterial meningitis in children?
Under 3 Months: **Cefotaxime and Amoxicillin** (covers for listeria) Over 3 Months: **Ceftriaxone**
119
What Post Exposure Prophylaxis should be done in Meningitis cases?
Contact Tracing: * This risk is highest for people that have had **close prolonged contact within the 7 days prior to the onset of the illness.** * The risk decreases 7 days after exposure. Therefore, if no symptoms have developed 7 days after exposure they are unlikely to develop the illness. **A single dose of ciprofloxacin. It should be given as soon as possible and ideally within 24 hours of the initial diagnosis.**
120
What is the management of Viral Meningitis?
Usually only requires Supportive treatment **Acyclovir** can be used to treated suspected or confirmed HSV/VZV infections
121
What are some complications of Meningitis?
* **Hearing loss** is a key complication * **Seizures** and epilepsy * Cognitive impairment and learning disability * Memory loss * Cerebral palsy, with focal neurological deficits such as limb weakness or spasticity * Septic Shock and DIC * Coma and Death
122
Define Encephalitis?
Encephalitis is a pathological condition characterised by inflammation of the brain parenchyma, also known as the "encephalon".
123
What is the aetiology of Encephalitis?
* Predominantly Viral Infection * Bacterial and fungal pathogens can also lead to encephalitis (Rarely in the UK) * Autoimmune Encephalitis - NMDA receptor antibodies
124
What are the most common Viral causes of Encephalitis?
**Herpes Simplex Virus Type 1 (HSV-1)** Others: * HSV-2 * CMV * EBV * VZV * HIV
125
What are the clinical features of Encephalitis?
**Altered Mental Status** Fever Flu-like prodromal illness Seizures Acute onset Focal neurological deficits Headaches Behavioural changes
126
What investigations are done in Encephalitis?
**Encephalitis should be suspected in any patient presenting with sudden onset behavioural changes, new seizures, and unexplained acute headache** * A routine panel of blood tests * Blood cultures and viral PCR * **Lumbar Puncture + Cerebrospinal fluid (CSF) analysis with viral PCR** * Consideration for malaria blood films in case of exposure risk **CNS Imaging: CT/MRI**
127
What may be seen on LP and MRI in Encephalitis?
**LP** Lymphocytosis and raised protein. May have positive HSV-PCR **MRI** Temporal lobes affected Bilateral Multifocal Haemorrhage
128
What is the management of Encephalitis?
Intravenous antiviral medications are used to treat the suspected or confirmed underlying cause: **Aciclovir** treats herpes simplex virus (HSV) and varicella zoster virus (VZV) **Ganciclovir** treats cytomegalovirus (CMV) Supportive management of complications: Anti-convulsant for seizures
129
What are some side effects of Aciclovir?
**Common** * Generalised fatigue/malaise * Gastrointestinal disturbance * Photosensitivity and urticarial rash Others: * Acute renal failure * Haematological abnormalities * Hepatitis * Neurological reactions
130
What are some complications of Encephalitis?
**Lasting fatigue and prolonged recovery** Change in personality or mood Changes to memory and cognition Learning disability **Headaches** Chronic pain Movement disorders Sensory disturbance **Seizures** Hormonal imbalance
131
Define Guillain-Barre Syndrome?
Guillain-Barré syndrome is an acute immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni)
132
What is the pathophysiology of GBS?
Guillain-Barré is thought to occur due to a process called molecular mimicry. Antibodies against the pathogen aslo react to **gangliosides** (anti-ganglioside antibodies) which are components of cell membranes and myelin sheath. This leads to demyelination and a peripheral polyneuropathy
133
What are the clinical forms of GBS?
* **Acute inflammatory demyelinating polyneuropathy** * Acute motor axonal neuropathy * Acute Sensory and motor axonal neuropathy * **Miller-Fisher variant** characterised by ophthalmoplegia, ataxia, and areflexia
134
What is Miller-Fischer Syndrome?
Variant of Guillain-Barre syndrome associated with **ophthalmoplegia, areflexia and ataxia.** * The eye muscles are typically affected first * Usually presents as a **descending paralysis** rather than ascending * **Anti-GQ1b antibodies** are present in 90% of cases
135
What is the Aetiology of GBS?
Typically occurs **1-3 weeks following an infection** * Campylobacter jejuni * Cytomegalovirus * Epstein Barr Virus
136
What is the symptomatic progression of GBS?
* Gastroenteritis * 1-3 weeks later: Neurological Symptoms start * 2-4 weeks later: Symptoms Peak * Months to years: Recovery period
137
What are the clinical features of GBS?
Symptoms occuring 3-4 weeks post infection: * **Progressive ascending symmetrical limb weakness** * Lower back pain due to radiculopathty * Paraesthesia preceding motor symptoms * **hyporeflexia** * **Cranial Nerve Signs** - Ophthalmoplegia, facial nerve palsy. * **Autonomic Dysfunction** - Urinary retention, diarrhoea, Arrhythmias * **Potential respiratory distress in severe cases**
138
What are some differential Diagnoses for Guillain Barre Syndrome?
**Brainstem strokes** Polio Lyme Disease CMV HIV TB Transverse Myelitis Myasthenia Gravis Lambert Eaton Myasthenic Sydrome
139
What criteria is used to diagnose GBS?
Brighton Criteria
140
What are the investigations for GBS? First line Other?
**Nerve Conduction studies** * Reduced signal conduction velocity * Prolonged distal motor latency * Increased F wave latency **Lumbar Puncture + CSF analysis** * raised protein with normal cell count and glucose Other: * **Spirometry** * Monitoring of FVC for respiratory muscle involvement * Serological - **Anti-ganglioside antibodies**
141
What is the management of GBS?
Management for Severe cases: (eg inability to walk) * **IV Immunoglobulins are first line** * **Plasmapheresis is second line** Other: * Supportive care * VTE Prophylaxis - TEDS + LMWH * Monitoring FVC for respiratory failure * Analgesia - NSAIDS or Opiates for Radiculopathy pain * Manage complications
142
What is the leading cause of death in GBS?
**Pulmonary Embolism** hence the VTE prophylaxis
143
What is the prognosis of GBS?
Recovery can take months to years Most patients make a fully recovery **5% mortality due to respiratory or cardiovascular complications**
144
Define Bulbar Palsy?
A subtype of lower motor neurone lesion impacting the Glossopharyngeal, Vagus and Hypoglossal cranial nerves. This leads to impairments of speech and swallowing
145
What is the aetiology of Bulbar Palsy?
* Motor Neurone Disease - **primarily PBP** * Myasthenia Gravis * Guillain Barre Syndrome * Brainstem Stroke - **Lateral medullary Syndrome or Wallenberg's Syndrome** * Syringobulbia - Fluid-fillled cavity within the spinal cord.
146
What are the clinical features of Bulbar Palsy?
* Absent or normal Jaw Jerk Reflex * Absent **Gag Reflex** * Flaccid fasciculating tongue * Nasal speech (often quiet) * **Signs of the underlying cause**
147
What are some differential diagnoses for Bulbar palsy?
* **Pseudobulbar Palsy:** Upper motor neurone lesion of cranial nerves causing dysarthria and dysphagia * **Brainstem tumour** * **Multiple Sclerosis** * **Polymyositis and Dermatomyositis:** Muscle weakness of the pharyngeal muscles
148
What are the investigations for Bulbar Palsy?
**Aim at establishing underlying aetiology** * Neurological Examination - Cranial Nerve Exam * **EMG and Nerve Conduction Studies** - Help diagnose MG or MND * Blood tests and antibody screening * **MRI** to identify brainstem lesions or presence of a syrinx * **Lumbar puncture** to rule out infective or autoimmune causes
149
What is the management of Bulbar Palsy?
**Symptomatic relief and management of underlying cause** * Speech and swallowing therapy * Nutritional support due to Dysphagia and poor oral intake * Regular Monitoring to assess progression and manage complications * **Pharmacological management of underlying cause**
150
Define Cerebellar Syndrome?
A cluster of clinical manifestations resulting impaired function of the cerebellum.
151
# VIITAMIN C What are some causes of Cerebellar Dysfunction?
* **Vascular:** Stroke of posterior circulation * **Infectious:** Lyme disease, Cerebellar Abscess * **Inflammatory:** Multiple Sclerosis * **Traumatic:** Trauma to posterior fossa * **Alcohol** * **Metabolic:** Hypothyroidism * **Iatrogenic:** Phenytoin, Carbamazepine * **Neoplastic:** Primary and Secondary Tumours * **Congenital:** Friedrich's Ataxia, Spinocerebellar Ataxias (eg. SPG7)
152
# VANISHED What are the clinical Manifestations of Cerebellar Dysfunction?
**V**ertigninous Symptoms **A**taxia **N**ystagmus **I**ntention Tremor **S**lurred Staccato Speech (dysarthria) **H**ypotonia **E**xaggerated Broad Based Gait **D**ysdiadochokinesia
153
What symptoms arise from cerebellar vermis lesions?
Truncal Ataxia Gait instability **Few cerebellar signs in the limbs**
154
What symptoms arise from cerebellar hemisphere lesions?
VANISHED signs in **Ipsilateral Limb**
155
What are some differential diagnoses for Cerebellar Dysfunction?
* Stroke * Multiple Sclerosis * Posterior Fossa Tumour * Alcoholism * Medication side effects: **Phenytoin, Carbamazepine** * Tumours * Hereditary conditions
156
What are the investigations for Cerebellar Dysfunction?
**History:** Alcohol intake, Head injury, infection **Neuroimaging:** CT or MRI to identify stroke, tumour, trauma **Serological Tests:** Identify infectious or inflammatory causes **Lumbar Puncture:** Evidence of infection, inflammation or malignancy **Genetic Testing:** Diagnose hereditary conditions
157
What is the management of Cerebellar Dysfunction?
**Treat Underlying Cause** * Medications - manage symptoms * Surgery to remove tumours or address trauma * Rehabilitation therapies - physical, speech, occupational * Lifestyle modifications - Alcohol cessation
158
What is Herpes Zoster Ophthalmicus?
Herpes zoster ophthalmicus (HZO) describes the reactivation of the varicella zoster virus in the area supplied by the ophthalmic division of the trigeminal nerve. **It accounts for around 10% of case of shingles.**
159
What are the clinical features of Herpes Zoster Ophthalmicus?
* Vesicular rash around the eye, which may or may not involve the actual eye itself * **Hutchinson's sign:** rash on the tip or side of the nose. Indicates nasociliary involvement and is a strong risk factor for ocular involvement
160
What is the management of Herpes Zoster Ophthalmicus?
* **Oral antiviral treatment for 7-10 days** * Ideally started within 72 hours * Intravenous antivirals may be given for very severe infection or if the patient is immunocompromised * **Topical antiviral treatment is not given in HZO** * Topical corticosteroids may be used to treat any secondary inflammation of the eye * Ocular involvement requires urgent ophthalmology review
161
What are some complications of Herpes Zoster Ophthalmicus?
* **Ocular:** Conjunctivitis, Keratitis, Episcleritis, Uveitis * Ptosis * Post herpatic Neuralgia
162
What is Ramsay Hunt Syndrome?
**Herpes Zoster Oticus** is caused by the reactivation of the varicella zoster virus in the **geniculate ganglion of the Facial nerve**
163
What are the clinical features of Ramsay Hunt Syndrome?
* Auricular Pain - **often the first feature** * **Vesicular rash** around the ear canal, pinna and can extend to the anterior 2/3rds of the tongue and hard palate * **Facial Nerve Palsy** * **Vertigo and tinnitus**
164
What is a typical patient presenting with Ramsay Hunt Syndrome?
Patient with facial nerve palsy, auricular pain and a vesicular rash around their ear
165
What is the management of Ramsay Hunt Syndrome?
**Treatment should be initiated within 72 hours** Oral Aciclovir and Corticosteroids (prednisolone) May also require lubricating eye drops
166
Define Cerebral Palsy?
A **Permanent** disorder of movement and posture due to a **non-progressive lesion** of the motor pathways in the **developing brain**. It is not a progressive condition, however the nature of the symptoms and problems may change over time during growth and development. There is huge variation in the severity and type of symptoms, ranging from completely wheelchair bound and dependent on others for all activities of daily living, to para-Olympic athletes with only subtle problems with coordination or mobility.
167
What is the Epidemiology of Cerebral Palsy?
2/3 in 1000
168
What are some causes of Cerebral Palsy?
**Antenatal (80%):** * Maternal infections: Rubella, CMV, Toxoplasmosis * Trauma during pregnancy * Cerebral Malformation **Perinatal (10%):** * Birth asphyxia (**Hypoxic Ischemic Encephalopathy**) * Pre-term birth * Intrumental Delivery **Postnatal (10%):** * Meningitis * Intraventricular Haemorrhage * **Kernicterus** (Severe neonatal jaundice) * Head injury
169
What are the types of Cerebral Palsy?
**Spastic: hypertonia (90%)** (increased tone) and reduced function resulting from damage to upper motor neurones * Hemiplegic * Diplegic * Quadriplegic **Dyskinetic:** problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia. **Ataxic:** problems with coordinated movement resulting from damage to the cerebellum **Mixed:** a mix of spastic, dyskinetic and/or ataxic features **Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.**
170
What are some clinical features of Cerebral Palsy?
* **Motor Problems** * Abnormal tone early infancy * Delayed motor milestones * Abnormal gait - **Hemiplegic/Diplegic Gait** * Feeding difficulties.
171
What are some non-motor problems associated with Cerebral Palsy?
* Learning Difficulties (60%) * Epilepsy (30%) * Squints (30%) * Hearing Impairments (20%)
172
What are some differential diagnoses to Cerebral Palsy?
* Muscular Dystrophies * Metabolic Disorders * Hereditary spastic paraplegia * Juvenile idiopathic arthritis
173
What are some investigations performed in suspected Cerebral Palsy?
**Clinical Diagnosis often** * **Neuroimaging:** MRI to visualise the extent and nature of the brain lesions * **Genetic Testing:** considered for differential diagnoses when there is a suspicion of an underlying genetic disorder
174
What is the management of Cerebral Palsy?
**Requires a Multidisciplinary Team Approach** * **Paediatricians:** Optimise Medications: * Muscle Relaxants - **Baclofen** * Anti-epileptic Drugs * Glycopyrronium Bromide - For excessive drooling * **Surgery:** Musculoskeletal deformity correction / Tendon release * **Physiotherapy:** Stretch and strengthen muscles and maximise function * **Occupational Therapy:** Manage patients everyday lives and ADLs * **Speech and Language Therapy:** Aid with speech and swallowing problems as some patients may require an NG or PEG tube (requires dieticians)
175
What are some complications of Cerebral Palsy?
* Learning disability * Epilepsy * Kyphoscoliosis * Muscle contractures * Hearing and visual impairment * Gastro-oesophageal reflux
176
Define Hypoxic Ischaemic Encephalopathy (HIE)?
A form of brain damage that occurs due to antenatal or perinatal hypoxia
177
What is the epidemiology of HIE?
1-2 per 1000 Incidence is higher in premature and low birth weight infants
178
What are some causes of Hypoxic Ischaemic Encephalopathy?
**Pre-partum** * Placental Abruption * Maternal Shock **Intrapartum** * Intrapartum haemorrhage * Cord compression * Prolapsed cord * Nuchal Cord **Post Partum** * Prolonged Respiratory Arrest
179
What is the pathophysiology of Hypoxic Ischaemic Encephalopathy?
A lack of oxygen in the foetal circulation which leads to an insufficient oxygen supply to the brain This ischaemia culminates in irreversible brain damage both from primary neuronal death and secondary reperfusion injury
180
What are the clinical features of HIE?
Presentation varies along with the degree of neurological damage. **Mild:** Irritability, Slight changes in behaviour **Severe:** Hypotonia, Poor responsiveness to stimuli, Severe prolonged seizures
181
What are some differential diagnoses for HIE?
Meningitis Metabolic Disorders Intracranial Haemorrhage Drug withdrawal
182
What are the investigations performed in suspected HIE?
**EEG Monitoring:** Evaluate seizure activity and brain function **Multiple MRI Scans:** To assess extent of brain injury and areas affected
183
What is the management of HIE?
**Depends on presentation and systemic complications:** * **Respiratory Support** * **Anticonvulsant therapy:** To control Seizures * **Careful fluid balance:** To prevent further complications * **Cooling Therapy:** To induce mild hypothermia and prevent further damage from secondary reperfusion injury
184
Define Malaria?
Malaria is an infectious disease caused by members of the Plasmodium family of protozoan parasites. Protozoa are single-celled organisms. **The most severe and dangerous type is Plasmodium falciparum, which accounts for about 80% of malaria cases in the UK.**
185
How is Malaria spread?
Through bites from the **Female Anopheles Mosquitoes** that carry the disease
186
What are the different types of plasmodium that can cause Malaria?
* **Plasmodium falciparum** (the most common and severe form) * Plasmodium vivax * Plasmodium ovale * Plasmodium malariae * Plasmodium knowlesi
187
What is the lifecycle for Malaria?
1. Inoculation of parasites (**sporozoites**) passed from the blood to the liver 2. Asexual division in the liver maturing into **Schizonts** 3. Parasite emerges from the liver to infect red blood cells as mature **Merozoites** **P. vivax and P. ovale lay down Hypnozoites in the liver that remain dormant and are immune to conventional anti-malarial drugs and so can reactivate years later** 4. Infected RBCs rupture releasing loads of merozoites into the blood and cause **Haemolytic Anaemia**
188
How many hours do P. vivax, P. ovale and P. falciparum infect RBCs for before they rupture?
P. vivax and P. ovale - **48 hours cyclical fever spikes** P. falciparum - **More frequent and irregular fever spikes**
189
What are some protective factors for malaria?
Sickle Cell Disease G6PD Deficiency HLA-B53 Absence of Duffy Antigens
190
What are the clinical features of Malaria?
**Non-specific symptoms:** **Fever** (often cyclical every 48 hours) Fatigue Myalgia Headache Nausea and Vomiting **Signs:** Pallor due to haemolytic anaemia Hepatosplenomegaly Jaundice due to the rupture of RBCs
191
What is the gold standard investigation for Malaria?
**Malaria Blood Film:** * Thick - Sensitive to Malaria * Thin - Used to determine Species **3 Negative samples** taken over **3 consecutive days** are required to **exclude** malaria due to the cyclical release of parasites from the blood every 48-72 hours.
192
What is the management of Malaria?
Anti-malarials: Artesunate or Chloroquine * **Quinine plus Doxycycline** * **Quinine plus clindamycin** **There are increasing rates of resistance to chloroquine where Artesunate should be used** Artesunate is AVOIDED in pregnancy
193
What is the treatment for complicated malaria often due to P. falciparum infection?
* Admitted to HDU/ICU and monitored for complications * **Artesunate** is first line Quinine dihydrochloride may also be used
194
What are some complications of P. falciparum Malaria?
* Cerebral malaria: seizures, coma * Acute renal failure: blackwater fever, secondary to intravascular haemolysis, mechanism unknown * Acute respiratory distress syndrome (ARDS) * Hypoglycaemia * Disseminated intravascular coagulation (DIC)
195
What are the features of Cerebral Malaria?
Altered consciousness that can range from confusion to deep coma Seizures Neurological deficits **This is a medical emergency that requires urgent intervention**
196
What are the different Primary Brain Tumours?
* Gliomas (Astrocytoma, Ependymoma, Oligodendroma, Glioblastoma * Meningioma * Schwannoma * Pituitary adenoma
197
What is the most common Primary Brain Tumour in Adults and in Children?
Adults: Glioblastoma followed by Meningioma Children: Astrocytoma and Craniopharyngioma
198
What is the most common form of brain tumour?
Metastatic brain cancer
199
What are the cancers that will most commonly spread to the brain to cause metastatic brain cancer?
**Lung cancer (Most common)** Breast Bowel Skin (melanoma) Kidney
200
What are the clinical features of brain tumours?
**Wide range of symptoms depending on location and size** * **Headaches:** Often severe and constant that are worse in the mornings and when lying down * **Nausea and Vomiting** accompanying the headaches * **Seizures:** often the first sign of a brain tumour * **Cognitive changes:** Memory loss, confusion, difficulty concentrating * **Focal Neurological Deficits:** Motor and sensory symptoms * **Visual changes:** if pressing on the optic chiasm or nerve * **Personality changes:** irritability, mood swings, depression
201
What are the red flag signs for brain tumours?
**New Headaches:** * Severe and persistent * Woken by headache * Worse in morning * Worse Lying down * Associated with N+V * Exacerbated by coughing, sneezing and drowsiness **Features of raised ICP (papilloedema)** **Focal neurology (motor, sensory, visual)**
202
What is a Glioblastoma Multiforme?
Glioblastoma (GBM), also referred to as a grade IV astrocytoma, is a fast-growing and aggressive brain tumour **Most common primary tumour in adults** Often fatal within 1 year of Dx * On imaging they are solid tumours with central necrosis and a rim that enhances with contrast. * Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema. * Histology: Pleomorphic tumour cells border necrotic areas
203
What is a meningioma?
**Second most common primary brain tumour in adults** Benign extrinsic tumours arising from the meninges. CT/MRI scan with show contrast enhancement
204
What is a Pilocytic Astrocytoma? Finding on histology?
**Most common primary brain tumour in children** Has **Rosenthal fibres (corkscrew eosinophilic bundles)** on histology
205
What is a Medulloblastoma?
* A medulloblastoma is an aggressive paediatric brain tumour that arises within the infratentorial compartment. It spreads through the CSF system. Treatment is surgical resection and chemotherapy. * Histology: Small, blue cells. Rosette pattern of cells with many mitotic figures
206
What is an Ependymoma?
* Commonly seen in the 4th ventricle * May cause hydrocephalus * Histology: perivascular pseudorosettes
207
What is an Oligodendroma? Findings on histology
* Benign, slow-growing tumour common in the frontal lobes * Histology: Calcifications with 'fried-egg' appearance
208
What is a Haemangioblastoma?
* Vascular tumour of the cerebellum * Associated with von Hippel-Lindau syndrome * Histology: foam cells and high vascularity
209
What is a Craniopharyngioma? Derived from? Presentation?
**Most common paediatric supratentorial tumour** Solid/cystic tumour of the sellar region (Sellar turcica) derived from **Rathke's pouch** * Presents with Hormonal disturbance, hydrocephalus and bitemporal hemianopia
210
What are the investigations for a brain tumour?
MRI head - locate tumour Biopsy - determine grade Fundoscopy - Papilloedema due to raised ICP NO LP as this is CI in raised ICP
211
What is the management of brain tumours?
Depends on Type, Grade and Site: Tx is non-curative except for Grade I tumours Surgical removal if possible/reduce ICP (dexamethasone to reduce oedema) Chemotherapy/Radiotherapy Before/during/after surgery. (Radiotherapy is mainstay of treatment) Palliative Care
212
What are the differential Diagnoses of a brain tumour?
Aneurysm Abscess Cyst Haemorrhage Idiopathic intracranial hypertension
213
What are Pituitary Tumours?
* Pituitary gland – sits in pituitary fossa (behind nose and below base of brain) * Tumours are almost always benign and usually curable * Excessive effects of tumour * Local effects of tumour – bitemporal hemianopia * Inadequate hormone production by the remaining pituitary gland * Treated with Transsphenoidal surgical resection
214
What is an Acoustic Neuroma?
**Vestibular Schwannoma** (previously termed acoustic neuroma) * A benign tumour of schwaan cells forming the myelin sheath arising from the auditory (vestibulocochlear) nerve often found at the **cerebellopontine angle.**
215
Where do acoustic neuromas occur?
From the vestibulocochlear nerve at **the cerebellopontine angle** (sometimes referred to as cerebellopontine angle tumours)
216
What are the clinical features of an Acoustic Neuroma?
40-60 years presenting with gradual onset classically presenting with: * **Unilateral sensorineural Hearing loss** (often first symptom) * **Vertigo** * **Unilateral Tinnitus** * **Absent Corneal Reflex** **Other features:** * Dizziness or imbalance * Sensation of fullness in the ear * Facial nerve palsy - due to compression if the tumour grows large enough
217
What is the pathophysiology of the features of Vestibular Schwannomas?
Features can be predicted by the affected cranial nerves * cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus * cranial nerve V: absent corneal reflex * cranial nerve VII: facial palsy
218
What condition is associated with vestibular schwannomas?
Neurofibromatosis type II **Bilateral acoustic neuromas**
219
What are the investigations for an Acoustic neuroma?
**MRI of the cerebellopontine angle is the investigation of choice** **Audiometry:** pattern of sensorineural hearing loss
220
What may be seen on histology in an Acoustic neuroma?
Antoni A or B patterns Verocay bodies
221
What is the treatment for an Acoustic Neuroma?
Observation (watch and wait) Radiotherapy to reduce growth Surgery to remove the tumour
222
What are some complications of an Acoustic Neuroma?
* Vestibulocochlear nerve injury, with permanent hearing loss or dizziness * Facial nerve injury, with facial weakness
223
Define Bell's Palsy?
Defined as an acute, **unilateral lower motor neuron palsy** causing facial nerve paralysis without sparing the extraocular muscles and muscles of mastication. Often Idiopathic
224
What is the Aetiology of Bell's Palsy?
Largely idiopathic **Viral infections have been implicated as a particular cause** * **Reactivation of HSV 1** * EBV * VZV
225
What are the clinical features of Bell's Palsy?
**Acute (but NOT sudden; around 72 hours) onset of unilateral lower motor neurone facial weakness** * lower motor neuron facial nerve palsy → forehead affected * in contrast, an upper motor neuron lesion 'spares' the upper face * post-auricular pain (may precede paralysis) * altered taste * dry eyes * hyperacusis
226
What are some other causes of facial nerve palsy?
* Bell's palsy (pregnancy, DM) * Ramsay Hunt syndrome * Parotid gland tumour * Otitis media, cholesteatoma * Cerebello-pontine angle tumour * Stroke
227
What are the investigations for Bell's Palsy?
**Primarily a clinical diagnosisof exclusion** * Full Blood Count * ESR and CRP * Viral Serology * Lyme serology * Otoscopy * EMG * MRI/CT
228
What is the management of Bell's Palsy? 1st line Supportive Mx?
* Prompt administration (**Within 72 hours**) of **Oral steroids: 50mg of oral prednisolone or prednisone once daily for 10 days, followed by a taper.** * Aciclovir can be considered in select cases **Supportive Treatments:** * Artificial tears and ocular lubricants * Eye patch
229
What is the prognosis of Bell's Palsy?
**Complete Recovery:** 70-80% within 34 months **Incomplete recovery:** some patients may experience residual weakness or persistent facial symptoms Increased age and severity are associated with increased risk of incomplete recovery.
230
Define Epilepsy
Epilepsy is an umbrella term for a neurological condition where there is a tendency to generate epileptic seizures. **>2 episodes more than 24 hours a part**
231
Define a seizure
Seizures are transient episodes of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
232
What is the epidemiology of Epilepsy?
* Epilepsy affects approximately 50 million people worldwide, making it one of the most common neurological diseases. * Incidence rates vary depending on age, with higher rates in the very young and the elderly. * Both males and females are affected equally.
233
What is an epileptic seizure?
Paroxysmal event in which changes of behaviour, sensation or cognitive processes are caused by excessive (too much voltage), hypersynchronous neuronal discharges in the brain (unprovoked)
234
What is a Pseudoseizure (non-epileptic seizure)?
Paroxysmal event in which changes in behaviour, sensation and cognitive function caused by mental processes associated with psychosocial distress
235
What is the aetiology of Epilepsy?
Epilepsy can be **idiopathic, cryptogenic, or symptomatic.** Factors such as genetic predisposition, brain trauma, tumours, strokes, infectious diseases, or developmental disorders can contribute to the onset of epilepsy.
236
What are some risk factors for Epilepsy?
Family History Head Trauma Premature babies Cerebral Infections Dementia (10x more likely) Drug use - cocaine Cerebrovascular events
237
# VITAMIN DE List some causes of seizures?
**V**ascular - Stroke, HTN **I**nfection - Meningitis, Encephalitis **T**rauma/Toxins - Drugs/alcohol **A**utoimmune - SLE **M**etabolic - Hypocalcaemia, Hypoglycaemia, Hypo/hypernatraemia **I**diopathic - Epilepsy **N**eoplasms **D**ementia + Drugs (cocaine) **E**clampsia + everything else
238
What are the different types of Seizure?
* Generalised Seizure: Excessive neuronal activity across the whole brain * Focal (partial) seizure: Excessive neuronal activity in a specific part of the brain * Simple Focal * Complex Focal * Focal-Bilateral
239
What is a generalised Seizure?
A seizure that starts within both hemispheres of the brain at onset. They are bilateral **ALWAYS a loss of consciousness**
240
What are the different types of a Generalised Seizure?
**Non-Motor:** * **Absence seizures (petit mal):** brief pauses for less than 10 seconds. **Motor:** * **Tonic-clonic (Grand mal) seizures:** characterized by loss of consciousness, stiffening (tonic), and jerking (clonic) of limbs. **Post-ictal confusion is common.** * **Myoclonic seizures:** sudden jerks of a limb, trunk, or face. * **Atonic seizures:** sudden loss of muscle tone, causing the patient to fall, with consciousness retained.
241
What is an Absence Seizure?
* Absence seizures typically happen in children. * The patient becomes blank, stares into space and then abruptly returns to normal. * During the episode they are unaware of their surroundings and won’t respond. * These typically only lasts 10 to 20 seconds.
242
What is an Atonic Seizure? What condition is typically associated with Atonic Seizures?
* Atonic seizures are also known as drop attacks. * They are characterised by brief lapses in muscle tone. * These don’t usually last more than 3 minutes. * They typically begin in childhood. * **They may be indicative of Lennox-Gastaut syndrome.**
243
What is Todd's Paralysis?
Period after an epileptic seizure in which the patient experiences temporary paralysis
244
What are Focal Seizures?
Seizures that originate within one side of the brain and are usually confined to one region. They may progress to secondary lobes (Focal-bilateral seizures)
245
What are the different types of Focal Seizure?
**With impaired consciousness (complex):** * Patients lose consciousness, usually post an aura or at seizure onset. * Commonly originate from the **temporal lobe**, * post-ictal symptoms such as confusion are common. **Without impaired consciousness (simple):** * Patients retain consciousness, experiencing only focal symptoms. * Post-ictal symptoms are absent **Evolving to a bilateral** * convulsive seizure ('secondary generalised'): * patients first experience a focal seizure that evolves into a generalized seizure, typically tonic-clonic.
246
What are the symptoms of a focal seizure that arises from the Temporal Lobes?
They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: * Hallucinations (auditory, gustatory, olfactory) * Memory flashbacks * Déjà vu * Automatisms (Lip smacking, grabbing, plucking)
247
What is the Jacksonian March?
Where Focal Seizures may start with one muscle group being affected however this then spreads to involve other muscle groups as the abnormal electrical activity spreads. **Associated with Frontal lobe focal seizures**
248
How are seizures classified?
Where the seizures began Level of awareness during the seizure Other features of the seizure e.g. motor
249
What are the characteristics of an Epileptic Seizure?
* Duration – 30-120 seconds * Positive ictal symptoms – excess of something * Seeing/hearing something that isn’t there * Feeling touch when you aren’t being touched * Positive postictal symptoms * May occur from sleep * May be associated with other brain dysfunction – bleeds, stroke, tumours etc. * Tongue Biting * Incontinence * Typical seizure phenomena – lateral tongue bite, déjà v
250
What are the characteristics of a Pseudoseizure (non-epileptic)?
* Situational * Duration 1 – 20 mins * Dramatic motor phenomena or prolonged atonia (**Pelvic Thrusting**) * Eyes closed * Ictal crying and speaking * Surprisingly rapid or slow postictal recovery * History of psychiatric illness, other somatoform disorders
251
What are some differential diagnoses for Epilepsy?
* **Syncope:** characterized by a sudden, transient loss of consciousness and postural tone followed by spontaneous recovery. Triggered by low blood flow to the brain. * **Transient Ischemic Attack (TIA):** brief episodes of focal neurologic dysfunction caused by ischemia that does not cause lasting brain injury. Symptoms depend on the brain area affected. * **Migraines:** characterized by recurrent headaches often accompanied by a variety of symptoms such as visual disturbances (auras), nausea, vomiting, dizziness, extreme sensitivity to sound, light, touch and smell, and tingling or numbness in the extremities or face. * **Panic Disorder:** sudden periods of intense fear that may include palpitations, sweating, shaking, shortness of breath, numbness, or a feeling that something terrible is going to happen.
252
What are the investigations for seizures and epilepsy?
**Electroencephalogram (EEG)** shows different wave patterns for different forms of epilepsy **MRI brain** is used to diagnose structural pathology (e.g., tumours). **Additional investigations can be considered to exclude associated pathology:** * ECG * Serum electrolytes, including sodium, potassium, calcium and magnesium * Blood glucose for hypoglycaemia and diabetes * Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected
253
When is Neuroimaging indicated in the investigations of seizures?
The first seizure is in children under 2 years old Focal seizures There is no response to first line anti-epileptic medications
254
What are the goals of epilepsy treatment?
* Aim for optimum quality of life through seizure control, balanced against potential side effects, particularly teratogenesis in women of childbearing age. * Initiation of medication may not always be appropriate after a "provoked" first seizure; discuss such cases with a specialist. * The choice of antiepileptic drugs involves complexity due to the lack of head-to-head trials comparing different medications. * Interactions with other medications, particularly with phenytoin and carbamazepine, should be considered. * Issues regarding teratogenicity, particularly with valproate, which carries a high risk of neural tube defects, should be considered. Lamotrigine is a better choice for women of childbearing age.
255
What is the management of Focal seizures?
**Monotherapy up to 3rd line should be considered and then adjunctive therapy** **1st Line:** Lamotrigine or Levetiracetam **2nd Line:** Carbamazepine, Oxcarnazepine, Zonisamide **3rd Line:** Lacosamide
256
What is the management for Generalised Tonic-clonic seizures?
**1st Line:** Male = Sodium Valproate, Female = Lamotrigine/Levetiracetam **2nd Line:** Lamotrigine, Levetiracetam **Lamotrigine or Levetiracetam is first line in women of childbearing age who can have children**
257
What is the management of an Generalised Absence Seizure?
**1st Line**: Ethosuximide **2nd Line:** Sodium Valproate (male), Levetiracetam/Lamotrigine (female)
258
What general advice should be given to patients and families presenting with seizures?
**recognising, managing and reporting further seizures. Avoid situations where a seizure will put the patient at risk** * Take showers rather than baths * Be very cautious with swimming unless seizures are well controlled and they are closely supervised * Be cautious with heights * Be cautious with traffic * Be cautious with any heavy, hot or electrical equipment
259
What are some notable side effects of sodium valproate?
**Teratogenic, so patients need careful advice about contraception** Liver damage and hepatitis Hair loss Tremor
260
What are some notable side effects of Carbamazepine?
Agranulocytosis Exacerbate Absence seizures Aplastic anaemia Induces the P450 system so there are many drug interactions
261
What are some notable side effects of Phenytoin?
**Enzyme Inducer** **Dizziness** Folate and vitamin D deficiency * Megaloblastic anaemia (folate deficiency) * Osteomalacia (vitamin D deficiency) Gingival hyperplasia
262
What are some notable side effects of Ethosuximide?
Night terrors Rashes
263
What are some notable side effects of Lamotrigine?
* Stevens-Johnson Syndrome or DRESS syndrome: Life threatening skin rashes * Leukopenia
264
What is the acute management of seizures whilst the patient is experiencing an episode?
* Put the patient in a safe position (e.g. on a carpeted floor) * Place in the recovery position if possible * Put something soft under their head to protect against head injury * Remove obstacles that could lead to injury * Make a note of the time at the start and end of the seizure * Call an ambulance if lasting more than 5 minutes or this is their first seizure.
265
What are some complications of Epilepsy?
* **Status epilepticus:** Seizures lasting more than 5 minutes, necessitating immediate medical intervention. * **Psychiatric complications:** Increased risk of depression and suicide. * **Sudden unexpected death in epilepsy (SUDEP):** Thought to occur due to excessive electrical activity inducing a cardiac arrhythmia and subsequent death.
266
What is the DVLA guidance on epilepsy?
**You must stop driving straight away and inform DVLA** **First unprovoked/isolated seizure** * 6 months off if no relevant structural abnormalities on imaging or no definite epileptic form on EEG. * If conditions not met then increased to 12 months **Established Epilepsy or multiple unprovoked seizures:** * Apply for license after seizure free for 12 months * If no seizures for 5 years then a til 70 license is restored **Withdrawal of epilepsy medication:** * Should not drive during withdrawal of epilepsy medication * Should not drive for 6 months after last dose
267
What is Status Epilepticus?
Status epilepticus is defined as a seizure **lasting 5 minutes or more OR multiple seizures over 5 minutes without returning to a full level of consciousness between episodes**
268
What is the management of Status Epilepticus?
* Secure the airway * Give high-concentration oxygen * Assess cardiac and respiratory function * Check blood glucose levels and give glucose if patient is Hypoglycaemic * If suspiscion of alcohol abuse or impaired nutrition then give Pabrinex (thiamine replacement) * Gain intravenous access (insert a cannula) * **IV lorazepam 4mg Bolus**, repeated after 10 minutes if the seizure continues * If seizure is persistent then IV **Phenytoin** * If no response (refractory) within 45 minutes of onset then **Induce GA or give Phenobarbitol**
269
What are some medical options for management of status epilepticus in the community?
Buccal midazolam 10mg Rectal diazepam 10-20mg
270
What are the investigations done in status epilepticus?
**Check patients Oxygen saturations and blood glucose as these are common and rapidly reversible causes of seizures** **Blood tests:** ABG, FBC, U&E, LFT, CRP, Ca, Mg, Clotting
271
What is a Stroke?
A sudden interruption in the vascular supply of the brain that leads to infarction of brain tissue.
272
What is a Febrile Convulsion?
Seizures that occur in children whilst they have a fever They are not caused by epilepsy or other underlying neurological pathology.
273
What is the definition of an Ischaemic stroke?
A sudden onset focal neurological deficit of vascular aetiology brought on by transient decrease in blood flow commonly due to a blockage. With symptoms lasting >24 hours (or with evidence of infarction on imaging).
274
What are the main causes of an Ischaemic Stroke?
* Large Vessel Atherosclerosis (50%) * Intracranial small vessel atherosclerosis (25%) * Cardio-embolic (AF) (20%)
275
What are the risk factors for an ischaemic stroke?
* older age * male sex * family history of ischaemic stroke * Previous stroke or TIA * hypertension * smoking * diabetes mellitus * atrial fibrillation. Weaker risk factors: hypercholesterolaemia, obesity, poor diet, oestrogen-containing therapy, and migraine
276
What are the clinical features of a stroke?
* Limb weakness * Facial weakness * Dysphasia (speech dioxfsturbance) * Visual field defects * Sensory loss * Ataxia and vertigo (posterior circulation infarction) Stroke symptoms are usually asymmetrical
277
What is the first line investigation for someone presenting with a stroke?
**Non-contrast** Head CT Scan (very sensitive to haemmorhagic, but will appear normal in the first few hours of an ischaemic stroke)
278
What assessment criteria is used in ED to assess for stroke?
**ROSIER Score** (Recognition of Stroke in Emergency Room) * Loss of consciousness or syncope (-1 point) * Seizure activity (-1 point) **New, acute onset of:** * asymmetric facial weakness (+ 1 point) * asymmetric arm weakness (+ 1 point) * asymmetric leg weakness (+ 1 point) * speech disturbance (+ 1 point) * visual field defect (+ 1 point) **Stroke is likely if score > 0**
279
What is the most sensitive test for confirming an TIA?
Diffusion Weighted MRI Performed after specialist assessment in TIA clinic to determine the territory of ischaemia **NICE suggests to not offer CT brain scan in suspected TIA unless suspicion of alternative diagnosis**
280
What are some post-acute ischaemic stroke investigations?
* **Carotid Imaging:** ultrasound/CT/MRI or angiogram (critical carotid artery stenosis) * **ECG** (often ambulatory) **Others:** * CT/MR angiography (intracranial and extracranial stenosis) * Vasculitis and Thrombophilia screens (in young patients)
281
What are some post-acute Haemmorhagic stroke investigations?
Serum toxicology screen (sympathomimetic drugs (e.g. cocaine) are a strong risk factor)
282
What is the acute management of an ischaemic stroke?
Once a head CT has ruled out haemorrhage: Exclude Hypoglycaemia * **300mg Aspirin for 2 weeks** started ASAP once haemorrhagic stroke is ruled out * Admission to specialist stroke centre **THROMBOLYSIS (ATLEPLASE) IF:** (Assuming no contraindications, and haemorrhagic stroke excluded). * Within 4.5h since symptom onset * Between 4.5-9h since symptom onset AND imaging shows potential to salvage tissue * If woken with symptoms: Within 9h of midpoint of sleep AND potential to salvage tissue **MECHANICAL THROMBECTOMY IF:** (Assuming adequate pre-stroke functional status). * Within 6h of symptom onset, with occlusion of proximal anterior circulation * Between 6-24h of symptom onset/wellness, with occlusion of proximal anterior circulation AND potential to salvage brain tissue * Within 24h of symptom onset/wellness, with occlusion of proximal posterior circulation (i.e. basilar / posterior cerebral atery) AND potential to salvage brain tissue **If a person meets criteria for thrombolysis and mechanical thrombectomy, both can be given.** **MOST IMPORTANT:** * Within 4.5h, exclude haemorrhage, then give thrombolysis. * Within 6h, if proximal anterior circulation stroke, do mechanical thrombectomy. (Do both if both apply). **Beyond this, you need to do a CT perfusion / diffusion-weighted MRI scan to assess the potential to salvage brain tissue.**
283
What anti-coagulation should be given to patients who have an ischaemic stroke/TIA and AF?
After a stroke/TIA stop anticoagulation before haemorrhagic stroke is ruled out **Once haemorrhagic stroke is ruled out:** * **TIA:** Start DOAC immediately and no need to change anything else * **Stroke:** 2 weeks Clopidogrel then stop clop and restart DOAC again
284
Give some absolute contraindications for thrombolysis
T - Trauma or Recent Surgery Significant head trauma or major surgery in the past 3 months. H - Hemorrhage Any active bleeding or a history of intracranial hemorrhage. R - Recent Stroke Previous ischemic stroke within the past 3 months, unless this is the current indication for treatment. O - On Anticoagulants Use of anticoagulants with an elevated INR >1.7 or prolonged aPTT. M - Malignancy or Aneurysm Known intracranial neoplasm or cerebral aneurysm. B - Blood Pressure Elevated Severe hypertension: systolic >185 mmHg or diastolic >110 mmHg, unless safely lowered before treatment. O - Organ Bleeding Recent gastrointestinal or urinary tract bleeding (within the last 21 days). L - Low Platelets Platelet count <100,000/mm³ or coagulopathy. Y - Young (Age Not a Limitation) Age is not an absolute contraindication but proceed cautiously in elderly patients. S - Seizures Seizure at stroke onset if it suggests stroke is due to another cause (e.g., postictal phenomenon). I - Imaging Abnormalities CT/MRI showing evidence of: Intracranial hemorrhage. Extensive early ischemic changes (indicative of large infarct). S - Surgery Arterial puncture in a non-compressible site or lumbar puncture in the last 7 days.
285
What are the key steps for secondary stroke prevention?
* **Hypertension Control** - Anti-hypertensive therapy should be initiated 2 weeks post-stroke. * **Antiplatelet therapy** - Clopidogrel (75 mg once daily) or aspirin + Dipyridamole if clopidogrel is CI/ not tolerated * **Lipid-lowering therapy** - High dose Atorvostatin (20-80mg) once nightly (**delayed at least 48 hours**) Address Modifiable Risk Factors: * **Tobacco** - smoking cessation * **Sugar** - Diabetes Screen * Exercise * **Surgery** - Carotid endarectomy in patients with ipsilateral carotid artery stenosis
286
287
What are some stroke differential diagnoses?
* Hypoglycaemia * Drugs / Alcohol * Seizure * Migraine * Bell's Palsy * Tumour / Space occupying lesion * Meningitis / Encephalitis
288
What is the definition of a Haemmorhagic Stroke?
A cerebrovascular event that occurs when the wall of a blood vessel in the brain weakens and ruptures. This rupture causes bleeding in the brain, leading to haematoma formation, and, consequently, neuronal injury.
289
What is the epidemiology of haemmorhagic strokes?
They make up 15% of all strokes (the other 85% being ischaemic) * Increasing prevalence with age * More common in men * Intra-cerebral haemmorhages more common in Asians. Three quarters are intra-cerebral haemmorhages and the rest are sub-arachnoid.
290
What are the risk factors for a haemmorhagic stroke?
* Age * Chronic Hypertension * Male sex * Family history of haemorrhagic stroke * Haemophilia * Hereditary Polycystic Kidney disease and Ehlers-Danlos Syndrome * Cerebral amyloid angiopathy/hypertension * Anticoagulation therapy * Illicit sympathomimetic drugs (e.g. cocaine and amphetamines) * Vascular malformations (younger patients) Weaker RFs include: NSAIDs, Heavy alcohol use and Thrombocytopenia
291
What is the clinical presentation of a Sub-arachnoid Haemmorhage?
* Severe "Thunderclap" Headache * Neck Stiffness and Photophobia * Kernig's sign * Brudinski's sign
292
What is the general clinical presentation for a haemmorhagic stroke?
* Severe headache * Altered consciousness, ranging from drowsiness to coma * Vomiting * Focal neurologic signs * Seizures * Hypertension
293
What investigations should be done when a haemmorhagic stroke is suspected
**Head CT** - Is first line and diagnostic **MRI of the head** - Can help identify underlying causes MR Angiogram or Digital Substraction Angiography - Helps to identify vascular abnormalities that could have caused the bleed
294
What is the management for a haemmorhagic stroke?
After a head CT: STOP anticoagulant drugs + Vit K to reverse them **Surgical**: * Burr Hole Craniotomy (for Subdural haemmorhage) * Surgical Clipping or endovascular coiling (for Subarachnoid haemmorhage) **Pharmacological** * Hypertensive control * IV Fluids - To maintain cerebral perfusion * Nimlodipine (CCB) - Reduces cerebral artery spasm (subarachnoid) * IV Mannitol - Reduces Intra-cranial pressure (Sub and extra dural)
295
What is the definition of a TIA?
A sudden-onset focal neurological deficit with vascular aetiology, typically with symptoms resolving in less than 1 hour. It's caused by focal brain, spinal chord or retinal ischaemia without evidence of acute infarction.
296
What are the risk factors for a TIA?
* Increased age * Male sex * hypertension * diabetes mellitus * high cholesterol * atrial fibrillation and carotid stenosis * smoking, * History of past TIAs / Strokes or cardio-embolic events. * Family history of cardiovascular disease / stroke
297
What are the clinical features of a TIA?
Sudden onset neurological deficits (**Most resolving within 1 hour**) * Speech difficulty (dysphasia) * Arm or leg weakness * Sensory changes * Ataxia, vertigo or loss of balance * Visual disturbances: Homonymous hemianopia, diplopia or Amaurosis Fungax
298
What is Amaurosis Fugax?
The sudden painless loss of vision in 1 eye Caused by an emboli passing into the retinal, ophthalmic or ciliary artery; causing temporary retinal hypoxia.
299
What are some differential diagnoses for a TIA?
Ischaemic Stroke Haemorrhagic Stroke Migraine with Aura Focal Motor Seizures
300
What is the main diagnostic investigation for a TIA?
Diffusion weighted MRI Scan
301
What other investigations are recommended for a TIA?
Carotid Ultrasound Echocardiogram (looking for cardiac thrombus) 24 Hour Tape (looking for AF) Blood Tests (glucose, lipid profile, clotting factors)
302
What is the management for a TIA? Initial Mx Assessment of Underlying Cause Secondary Prevention? Specialist initiated therapy? DVLA Rules?
**Immediate assessment (ideally within 24 hrs of symptoms)** * **300mg Aspirin** immediately unless CI * Referral for specialist assessment within 24 hrs (within 7 days if TIA more than 7 days ago) **Assessment of Underlying Cause** * Carotid Imaging (and subsequent intervention if significant stenosis) * ECG/ambulatory ECG **Secondary Prevention:** Lifestyle modification * Regular exercise * Smoking cessation * Healthy Diet **Control of vascular risk factors** * Hypertension (ACE inhibitor (e.g. ramipril), or ARB (e.g. candesartan) * Diabetes * Cholesterol (Atorvastatin 80mg OD) **Specialist initiated Antiplatelet Therapy started within 24 hours** Given as long as no CI/high risk of bleeding * Patients within 24hr onset of TIA with low risk of bleeding given DAPT: * Clopidogrel ( initial 300mg then 75mg OD) + Aspirin (initial 300mg then 75mg OD) for 21 days * Followed by Clopidogrel monotherapy 75mg OD * Alternative is Ticagrelor + clopidogrel for 30 days * If not appropriate for DAPT * Clopidogrel 300mg loading dose followed by 75mg OD * PPI considered for DAPT therapy. **No driving until seen by specialist** * 1 TIA = 1 month no driving and no DVLA informed * Multiple TIAs = 3 months no drive and inform DVLA
303
What is the classification of Strokes?
**Oxford Stroke Classification** also known as **Bamford** **LOOK, MOVE, SPEAK** * Homonymous hemianopia * Hemiparesis/Hemisensory loss of face, arm & leg * High cognitive dysfunction (eg. dysphasia)
304
What are the features of an **Anterior Cerebral Artery Infarct**?
Contralateral hemiparesis and sensory loss, lower extremity > upper
305
What are the features of a **Middle Cerebral Artery Infarct**?
Contralateral hemiparesis and sensory loss, upper extremity > lower Contralateral homonymous hemianopia Aphasia
306
What are the features of a **Posterior Cerebral Artery Infarct**?
Contralateral homonymous hemianopia with macular sparing Visual agnosia
307
What is a Total Anterior Circulation Infarct (TACI) defined by?
* Contralateral hemiplegia or hemiparesis, AND * Contralateral homonymous hemianopia, AND * Higher cerebral dysfunction (e.g. aphasia, neglect) A TACI involves the anterior AND middle cerebral arteries on the affected side.
308
What is a Partial Anterior Circulation Infarct (PACI) defined by?
* 2 of the factors for a TACI OR * Higher cerebral dysfunction alone. A PACI involves the anterior OR middle cerebral artery on the affected side.
309
What is a Lacunar Infarct defined by?
**A LACI affects small deep perforating arteries, typically supplying internal capsule or thalamus.** It can be any of: * A pure motor stroke * Pure sensory stroke * Sensorimotor stroke * Ataxic hemiparesis * Dysarthria-clumsy hand syndrome.
310
What is a Posterior Circulation Infarct (POCI) defined by?
A POCI involves the vertebrobasilar arteries and associated branches (supplying the cerebellum, brainstem, and occipital lobe). Presents with 1 of the following: * Cerebellar or brainstem syndromes * Loss of consciousness * Isolated homonymous hemianopia
311
What are the 4 Posterior Stroke Syndromes?
* **Basillar Artery Occlusion** - Causes locked in syndrome, loss of consciousness or sudden death * **Wallenberg's syndrome** (lateral medullary syndrome) caused by occlusion of the posterior inferior cerebellar artery * **Lateral pontine syndrome** - Caused by occlusion of the anterior inferior cerebellar artery * **Weber's syndrome/ Medial Midbrain Syndrome** - Caused by occlusion of the paramedian branches of the upper basilar and proximal posterior cerebral arteries
312
Define Locked in Syndrome
It's Quadriparesis with preserved consciousness and ocular movements. Its caused by Basillar Artery Occlusion
313
What is the clinical presentation of Lateral Medullary Syndrome (Wallenbergs)?
**Crossed sensory findings:** * Ipsilateral loss of pain and temperature sensation on the face * Contralateral loss of pain and temperature sensation over the contralateral body. **Cerebellar Features:** * Ataxia * Nystagmus **Cranial Nerve Involvement::** * Dysphagia * Hoarseness * Horners Syndrome - due to damage of the lateral medulla which controls sympathetic 1st order neurones
314
Describe Kernig's Sign
The patient is positioned lying on their back with both the hip and knee flexed at 90 degrees. The examiner then attempts to extend the patient's knee. If the patient experiences pain and resistance to knee extension, especially when attempting to straighten the leg, it is considered a positive Kernig's sign. This sign suggests meningeal irritation or inflammation.
315
Describe Brudzinski's Sign
The examiner gently flexes the patient's neck forward toward the chest while the patient is lying on their back. If the patient involuntarily flexes their hips and knees in response to neck flexion, it is considered a positive Brudzinski's sign.
316
What is the clinical presentation of Lateral Pontine Syndrome?
Its the same as Wallenberg's Syndrome, but with additional involvement of pontine cranial nerve nuclei (CN 5, 6,7) Leading to additional: * Ipsilateral facial paralysis * Ipsilateral Deafness
317
What is the clinical presentation of Weber's Syndrome?
It causes an ipsilateral oculomotor nerve palsy and contralateral hemiparesis.
318
Define Haemorrhage?
An active ongoing bleed
319
Define Haematoma
A bleed that has mostly clotted and hardened
320
Where can intracranial haemorrhages occur?
Extradural Subdural Subarachnoid Intracerebral
321
What are the main blood vessels within the meninges?
Extradural – middle meningeal artery - from maxillary artery Subdural – bridging veins Subarachnoid – circle of Willis Pia – no vessels as it forms part of the blood-brain barrier
322
What is an Extradural Haematoma (EHD)?
A pathological condition where blood collects between the dura mater, the outermost meningeal layer, and the inner surface of the skull. This condition is commonly arterial in origin, with the middle meningeal artery often implicated.
323
What is the epidemiology of an EDH?
EDH predominantly affects young patients who have experienced a head injury, such as during sports or road traffic accidents.
324
What is the Aetiology of an EDH?
EDH is almost **always trauma-related, specifically severe head trauma** that results in a tear of the middle meningeal artery. The cause of an EDH is typically an identifiable traumatic event
325
What is the most common artery damaged in an EDH?
**Middle Meningeal Artery** due to damage to the temporoparietal region
326
What are some rarer non-traumatic causes of an EDH?
Haemorrhagic tumour Coagulopathy Infection Vascular Malformation
327
What are the risk factors for an EDH?
Younger age (20-30) Male Anticoagulant usage High contact/dangerous sports
328
Why is an EDH less likely to occur in the elderly?
The Dura matter is more firmly adhered to the skull so blood is less likely to accumulate in this region.
329
What is the typical presentation of an Extradural bleed?
Patients with EDH often present with a characteristic clinical course: * Initial brief loss of consciousness following the trauma * A period of regained consciousness and apparent recovery (the lucid interval) * Subsequent deterioration of consciousness and the onset of a headache and signs of raised ICP such as fixed and dilated pupil
330
Why do you get a fixed and dilated pupil in and EDH?
Expanding haematoma leads to brain herniation. The uncus of the temporal lob herniates around the tentorium cerebelli Compresses parasympathetic fibres of CN3 leading to a fixed and dilated pupil
331
What are some differential diagnoses for an EDH?
* **Subdural haematoma:** Presents with fluctuating levels of consciousness, memory impairment, and headache. This is more common in older patients, often with a history of minor head trauma. * **Subarachnoid haemorrhage:** Characterized by a sudden, severe headache (often described as a 'thunderclap' headache), nausea, vomiting, and neck stiffness. * **Intracerebral haemorrhage:** Presents with sudden onset severe headache, vomiting, high blood pressure, and signs of increased intracranial pressure. * **Cerebral contusion:** Might manifest with loss of consciousness, seizures, and neurological deficits specific to the affected cerebral region.
332
What is the first line and diagnostic investigation for an extradural haemorrhage?
**Head CT Scan** * Shows a lentiform/biconvex hyperdense extra-axial collection (usually unilateral and supratentorial) * **Collection is often confined within suture lines** * Secondary features: Midline shift, Uncal herniation
333
What does this show?
CT head showing an Extradural Haemorrhage
334
What is the management of an EDH?
Management of EDH depends on the severity of the symptoms and the extent of the mass effect on the brain. * Stabilise the patient: ABCDE management * Urgent neurosurgical referral: * Burr hole craniotomy * Ligation of bleeding vessels * IV mannitol to reduce ICP
335
What are some complications of an EDH?
Cerebral oedema Raised intracranial pressure and herniation Ischaemia: can occur due to mass effect, herniation, hypoperfusion, vasospasm Seizures Infection
336
Define a subdural haemorrhage (SDH)?
An accumulation of venous blood in the potential space between the dura mater and arachnoid mater of the brain.
337
How can SDH be classifed?
**Acute:** Symptoms usually develop within 48 hours of injury, characterised by rapid neurological deterioration **Subacute:** Symptoms manifest within days to weeks post-injury, with a more gradual progression. **Chronic:** Common in the elderly, developing over weeks to months. Patients may not recall a specific head injury.
338
What is the epidemiology of a subdural haemorrhage?
Typically occurs at extremes of age (mainly elderly) and is the result of a minor trauma causing shearing forces and bridging vein tears.
339
What are the risk factors for a SDH?
* Advancing age >65yrs * Bleeding disorders or anticoagulation use * Dementia (causes brain atrophy) * Chronic Alcohol use * Recent head trauma * Abused children (Shaken baby syndrome) **The patients typically have atrophied brains which makes the vessels more prone to rupture**
340
What blood vessel is the common culprit in a subdural haemorrhage?
Tearing of bridging veins due to shearing or deceleration injury
341
What is the pathophysiology of a Subdural haemorrhage?
* Trauma either due to deceleration due to violent injury or due to dural metastases results in bleeding from bridging veins between cortex and venous sinuses * Bridging veins bleed and form a haematoma (solid swelling of clotted blood) between the dura and arachnoid * This reduces pressure 🡪 bleeding stops * Days/weeks later the haematoma starts to autolyse due to the massive increase in oncotic and osmotic pressure 🡪 water is sucked into the haematoma 🡪 haematoma enlargement 🡪 gradual rise in intra-cranial pressure (ICP) over many weeks * This shifts midline structures away from the side of the clot and can lead to tectorial herniation and coning (brain herniates through foramen magnum) if left untreated
342
What is the clinical presentation of a subdural haemorhage? Neuroogical Symptoms Physical Examination Findings Behavioural changes Associated Features
**Neurological Symptoms:** * Altered Mental Status: Ranging from mild confusion to deep coma. Fluctuations in the level of consciousness are common. * Focal Neurological Deficits: Weakness on one side of the body, aphasia, or visual field defects, depending on the haematoma's location. * Headache: Often localised to one side, worsening over time. * Seizures: May occur, particularly in acute or expanding hematomas. **Physical Examination Findings:** * Papilloedema: Indicates raised intracranial pressure. * Pupil Changes: Unilateral dilated pupil, especially on the side of the haematoma, indicating compression of the third cranial nerve. * Gait Abnormalities: Including ataxia or weakness in one leg. * Hemiparesis or Hemiplegia: Reflecting the mass effect and midline shift. **Behavioural and Cognitive Changes:** * Memory Loss: Especially in chronic SDH. * Personality Changes: Irritability, apathy, or depression. * Cognitive Impairment: Difficulty with attention, problem-solving, and other executive functions. **Other Associated Features:** * Nausea and Vomiting: Secondary to increased intracranial pressure. * Drowsiness: Progressing to stupor and coma in severe cases. * Signs of Increased Intracranial Pressure: Such as bradycardia, hypertension, and respiratory irregularities (Cushing's triad).
343
What are some differential diagnoses for a subdural haemorhage?
* **Epidural haematoma:** Characterized by a brief loss of consciousness, followed by a "lucid interval" and then rapid neurological deterioration. * **Traumatic brain injury:** Symptoms may include headache, confusion, lightheadedness, dizziness, blurred vision, or tired eyes. * **Stroke:** Presents with sudden numbness or weakness, especially on one side of the body, confusion, trouble speaking or understanding, trouble seeing in one or both eyes, and trouble walking, dizziness, or loss of balance or coordination. * **Migraine:** Recurrent headaches that might be accompanied by nausea, vomiting, and sensitivity to light and sound.
344
What is the diagnostic investigation for a subdural haemorrhage? What is seen at the different time intervals?
**Head CT Scan:** The appearance of the clot varies based on its age: * **Hyperacute phase (<1 hour):** The clot may appear isodense, with underlying cerebral oedema. * **Acute phase (<3 days):** The clot appears as a crescent-shaped hyperdense extra-axial collection over the affected hemisphere. * **Sub-acute phase (3 days to 3 weeks):** The clot appears more isodense compared to the adjacent cortex, making identification more difficult. **Contrast-enhanced CT or MRI can aid identification.** There may be associated mass effect causing midline shift and sulcal effacement. * **Chronic phase (>3 weeks):** The haematoma appears hypodense relative to the adjacent cortex.
345
What does this show?
A Subdural Haemorrhage
346
What percentage of Subdural haemorrhages are bilateral in nature?
15% in adults 80% in infants
347
What is the management of a Subdural Haemorrhage?
The management of a subdural haematoma depends on its stage: **Surgical Decompression:** * **Acute haemorrhages:** Craniotomy is the preferred method. * **Chronic haemorrhages:** Burr holes are typically recommended. The overall goal is to relieve pressure on the brain and to treat or prevent any related neurological symptoms or complications.
348
Define a Subarachnoid haemorrhage?
A Subarachnoid Haemorrhage (SAH) is a bleed that occurs in the subarachnoid space, which lies beneath the arachnoid mater, one of the protective layers of the brain.
349
What is the epidemiology of an SAH?
Typical age 35-65 – mean age 50 Account for ~5% of strokes 50% die straight away or soon after 8-12 per 100, 000/year
350
Who do SAHs more commonly affect?
Black patients Female patients Age 45-70
351
What is the commonest cause on an SAH?
**Head Trauma** causing a traumatic SAH. **Spontaneous SAH** * 80% of SAH is due to a ruptured **berry aneurysm** without trauma
352
What are possible causes of an SAH?
**Traumatic SAH:** Caused by trauma **Spontaneous SAH:** * **Berry aneurysm rupture** * Idiopathic * AVM * Pituitary apoplexy
353
What are some risk factors for SAH?
* Hypertension * Adult polycystic kidney disease * Ehlers-Danlos Syndrome * Increased Age >50yrs * Family History * Excessive alcohol consumption * Smoking **Strong associations with cocaine use and sickle cell disease**
354
Where do berry aneurysms often occur?
At junctions of arteries within the Circle of Willis Most commonly the **Anterior Communicating Artery**
355
What is the clinical presentation of an SAH?
* **Sudden onset severe headache** described as the worst headache of the patient's life * History of physical exertion or coitus prior to onset * Possible loss of consciousness or vomiting * A previous 'sentinel headache' that was similar but not as severe * Reduced level of consciousness (reduced GCS) * **Meningism (e.g., neck rigidity)** * Nausea and Vomiting * Seizures and Coma * Retinal haemorrhages as seen with ophthalmoscope examination * Localising focal neurological signs depending on the location of the bleed
356
What are some signs of an SAH?
Meningeal irritation: * Kernig's sign * Brudzinski's sign Sub-hyaloid haemorrhages (bleeding between retina and vitreous membrane) ± papilloedema
357
What are the characteristics of the headache experienced in an SAH?
Occipital Thunderclap Headache Sudden onset Worst headache of their life (0-10 instantly) May have a sentinel headache preceding this
358
Why may you get nerve palsies in SAH?
3rd nerve palsy: An aneurysm arising from the posterior communicating artery will press on the 3rd nerve, causing a palsy with a fixed dilated pupil 6th nerve palsy: A non-specific sign which indicates raised intracranial pressure
359
What are some differential diagnoses for an SAH?
* **Migraine:** Characterised by recurrent, severe, pulsating headache lasting from 4 to 72 hours. May be accompanied by nausea, vomiting, photophobia, and phonophobia. * **Tension headache:** Presents with bilateral, pressing or tightening pain that does not worsen with physical activity. The intensity of the pain is usually mild to moderate. * **Intracerebral haemorrhage (ICH):** Accompanied by sudden onset of headache, nausea, vomiting, and neurological deficits based on the location of the haemorrhage. * **Meningitis:** Fever, severe headache, neck stiffness, photophobia, and altered mental status are typical signs.
360
What is the first line investigation for an SAH?
* **Non-contrast CT head** is the first-line investigation due to its high sensitivity, especially within the first 24 hours of symptom onset. * **Star shaped sign** * Hyperdense blood distributed in basal cisterns * Diagnostic with 100% sensitivity if performed within 6 hours of onset.
361
What is the investigation structure for an SAH?
* **CT head is the first-line investigation** **If CT head is done < 6 hours after onset and is normal** * Consider alternative Diagnosis **If CT head is done > 6 hours after onset and is normal** * Do a lumber puncture (LP): * LP should be performed **at least 12 hours** following the onset of symptoms to allow the **development of xanthochromia** (the result of red blood cell breakdown). * xanthochromia helps to distinguish true SAH * A normal or raised opening pressure **After SAH is confirmed then Ix to find causative pathology** * CT- angiogram for evidence of berry aneurysms/AVM
362
What does this show?
Subarachnoid Haemorrhage
363
What is the management of an SAH?
**1st line - Neurosurgery Referral:** * 1st - Endovascular coiling * 2nd - Surgical clipping **Medical Management:** * Give immediate Nimodipine (CCB) 60mg for 3 weeks - prevents vasospasm (only in critical care setting) * IV Fluids - maintain cerebral perfusion and for resuscitation * Monitoring for complications **Supportive** * bed rest * analgesia * venous thromboembolism prophylaxis * discontinuation of antithrombotics (reversal of anticoagulation if present)
364
What is the prognosis of an SAH?
Mortality is 50% with a significant proportion not reaching the hospital Patients admitted with a GCS of >14 have a >90% survival and experience low morbidity
365
What are some complications of an SAH?
Re-bleeding Cerebral ischaemia - due to vasospasm Hydrocephalus – due to blockage of arachnoid granulations Hyponatraemia - often due to SIADH Seizures
366
What is the Cushing's Triad?
Hypertension (Wide Systolic Pulse Pressure) Bradycardia Respiratory irregularity
367
What is the Pathophysiology of Cushing's Triad
* Increased ICP - exceeds MABP of cerebral vessels - causes cerebral ischaemia * Cerebral ischaemia activates Symp NS - Increases adrenergic action on alpha1 receptors - increases vasoconstriction causing HTN * HTN activates baroreceptors of aortic arch - activates P.symp NS to decrease HR (bradycardia) * HTN presses on respiratory centre - causes irregular breathing
368
What is an intracerebral haemorrhage?
Bleeding within the brain tissue They present similarly to an ischaemic stroke with sudden onset focal neurological symptoms. They may occur spontaneously or secondary to ischaemic stroke, tumours or aneurysm rupture
369
Where do intracerebral haemorrhages occur?
* Lobar intracerebral haemorrhage * Deep intracerebral haemorrhage * Intraventricular haemorrhage * Basal ganglia haemorrhage * Cerebellar haemorrhage
370
Define Giant Cell Arteritis?
Giant cell arteritis (GCA), also known as temporal arteritis, is a condition where the arteries, particularly those at the side of the head (the temples), become inflamed.
371
What is the epidemiology of GCA?
* GCA is the most common type of primary vasculitis. * It affects adults over the age of 50 and has a female preponderance. * Caucasian individuals have the highest incidence of disease. * Often associated with Polymyalgia Rheumatica
372
What are the clinical features of GCA?
* **Temporal headache:** GCA can cause blindness and stroke, so should be considered in elderly people with any headache * **Jaw claudication:** Pain on chewing food * **Amaurosis fugax:** transient monocular blindness, often described as a dark curtain descending vertically * **Systemic features:** these are common and include fatigue, fevers, weight loss and malaise The onset can be acute or insidious. **In addition, GCA and polymyalgia rheumatica often occur together, so symmetrical proximal muscle weakness and oligoarthritis may occur.**
373
What may be found on examination in GCA?
* Thickened, tender temporal artery on examination, which may be pulseless * Scalp tenderness * (Rarely) arterial bruits, asymmetrical blood pressure and absent pulses
374
What are some complications of GCA?
Permanent monocular blindness Stroke Aortic aneurysms
375
What are the first line investigations for GCA?
* **Inflammatory marker blood tests (ESR and CRP)** * FBC: patients often have normochromic, normocytic anaemia * LFTs: 1/3rd have mildly abnormal LFTs
376
What is the gold standard investigation to diagnose GCA?
**Temporal Artery Biopsy** * Granulomatous inflammation * Infiltration of giant cells * 3-5cm of the artery should be biopsied due to skip lesions * A negative biopsy does not rule out disease **Doppler Ultrasonography** * Halo sign in the vessel wall
377
What is the management of GCA?
**Immediate High dose Steroids** * **40-60mg oral prednisolone OD**: if no visual symptoms * **500mg-1000mg IV Methylprednisolone** if visual symptoms * Taper steroids gradually over 1-2 years once symptoms resolved **Urgen Ophthalmology Review** **Other Medications:** * Bisphosphonates + Ca and Vit D for bone protection whilst on steroids * PPIs (Omeprazole) for gastric protection whilst on steroids * Low-dose Aspirin to further reduce risk of stroke and blindness
378
What are some complications of GCA?
Steroid-related complications (e.g., weight gain, diabetes and osteoporosis) Visual loss Cerebrovascular accident (stroke)
379
Define Horner's Syndrome?
Horner's syndrome is a condition characterised by a set of signs and symptoms that occur due to a disruption in the sympathetic nerve supply to the eye. The syndrome can be categorised into pre-ganglionic, post-ganglionic, and central causes, depending on the location of the sympathetic nerve interruption.
380
What is the aetiology of Horner's Syndrome?
* **Pancoast tumour:** This non-small cell lung carcinoma located at the superior sulcus of the lung affects the lower roots of the brachial plexus and the sympathetic chain. * **Stroke:** Specifically **lateral medullary infarction** or **Wallenberg's syndrome** can affect the central neuron. * **Carotid artery dissection:** Especially prevalent in younger patients, this factor can be a significant cause. Accompanying neck pain can be a red flag symptom. * **Additional factors:** Neck trauma, surgeries, or tumours affecting the sympathetic chain.
381
What are the clinical features of Horner's Syndrome?
* **Ptosis:** Drooping of the upper eyelid * **Miosis:** Constriction of the pupil * **Anhidrosis:** Lack of sweating on the affected side of the face Others: * Enophthalmos: In certain cases, the eye may appear sunken * Heterochromia: Eye colour may change, more commonly observed in congenital Horner's syndrome
382
What condition is shown here?
Horner's Syndrome: * Ptosis * Miosis * Anhidrosis
383
What are some differential diagnoses for Horner's Syndrome?
* **Oculomotor nerve palsy:** In addition to ptosis and pupillary abnormalities, patients typically exhibit ophthalmoplegia. * **Myasthenia gravis:** Apart from ptosis, which often varies throughout the day, symptoms include muscle weakness and fatigue. * **Bell's palsy:** Facial droop is present but it involves the entire side of the face and is usually accompanied by loss of taste on the front two-thirds of the tongue and hyperacusis on the affected side.
384
What are the investigations for Horner's Syndrome?
**Imaging:** MRI or CT of the head, neck and chest to identify potential structural causes such as tumour or vascular anomalies **Blood tests:** to assess for conditions that may be released to the underlying causes such as diabetes or autoimmune disorders
385
How can Horner's Syndrome lesion be localised?
**Central Lesions:** Anhidrosis of face, arm and trunk * Stroke * Syringomyelia * Multiple Sclerosis * Tumour * Encephalitis **Preganglionic lesions:** Anhidrosis of the face * Pancoast's Tumour * Thyroidectomy * Trauma * Cervical Rib **Post-ganglionic Lesions:** No anhidrosis * Carotid Artery Dissection * Carotid Aneurysm * Cavernous Sinus Thrombosis * Cluster headache
386
What is the management for Horner's Syndrome?
The management of Horner's syndrome primarily targets the underlying cause: * **Pancoast tumour:** Treatment may involve surgery, chemotherapy, and/or radiation therapy. * **Stroke:**stroke management protocol is followed * **Carotid artery dissection:** Depending on the severity, the patient may require anticoagulation therapy or carotid artery surgery. * **No underlying cause can be identified:** Observation and regular follow-up appointments may be sufficient.
387
What are the primary headaches?
**No underlying causes associated:** * Tension Headache * Migraine * Cluster Headache * Trigeminal Neuralgia
388
What are some causes of secondary headaches?
**Due to an underlying cause:** * Medication Overuse Headache * Subarachnoid Haemorrhage * Vasculitis (Giant Cell Arteritis) * Sinusitis * Illness * Brain tumours * Raised ICP * Meningitis/Encephalitis * Head Injury * Hormones
389
What is the Epidemiology of Tension headaches?
* Tension headaches are the most common cause of chronic recurring head pain * More likely to affect women. * They have a lifetime prevalence of 30-70%.
390
What is the clinical presentation of Tension Headaches?
* **Bilateral, non-pulsatile headaches** * Tightness/pressing sensation, like a **band around the head** * Scalp muscle tenderness * Resolve gradually and do not produce any visual symptoms
391
What are some potential triggers for Tension Headaches?
Stress Depression Alcohol Skipping meals Dehydration
392
What is the management of Tension Headaches?
* Reassurance * Simple Analgesia: **Paracetamol, Ibuprofen** * Addressing potential triggers such as stress **Prophylaxis Recommended by NICE** includes 10 sessions of acupuncture
393
What medication may be used if Tension headaches are frequent or chronic?
Amitriptyline
394
What are Cluster Headaches?
Cluster headaches are severe and unbearable unilateral headaches, usually centred around the eye.
395
What is the epidemiology of Cluster Headaches?
Typically affect 30-50 year old males Males 3:1 Smokers
396
What are some potential triggers for Cluster Headaches?
Alcohol Strong Smells Exercise
397
What are the clinical features of Cluster headaches?
**Headache:** * **Recurrent severe unilateral headache centred around one eye (periorbital)** * Sensation of a boring hot poker * Headache duration of **15mins to 3 hours** occuring **once or twice daily over 4-12 weeks**. This is followed by a **pain free period** of several months **Associated symptoms are typically unilateral:** * Red, swollen and watering eye * Pupil constriction (miosis) * Eyelid drooping (ptosis) * Nasal discharge * Facial sweating
398
What are some investigations that may be used in Cluster Headaches?
**Neuroimaging** is often used to assess for underlying brain lesions * MRI **with gadolinium contrast** is the Ix of choice
399
What is the management of Cluster Headaches?
* Avoid Triggers * **Acute Attacks:** **15L 100% oxygen** via a non-rebreathable mask and Subcutaneous **Triptans** * **Prophylaxis:** Verapamil
400
Define Migraines?
* Migraines are a neurological condition often characterised by intense, debilitating headaches, usually unilateral and pulsating in nature. * Symptoms may be preceded by an 'aura' which manifests as visual disturbances or sensory changes. * The pain usually lasts from 4-72 hours * Can be accompanied by nausea, vomiting, photophobia, and phonophobia.
401
What is the epidemiology of migraines?
* Migraines are one of the most prevalent neurological disorders worldwide, affecting roughly 12% of the global population. * It is more common in women, with a male to female ratio of approximately 1:3, likely related to hormonal influences. * The peak incidence occurs between the ages of 30-39.
402
What is the Aetiology of Migraines?
Not fully understood. Likely combination or genetic and environmental triggering factors
403
# CHOCOLATES What are some potential triggers of Migraines?
**C**hocolate **H**angovers **O**rgasms **C**heese **O**ral contraceptives **L**ie ins and disrupted sleep **A**lcohol **T**umult (loud noise) and bright lightts **E**xercise **S**tress
404
What are the different types of Migraine?
Migraine without aura Migraine with aura Silent migraine (migraine with aura but without a headache) Hemiplegic migraine
405
What are the main stages of a Migraine with an aura?
* Prodromal stage (can begin several days before the headache) * Aura (lasting up to 60 minutes) * Headache stage (lasts 4 to 72 hours) * Resolution stage (the headache may fade away or be relieved abruptly by vomiting or sleeping) * Postdromal or recovery phase
406
What is a Hemiplegic Migraine?
* The main feature of hemiplegic migraines is hemiplegia (unilateral limb weakness). Other symptoms may include ataxia (loss of coordination) and impaired consciousness. * **Familial hemiplegic migraine is an autosomal dominant genetic condition characterised by hemiplegic migraines that run in families**. * However, hemiplegic migraines also occur without any genetic link or family history. * Hemiplegic migraines can mimic a stroke or TIA. It is essential to exclude a stroke with sudden-onset hemiplegia.
407
What are the clinical features of a Migraine with aura?
Aura (usually visual or sensory symptoms preceding the headache) **Unilateral throbbing/pulsatile** headache lasting 4-72 hours Photophobia and phonophobia Nausea and/or vomiting
408
What are some potential symptoms experienced in the aura before a Migraine?
Aura can affect vision, sensation or language. **Visual symptoms are the most common:** * Sparks in the vision * Blurred vision * Lines across the vision * Loss of visual fields (e.g., scotoma) **Sensation changes** may include tingling or numbness. **Language symptoms** include dysphasia (difficulty speaking).
409
What is the International Headache Society Criteria for Migraines without Aura?
**A. At least 5 attacks fulfilling criteria B-D** **B. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated)** **C. Headache has at least two of the following four characteristics:** * Unilateral location * Pulsating quality * Moderate to severe pain intensity * Aggravation by or causing avoidance of routine physical activity **D. During headache, at least one of the following:** * Nausea and/or Vomiting * Photophobia and Phonophobia **E. Not better accounted for by another ICHD-3 Diagnosis**
410
What are some differential diagnosis for Migraines?
* **Tension-type headache:** Bilateral, band-like pressure or tightness, not worsened with physical activity, no associated nausea or vomiting. * **Cluster headache:** Severe, unilateral, orbital, supraorbital and/or temporal pain lasting 15-180 minutes, occurring up to 8 times a day, associated with autonomic symptoms like ptosis, miosis, lacrimation. * **Subarachnoid haemorrhage:** Sudden-onset severe headache, often described as "the worst headache of my life", associated with nausea, vomiting, and possible loss of consciousness. * **Giant cell arteritis:** New headache in a person over 50 years, scalp tenderness, jaw claudication, visual disturbances, elevated ESR and CRP.
411
What are the investigations for Migraines?
**Primarily a clinical diagnosis based on the history and examination** * If secondary causes of headaches are suspected then **Neuroimaging** (CT/MRI) and **blood tests** (ESR, CRP for GCA)
412
What is the acute management of Migraines?
* Avoidance of Triggers **Acute Management:** * Triptans (sumatriptan) * In addition: **Paracetamol or NSAIDs** * Female patients experiencing migraines with aura are **advised against taking the OCP**
413
What preventative measures can be taken for Migraines?
**Prophylaxis:** * Propranolol * Topiramate * Amitriptyline **NICE 2023:** * **Rimegepant** is an option for preventing episodic migraine in adults where at least 3 previous preventive treatments have failed. * Adults must have at least 4 migraine attacks per month but less than 15.
414
What condition may Triptans be contra-indicated in and why?
Ischaemic Heart Disease They are 5-HT receptor agonists and therefore can induce vasoconstriction increasing the risk of stroke **Can also lead to serotonin syndrome**
415
What are medication overuse headaches?
Medication overuse headache is associated with long-term use of analgesics.
416
What are the features of a Medication Overuse Headache?
* Present for 15 days or more per month * Developed or worsened whilst taking regular symptomatic medication * Patients using opioids and triptans are at most risk * May be psychiatric co-morbidity
417
What is the management of Medication overuse headaches?
The cornerstone of management involves discontinuation or reduction of the overused medication. **Simple Analgesia and Triptans** should be withdrawn abruptly **Opioid Analgesia** should be gradually withdrawn
418
Define Trigeminal Neuralgia?
A chronic pain condition characterized by severe, sudden, and brief bouts of shooting or stabbing pain that follow the distribution of one or more divisions of the trigeminal nerve, affecting the patient's facial region.
419
What is the Epidemiology of Trigeminal Neuralgia?
* Trigeminal neuralgia typically affects adults over the age of 50 * Higher prevalence in women.
420
What is the Aetiology of Trigeminal Neuralgia?
**Primary (idiopathic)** **Secondary:** * **Malignancy:** can lead to nerve compression or infiltration, resulting in pain * **Arteriovenous malformation:** abnormal, tangled blood vessels can compress the trigeminal nerve * **Multiple sclerosis:** demyelination in this condition can affect the trigeminal nerve * **Sarcoidosis:** granulomatous lesions can affect the trigeminal nerve * **Lyme disease:** infection and subsequent inflammation can affect the trigeminal nerve
421
What are the clinical features of Trigeminal Neuralgia?
* Unilateral facial pain that is sudden, severe, and brief. * Pain is felt in the divisions of the Trigeminal nerve * The pain is often described as shooting or stabbing, and can be triggered by lightly touching the affected side of the face, eating, or wind blowing on the face. * Neurological examination in these patients is typically normal.
422
What are some differential Diagnoses for Trigeminal Neuralgia?
* **Postherpetic neuralgia:** presents with persistent pain following the resolution of a shingles rash. The pain is typically continuous, aching, burning, or throbbing. * **Temporomandibular joint disorders:** presents with pain in the jaw joint and muscles controlling jaw movement. Other symptoms include difficulty chewing and joint locking. * **Giant cell arteritis:** presents with headache, scalp tenderness, jaw pain, and visual symptoms. Other symptoms include fatigue, loss of appetite, and fever. * **Cluster headache:** presents with severe, unilateral headaches occurring in clusters. Other symptoms include eye watering, nasal congestion, and ptosis on the affected side.
423
What are the investigations for Trigeminal Neuralgia?
* Trigeminal neuralgia is largely a clinical diagnosis, but investigations may be performed to rule out other causes of facial pain. * Neuroimaging such as MRI can be used to exclude secondary causes, including tumors or vascular compression.
424
What is the management of Trigeminal Neuralgia?
**Medical management:** * **Carbamazepine** (first-line treatment) * Phenytoin * Lamotrigine * Gabapentin **Surgical management includes:** * Microvascular decompression: a procedure to remove or relocate blood vessels that are in contact with the trigeminal root * Treatment of the underlying cause: such as removing a tumour or addressing an arteriovenous malformation * Alcohol or glycerol injections: used to damage the trigeminal nerve and reduce pain signals
425
What are some key Red flags associated with headaches?
* Fever, photophobia or neck stiffness (meningitis, encephalitis or brain abscess) * New neurological symptoms (haemorrhage or tumours) * Visual disturbance (giant cell arteritis, glaucoma or tumours) * Sudden-onset occipital headache (subarachnoid haemorrhage) * Worse on coughing or straining (raised intracranial pressure) * Postural, worse on standing, lying or bending over (raised intracranial pressure) * Vomiting (raised intracranial pressure or carbon monoxide poisoning) * History of trauma (intracranial haemorrhage) * History of cancer (brain metastasis) * Pregnancy (pre-eclampsia) * **Fundoscopy showing Papilloedema** suggests raised ICP due to either brain tumour, benign intracranial hypertension or an intracranial bleed
426
Define Diabetic Neuropathy?
Diabetic peripheral neuropathy (DPN) represents a spectrum of peripheral nerve disorders stemming from diabetes. The central driver behind their development is believed to be chronic hyperglycaemia.
427
What is the epidemiology of diabetic neuropathy?
DPN is a common complication of both type 1 and type 2 diabetes, with approximately 50% of long-term diabetic patients developing the condition. The risk increases with the duration of diabetes and poor glycaemic control.
428
What is the aetiology of diabetic neuropathy?
Chronic hyperglycaemia in diabetes is the primary cause of DPN, leading to damage to peripheral nerves through various mechanisms: * Accumulation of advanced glycation end products * Oxidative stress * Inflammatory pathways.
429
What are the different categories of diabetic peripheral neuropathy?
* Distal Symmetrical Sensory Neuropathy * Small-fibre Predominant Neuropathy * Diabetic Amyotrophy * Mononeuritis Multiplex * Autonomic Neuropathy
430
What is the most common type of diabetic peripheral neuropathy?
Distal Symmetrical Sensory Neuropathy
431
How does Distal Symmetrical Sensory Neuropathy present?
* Resulting from loss of large sensory fibres. * Presents with sensory loss in a 'glove and stocking' distribution, typically affecting touch, vibration and proprioception.
432
How does Small-fibre Predominant Neuropathy present?
* Due to the loss of small sensory fibres. * Manifests as **deficits in pain and temperature sensation**in a 'glove and stocking' distribution, often accompanied by episodes of burning pain.
433
How does Diabetic Amyotrophy present?
* Originates from inflammation of the lumbosacral plexus or cervical plexus. * Characterised by **severe pain around the thighs and hips**, along with proximal weakness.
434
How does Mononeuritis Multiplex present?
* Typically painful. * Defined as neuropathies involving two or more distinct peripheral nerves.
435
How does Autonomic Neuropathy present?
Presents with postural hypotension, gastroparesis, constipation, urinary retention, arrhythmias, and erectile dysfunction.
436
What are some differential diagnoses to diabetic peripheral neuropathy?
* **Vitamin B12 deficiency:** Can present with peripheral neuropathy, typically in a glove and stocking distribution. May also feature megaloblastic anaemia and glossitis. * **Alcohol-induced peripheral neuropathy:** Presents similarly to DPN but may also have accompanying signs of chronic alcohol misuse. * **Chronic Inflammatory Demyelinating Polyneuropathy (CIDP):** Often presents with both sensory loss and motor weakness, typically in a proximal and distal distribution. * **Hypothyroidism:** Can present with neuropathy, usually accompanied by other symptoms such as fatigue, weight gain, and cold intolerance.
437
What are the investigations for diabetic peripheral neuropathy?
* **Neurological examination:** To assess the extent of sensory and motor deficits. * **Nerve conduction studies:** To evaluate the nature and extent of neuropathy. * **Blood tests:** Including glucose levels, HbA1c, B12 levels, thyroid function tests, and liver function tests, to identify potential differential diagnoses or contributory conditions.
438
What are some complications of diabetic peripheral neuropathy?
* Foot ulcers * Cardiac, GI and genitourinary disturbances due to autonomic neuropathy
439
What is the management of Diabetic peripheral neuropathy?
**Control of blood glucose levels to slow disease progression** Pain control: **Amitriptyline, Gabapentin, Pregabalin, Duloxetine** * If first line doesn't work then try one of the other 3 drugs
440
What is Charcot Arthropathy?
Charcot arthropathy, also known as Charcot joint or neuropathic arthropathy, is a chronic, progressive condition characterised by painful or painless bone and joint destruction in the limbs that have lost sensory innervation. The condition primarily affects patients with peripheral neuropathy.
441
What is the aetiology of Charcot Arthropathy?
Charcot arthropathy is primarily neuropathy. The most common cause of this is **diabetes mellitus**, which leads to microvascular disease, autonomic neuropathy, and peripheral neuropathy, resulting in cumulative damage to the joints. Other: * Syringomyelia * Chronic alcohol abuse * Syphilis (Tabes Dorsalis)
442
What are the clinical features of Charcot Arthropathy?
Charcot arthropathy often presents with the '**6Ds**'(some of which are imaging features): **Destruction of bone and joint Deformity Degeneration Dense bones Debris of bone fragments Dislocation** It classically affects the **tarsometatarsal joints**, but it can involve any joint in a limb that has lost sensation due to neuropathy.
443
What is the main differential diagnosis to rule out in Charcot Arthropathy?
Osteomyelitis Will typically also cause systemic symptoms and increase inflammatory markers
444
What are the investigations for Charcot Arthropathy?
**Clinical Diagnosis** * X-rays are usually the first-line imaging study. They can demonstrate bone destruction, debris, sclerosis (dense bones), and dislocation. * MRI can provide more detailed imaging, particularly in early disease or when osteomyelitis is suspected. * Bone scans may be used in complex cases or when other imaging is inconclusive.
445
What is the conservative management of Charcot Arthropathy?
* Prolonged off-loading, often involving special footwear or plaster casts, to allow healing and prevent further damage. * Use of orthotics for long-term management and prevention of recurrences.
446
What is the medical management for Charcot Arthropathy?
* Bisphosphonates can help slow down the process of bone destruction. * Neuropathic pain agents, such as gabapentin or pregabalin, for pain management. * Topical anaesthetics can also be used to manage pain.
447
What is the surgical management of Charcot Arthropathy?
* Resection of bony prominences to prevent ulcers or improve fitting of footwear. * Correction of severe deformities, usually after the acute phase has settled. * Amputation may be required in severe cases or when there is a concurrent uncontrolled infection.
448
Define Meniere's Disease?
Ménière’s disease is a long-term inner ear disorder that causes recurrent attacks of vertigo, and symptoms of hearing loss, tinnitus and a feeling of fullness in the ear It is caused by a build up of endolymph in the labyrinth of the inner ear
449
What is the Pathophysiology of Meniere's Disease?
* The excessive build-up of endolymph in the labyrinth of the inner ear * Causes a higher pressure than normal and disrupting the sensory signals. * This increased pressure of the endolymph is called endolymphatic hydrops.
450
What is the epidemiology of Meniere's disease?
* Individuals between 30-60 * Predominantly affects only one ear
451
What is the Aetiology of Meniere's Disease?
Exact cause is **Unknown** Believed to be associated with the dilation of the endolymphatic spaces of the membranous labyrinth. This dilation leads to an increased fluid pressure within the inner ear, causing the characteristic symptoms of the disease.
452
What is the classical triad of symptoms caused by Meniere's disease?
**Recurrent episodes of:** * Hearing loss * Vertigo (most prominent symptom) * Tinnitus
453
What is the clinical presentation of Meniere's disease?
30-60yrs old with Unilateral episodes of: * **Vertigo:** Usually lasts for 20 mins to a few hours before settling. Episodes come in clusters over several weeks with periods (Months) of no vertiginous symptoms. **Vertigo is NOT triggered by movement** * **Hearing Loss:** unilateral sensorineural loss that fluctuates at first and gradually becomes more permanent. Affects **low frequencies first**. * **Tinnitus:** occurs with episodes of vertigo before becoming more permanent. It is usually unilateral **Other symptoms:** * Sensation of fullness in the ear * Unexplained falls (**Drop attacks**) without LOC * Imbalance that can persist after vertiginous episodes have resolved * Nausea and vomiting
454
What are some differential diagnoses for Meniere's disease?
* **Vestibular neuritis:** Characterized by sudden onset vertigo, nausea, vomiting, and unsteadiness * **Labyrinthitis:** Presents with vertigo, hearing loss, and tinnitus * **Benign paroxysmal positional vertigo (BPPV):** Characterized by brief episodes of mild to intense dizziness triggered by specific changes in the position of the head
455
What are the investigations for Meniere's disease?
**Clinical Diagnosis usually made by an ENT specialist** * Examination (Rinnes and Webers Tests) * Audiometric testing * Other imaging or laboratory tests may be used to rule out other potential causes of symptoms
456
What is the management for Meniere's Disease? Prophylaxis Acute attacks
* Prophylactic use of **Betahistine and vestibular rehabilitation** to reduce the frequency of attacks * Acute use of **prochlorperazine** to manage symptoms during attacks * Surgical approaches are available, but currently lack a strong evidence base.
457
What is the definition of Delirium?
It's an acute and fluctuating disturbance in attention and cognition. Often accompanied by a change in consciousness. It is typically reversible and frequently seen in the elderly.
458
What is the epidemiology of Delirium?
The prevalence increases with age, severity of illness and the presence of pre-existing cognitive impairment.
459
# PHONED CHIMPS What are the main causes of Delirium?
Pain Hypothermia/Pyrexia Organ dysfunction (renal or hepatic impairment) Nutrion Environmental changes Drugs and alcohol (OTC, illicit, Anti-cholinergics, Opiates, Anti-convulsants) Constipation Hypoxia Infection Metabolic disturbance (Electrolyte imbalance, Endocrinological issues, Wernicke’s Encephalopathy) Prescriptions Sleep deprivation
460
# Where THE F AM I What is the clinical presentation of Delirium?
Where: Disorientation Thought disorganisation Hallucinations Energy Changes: Can by hyperactive or hypoactive Fluctuating Acute and Altered sleep Memory problems Inattention
461
What are some differential diagnoses of Delirium?
Dementia Psychosis Depression Stroke
462
What initial investigations should be done for Delirium?
* Comprehensive physical examination and infection screen * Confusion Screen (including bloods and urinalysis) * Imaging (possibly Head CT/MRI, Chest XRay or ultrasound of the abdomen depending on scenario) * ECG
463
What is the non-pharmacological management of Delirium?
* Providing an environment with good lighting * Maintaining a regular sleep-wake cycle * Regular orientation and reassurance * Ensuring the patient's glasses and hearing aids are used if needed
464
What pharmacological management can be used for delirium?
**Haloperidol 0.5mg** is first line To be used with caution and only in patients who are extremely agitated and present a danger to themselves or others
465
What blood tests are involved in Confusion Screen?
* FBC (infection, anaemia, malignancy) * U&Es (hyponatraemia, hypernatraemia) * LFTs (liver failure with secondary encephalopathy) * Coagulation/INR (intracranial bleeding) * TFTs (hypothyroidism) * Calcium (hypercalcaemia) * B12 + folate/haematinics (B12/folate deficiency) * Glucose (hypoglycaemia/hyperglycaemia) * Blood cultures (sepsis)
466
Define Chronic Fatigue Syndrome (CFS)
Also known as **Myalgic Encephalomyelitis (ME)** CFS is a chronic, disabling condition that significantly impacts productivity. It is characterised by profound fatigue and impairment following minimal physical or cognitive effort.
467
What gender is more commonly affected by CFS?
Women more than men
468
What is the aetiology of CFS?
Exact cause remains unclear but potential triggers: * Viral and bacterial infections (EBV etc) * Genetic predisposition * Environmental influences * Psychological stress
469
What are the clinical features of CFS?
* Extreme fatigue * Post-exertional malaise * Sleep disturbances: Insomnia, Hypersomnia, Unrefreshing sleep * Cognitive impairment
470
What are some differential diagnoses for Chronic Fatigue Syndrome?
* **Fibromyalgia:** Characterized by widespread musculoskeletal pain, fatigue, and mood and sleep disturbances. Unlike CFS, exertional exhaustion is less pronounced. * **Major depressive disorder:** Features persistent feelings of sadness, loss of interest or pleasure in activities, and fatigue. However, it lacks the post-exertional malaise seen in CFS. * **Hypothyroidism:** Presents with fatigue, weight gain, cold intolerance, and depression. Distinguished from CFS by the presence of abnormal thyroid hormone levels.
471
What are the investigations for CFS?
**Clinical diagnosis** based on the patients history and symptoms persisting for >3 months **Blood Tests to rule out other pathology** * FBC * U&E * LFT * TFT * Glucose * ESR/CRP * Calcium * CK * Iron studies * Coeliac Screen
472
What is the management of CFS?
**MDT Approach** * Referral to PT, OT, social care to help guide disease **Energy Management:** * Self management strategy to manage activities within their energy limits * Establish baseline of achievable exercise and physical activity * "Pacing" self management for activities **Cognitive behavioural therapy (CBT):** A form of talk therapy that can help patients manage their symptoms. **Symptom control:** This may involve medications for pain, sleep disturbances, and other co-existing conditions.
473
Define Wernicke's Encephalopathy
Wernicke's encephalopathy is a neurological disorder caused by thiamine (vitamin B1) deficiency manifesting in a triad of specific clinical symptoms: **ataxia, confusion, and ocular abnormalities.**
474
What are the primary causes of Wernicke's Encephalopathy?
* **Chronic alcohol abuse:** Alcohol interferes with thiamine absorption and utilization. * **Malnutrition:** This can occur due to inadequate dietary intake, malabsorption disorders, or increased requirements. * **Bariatric surgery:** Rapid weight loss and reduced nutrient absorption can lead to thiamine deficiency. * **Hyperemesis gravidarum:** Persistent severe vomiting in pregnancy may lead to nutrient deficiencies, including thiamine.
475
What are the clinical features of Wernicke's Encephalopathy? What are the features of Korsakoff Syndrome?
**Wernicke’s Encephalopathy (COAT)** * Confusion * Ophthalmoplegia and Nystagmus * Ataxia * Thiamine Deficiency **Korsakoff Syndrome (RACK)** * Retrograde and anterograde amnesia * Altered temper/behavioural changes * Confabulation * Korsakoff Psychosis
476
What are some differential diagnoses for Wernicke's Encephalopathy?
* **Meningitis:** Presents with fever, headache, neck stiffness, and altered mental status. * **Stroke:** Sudden onset of focal neurological deficits, which may include difficulty speaking, face drooping, arm weakness. * **Encephalitis:** Characterized by fever, headache, behavioural changes, and sometimes, seizures. * **Korsakoff's syndrome:** Notable for severe anterograde and retrograde memory loss, often seen as a progression from untreated Wernicke's encephalopathy.
477
What are the investigations for Wernicke's Encephalopathy?
**Clinical Diagnosis** Investigations that may be done: * Blood tests: Thiamine levels, BMs, LFTs, * Neurological examination: Assessment of the characteristic triad of symptoms. * MRI Head: May show characteristic changes in specific brain regions, such as the mammillary bodies and periaqueductal area.
478
What is the management of Wernicke's Encephalopathy?
**IV Pabrinex** (High dose thiamine replacement)
479
What is the primary complication of Wernicke's Encephalopathy?
**Korsakoff Syndrome:** Wernicke's if left untreated will lead to damage to the mammillary bodies and cause irreversible anterograde and retrograde memory impairment. It will also cause behavioural changes
480
Define Narcolepsy
A chronic neurological disorder characterised by **disruption of the sleep wake cycle and REM sleep intrusion** leading to excessive daytime sleepiness
481
What is the pathophysiology of Narcolepsy?
Loss of **hypocretin producing neurons** in the hypothalamus leads to the dysregulation of wakefulness and sleep.
482
What is the epidemiology of Narcolepsy?
* It is a relatively rare condition * The second most common cause of disabling daytime sleepiness after OSA * Slight male predominance * Peak incidence is 20-30 years old
483
What is the Aetiology and some Risk factors for Narcolepsy?
Unknown Aetiology **Risk Factors:** * Low CSF Hypocretin * HLA DQB1 * Prader-Willi Syndrome * Neimann-Pick Disease
484
What are the clinical features of Narcolepsy?
Tetrad of: * **Excessive Daytime Sleepiness (Hypersolmnence):** Uncontrollable urge to sleep during the day, resulting in sudden and involuntary episodes of sleep known as 'sleep attacks. * **Cataplexy:** Sudden loss of muscle tone triggered by strong emotions such as laughter, anger, or surprise, leading to partial or complete collapse without loss of consciousness. * **Sleep Paralysis:** Transient inability to move or speak upon awakening from sleep or while falling asleep * **Hypnagogic/Hypnopompic Hallucinations:** Hypnagogic hallucinations occur during the transition from wakefulness to sleep, while hypnopompic hallucinations take place upon awakening from sleep. These vivid and often frightening sensory experiences may involve visual, auditory, or tactile sensations
485
What are some other effects on the patient caused by Narcolepsy?
Chronic fatigue and tiredness Poor performance at work Poor memory and concentration Car accidents
486
What are the investigations for Narcolepsy?
**1st Line:** * Acitgraphy and sleep diary * Overnight Polysomnography * Multiple Sleep Latency EEG Tests (MSLT)
487
What investigation may be indicated if Polysomnography and MSLT are unequivocal in investigating Narcolepsy?
**Cerebrospinal Fluid Hypocretin-1 Level:** Deficiency of this is firmly established as a cause of Narcolepsy type 1 and is a diagnostic marker
488
What is the conservative management of Narcolepsy?
**Sleep hygiene and Lifestyle changes** * Strict sleep schedule * Avoid sleep deprivation * Avoid alcohol, caffeine and smoking * Avoid late night exercise
489
What is the Medical management of Narcolepsy?
**Treatment of Excessive daytime sleepiness (EDS):** * CNS stimulants: **Modafinil, Pitolisant, Sodium Oxybate** **Treatment of Cataplexy:** * **1st Line:** Sodium Oxybate * **2nd Lie:** SNRIs (venlaflaxine) /SSRIs/TCAs
490
Define Myasthenia Gravis
Myasthenia gravis is an autoimmune disease marked by the production of antibodies that target the nicotinic acetylcholine receptors on muscle fibres. This immune-mediated interference reduces the ability of acetylcholine to trigger muscle contraction, resulting in muscle weakness.
491
What is the Epidemiology of Myasthenia Gravis?
Generally more common in Females Female peak (20-30yrs) - associated with autoimmune disease Male peak (50-60yrs) - associated with Thymoma
492
What type of Hypersensitivity reaction is Myasthenia Gravis?
Type 2
493
What are the risk factors for MG?
Females FHx Autoimmunity Thymoma/ Thymic Hyperplasia
494
What is the pathophysiology of MG?
**85% Anti-nACh Receptors:** * Bind to post synaptic receptor and competitively inhibit ACh binding. * ACh cannot bind during exertion and therefore there is progressive weakness of muscles * Auto-ABs Will also bind to complement factors and cause NMJ destruction **15% Anti MuSK:** * Inhibit MuSK from synthesising ACh Receptors so there is reduced expression on post synaptic membrane.
495
What are the clinical features of Myasthenia Gravis?
* Limb muscle weakness * Extra-ocular muscle involvement leading to drooping eyelids, diplopia * Facial muscle involvement causing difficulty in smiling or chewing * Bulbar muscle involvement causing a change in speech or difficulty swallowing * **Fatigable muscle weakness, bilateral ptosis, a myasthenic snarl, head droop, and bulbar features on examination**
496
Give some examples of drugs that can exacerbate Myasthenia Gravis?
**Pills likely to produce bad quality myasthenia gravis** Penicillamine, Lithium, Tetracycline Procainamide, Beta blockers, Quinolones, Macrolides, Gentamicin
497
What are some differential Diagnoses for Myasthenia Gravis?
* **Lambert-Eaton syndrome:** is a fluctuating weakness that improves with exercise, differentiating it from MG. This is usually due to underlying malignancy, most commonly small-cell lung cancer. It affects voltage-gated calcium channels in the presynaptic membrane. * **Brainstem gliomas:** are malignant tumours that present with bulbar symptoms, weakness, numbness, balance problems, and seizures depending on its location and structures affected. The symptoms are persistent and usually present with headaches and signs of increased intracranial pressure. * **Multiple sclerosis:** can present with any neurological sign that may fluctuate or persist over hours to days to weeks secondary to demyelination in the central nervous system. * **Botulism:** presents with ptosis, double vision, progressive weakness, and pupillary abnormalities accompanied by systemic symptoms. Ingestion of honey or contaminated foods may be elicited in the patient's history. * **Polymyositis and dermatomyositis:** cause proximal muscle weakness and is usually associated with pain. The pathology is the inflammation of the muscle itself. * **Graves ophthalmopathy:** presents with eyelid retraction and widened palpebral fissure. These are caused by autoantibodies targeted toward the structures of the eye.
498
What are the primary investigations for Myasthenia Gravis?
**1st Line:** * serum acetylcholine receptor antibody (AChR) * Serum Muscle-specific tyrosine kinase antibodies (MuSK) * Serial Pulmonary Function Test **Other Investigations** **Imaging investigations:** CT scan of the chest to identify thymic hyperplasia or thymoma **Nerve Conduction Studies/EMG:** Repetitive nerve stimulation testing leads to a decrement in evoked potential
499
What is the management of Myasthenia Gravis? Medical Mx 1st, 2nd, 3rd line Acute Crisis
Regular Medical Reviews MDT involvement. **Medical management:** * Acetylcholinesterase inhibitors: **Pyridostigmine is first line** * **Second Line:** Immunosuppressive Steroid therapy: **Prednisolone** * **Third Line:** Immunosuppressant: Azathioprine, Ciclosporin, Rituximab **Acute Crisis:** * Require intravenous immunoglobulin (IVIG) or plasmapheresis in severe, steroid-refractory, cases. **Surgical management:** * Thymectomy may be considered in patients with thymic hyperplasia or thymoma.
500
What is the main complication of MG?
Myasthenic Crisis: Severe acute worsening of Sx Often Triggered by another illness (URTI) Severe Respiratory weakness
501
What is the Treatment for Myasthenic Crisis?
IV Ig (immunoglobulin) and Plasmapheresis In a myasthenic crisis, **serial pulmonary function tests (spirometry) are performed**. If the forced vital capacity is 15 mL/kg or less, the patient should be considered for **mechanical ventilation**.
502
What is Lambert Eaton Syndrome?
A NMJ syndrome which has similar Sx to MG. Autoimmunity against VG-Ca channels thereby reducing ACh release at the NMJ causing muscle weakness.
503
What is the cause of Lambert Eaton Syndrome?
Unclear but likely a paraneoplastic syndrome: Typically occurs in Px with Small Cell Lung Cancer (SCLC)
504
What is the presentation of Lambert Eaton Syndrome?
Proximal muscle weakness that develops more slowly Sx start at extremities and progress towards the head Shares most of same Sx with MG
505
What is the difference between MG and Lambert Eaton Syndrome clinically?
Lambert Eaton Syndrome symptoms tend to improve following a period of strong muscle contraction. Post Tetanic Potentiation
506
What is the Treatment for Lambert Eaton Syndrome?
Dx and Tx underlying condition (often SCLC) Amifampridine - blocks K+ channels and increases ACh release + Steroids and Immunosuppressants
507
Define Neurofibromatosis
These are Autosomal Dominant genetic conditions that causes nerve tumours (neuromas) to develop throughout the nervous system. These tumours are benign but can cause neurological and structural problems.
508
What are the different types of Neurofibromatosis and the genetics involved?
Type 1 (NF1): Due to a loss of function mutation in the neurofibromin gene on chromosome 17 Type 2 (NF2): Due to a loss of function mutation in the Schwannomin (Merlin) tumour suppressor gene on chromosome 22 Type 3: Schwannomatosis
509
What is the epidemiology of Neurofibromatosis?
Type 1 is more common than type 2 NF1 is also known as **Recklinghausen's Disease**
510
What are the clinical features of Neurofibromatosis Type 1?
**Cutaneous features:** * **Cafe-au-lait spots:** Oval-shaped, coffee-coloured patches that continue to grow throughout life. Presence of 6 macules >5mm (or >15mm if post-pubertal) is a feature of NF1. * **Axillary or inguinal freckling** * **Neurofibromas:** Small nodular tumours in the skin. **Non-cutaneous features:** * **Lisch nodules:** Hamartomas on the iris appearing as brown patches/mounds, typically visible by age 6. * **Optic glioma** * **Scoliosis** and other bone malformations * **Phaeochromocytomas** * **Learning difficulties** * **Hypertension** * **Gastrointestinal issues:** Bleeding or obstruction due to tumours in the bowel. * **Epilepsy:** Due to tumours in the brain.
511
What are the clinical features of Neurofibromatosis Type 2?
* **Bilateral vestibular schwannomas (acoustic neuromas)**, causing sensorineural hearing loss, tinnitus, and vertigo. * Meningiomas * Spinal ependymomas * Posterior lens opacities * Cerebral calcification * Astrocytoma's * Glial hamartomas
512
What condition is this?
Neurofibromatosis
513
What is this cutaneous sign and what condition does it suggest?
Cafe-au-lait spot Suggests neurofibromatosis **May also suggest other conditions such as Familial Cafe au lait spots, McCune-Albright syndrome or Legius syndrome**
514
What are some differential diagnoses for neurofibromatosis?
* **Legius syndrome:** Similar to NF1 with cafe-au-lait spots and freckling but without neurofibromas or Lisch nodules. * **Segmental NF:** Similar to NF1 but symptoms are limited to one area of the body. * **McCune-Albright syndrome:** Cafe-au-lait spots with irregular borders, polyostotic fibrous dysplasia, and endocrine abnormalities. * **Other genetic syndromes:** Other genetic conditions can present with cafe-au-lait spots, including Bloom syndrome and Fanconi anemia.
515
What condition is particularly associated with neurofibromatosis type 2?
Acoustic neuromas. **A patient with Bilateral acoustic neuromas almost certainly has NF2**
516
What are the investigations for Neurofibromatosis?
**Genetic testing:** To confirm mutations in the NF1 or NF2 genes. **Neuroimaging:** To detect the presence of tumours or other brain abnormalities. **Slit lamp examination:** To identify Lisch nodules in NF1.
517
How is Neurofibromatosis diagnosed?
**Clinical diagnosis** Genetic testing may be used to confirm the diagnosis
518
What is the Diagnostic criteria for a clinical diagnosis of Neurofibromatosis type 1?
**CRABBING** **C**afé-au-lait spots (more than 15mm diameter is significant in adults) **R**elative with NF1 **A**xillary or inguinal freckling **B**ony dysplasia, such as **B**owing of a long bone or sphenoid wing dysplasia **I**ris hamartomas (**Lisch nodules**), which are yellow-brown spots on the iris **N**eurofibromas **G**lioma of the optic pathway
519
What is a neurofibroma? What neurofibromas are significant?
A type of peripheral nerve tumor that forms soft bumps on or under the skin * A single skin neurofibroma without other features does not indicate neurofibromatosis. * A plexiform neurofibroma is a larger, irregular, complex neurofibroma containing multiple cell types. * **Two or more are significant.** * **A single plexiform neurofibroma is significant.**
520
What is the management of Neurofibromatosis?
There is no treatment for NF and the disease is progressive. Management focuses on monitoring, managing symptoms and treating complications **Surveillance:** Regular monitoring for new symptoms or complications. **Symptomatic treatment:** Management of hypertension, epilepsy, or other complications as they arise. **Surgical intervention:** Removal of tumours or other interventions may be necessary in some cases.
521
What are some complications of Neurofibromatosis?
* Migraines * Epilepsy * **Malignant peripheral nerve sheath tumours (MPNST)** * **Gastrointestinal stromal tumour (a type of sarcoma)** * Renal artery stenosis, causing hypertension * Learning disability * Behavioural problems (e.g., ADHD) * Scoliosis of the spine * Vision loss (secondary to optic nerve gliomas) * Brain tumours * Spinal cord tumours with associated neurology (e.g., paraplegia) * Increased risk of cancer (e.g., breast cancer and leukaemia)
522
Define Normal Pressure Hydrocephalus (NPH)
A neurological disorder in which excess cerebrospinal fluid accumulates in the brain's ventricles, causing them to enlarge. Despite the term "normal pressure," the fluid causes pressure effects leading to characteristic symptoms. **However, the cerebrospinal fluid (CSF) pressure often appears normal on lumbar puncture, hence the term.**
523
What is the Epidemiology of NPH?
More common in older adults Considered one of the potentially reversible causes of dementia
524
What is the Aetiology of NPH?
Unclear: Associated with conditions that block the flow or absorption of CSF causing its accumulation such as: * SAH * Meningitis * Head injury * Surgical Complications
525
# Wet, Wacky, Wobbly What are the clinical features of NPH?
Classical triad: Wet, Wacky, Wobbly * **Dementia:** Often manifests as global cognitive impairment, with attention and memory disturbances. * **Magnetic gait:** Characterized by difficulty in lifting the feet off the floor, appearing as if they are "stuck." * **Incontinence:** Primarily urinary incontinence, but faecal incontinence can also occur.
526
What are some differential diagnoses for NPH?
* **Alzheimer's disease:** Presents with progressive memory loss, confusion, language difficulties, and mood changes. Unlike NPH, motor disturbances are usually not seen until late stages. * **Parkinson's disease:** Features bradykinesia, rigidity, resting tremor, and postural instability. Cognitive impairment may occur but is not an early feature as in NPH. * **Other forms of dementia:** Depending on their specific type, can present with varying cognitive and motor symptoms.
527
What are the investigations for NPH?
**Neuroimaging:** * CT/MRI shows dilated lateral ventricles and enlarged 4th ventricle * Hydrocephalus with Ventriculomegaly **Lumbar Puncture**
528
What is the management for NPH?
Relieve the pressure effects caused by excess CSF **Therapeutic lumbar puncture:** This procedure can alleviate symptoms and improve cognition and walking ability by removing CSF. **Ventriculoperitoneal shunt:** In patients responsive to lumbar puncture, neurosurgery may insert a shunt to permanently redirect the excess CSF from the brain to the abdomen.
529
What are some complications of a Ventriculoperitoneal shunt?
Seizures Infection Blockage Excessive drainage **Intraventricular haemorrhage** during shunt related surgery
530
What are the high yield features of: Myelopathy: Anterior Cord Syndrome Posterior Cord Syndrome Brown Sequard Cauda Equina
**Myelopathy:** (Cord compression) * LMN at the lesion as nerve root is compressed * UMN below the lesion **Anterior Cord Syndrome** * B/L motor weakness and B/L loss of pain and temp * DCML/proprioception is spared * MND ASAI can cause it **Posterior Cord** * DCML loss - proprioception **Brown Sequard** * Ipsilateral loss of corticospinal and DCML * Contralateral loss of Spinothalamic 2 spinal segments below **Cauda Equina** * LMN only
531
Define Meralgia Paraesthetica?
A neurological condition (**mononeuropathy**) characterized by compression of the **lateral femoral cutaneous nerve**, a purely sensory nerve supplying the **outer aspect of the thigh**. The primary feature of this syndrome is **neuropathic pain**
532
What is the epidemiology of Meralgia Paraesthetica?
* Middle age (30-60 years) * No significant gender predilection
533
What are some risk factors for Meralgia Paraesthetica?
Anything that increases pressure on the grain area: * Obesity * Pregnancy * Diabetes * Wearing tight belts or clothing * Trauma * Iatrogenic
534
What is the Aetiology of Meralgia Paraesthetica?
* Compression or entrapment of the lateral femoral cutaneous nerve under the inguinal ligament. * Direct injury to the nerve. * Conditions or factors that increase pressure on the nerve
535
Where does the Lateral Femoral Cutaneous nerve originate?
Varying combinations of L1, L2 and L3 nerve roots. It only carries sensory innervation to the upper outer thigh so there are no motor symptoms in Meralgia Paraesthetica
536
What are the clinical features of Meralgia Paraesthetica?
* Burning, tingling, coldness, or shooting pain * Numbness * Deep muscle ache * Symptoms are usually aggravated by standing, and relieved by sitting * They can be mild and resolve spontaneously or may severely restrict the patient for many years. **Sensory loss may occur but motor function is unaffected.**
537
What are the signs of meralgia Paraesthetica?
* Symptoms may be reproduced by deep palpation just below the ASIS (pelvic compression) and also by extension of the hip. * There is altered sensation over the upper lateral aspect of the thigh. * **There is no motor weakness.**
538
What are some differential diagnoses for meralgia Paraesthetica?
* **Lumbar radiculopathy:** Characterized by back pain that radiates down the leg, weakness, numbness, or difficulty controlling specific muscles. * **Hip arthritis:** Symptoms include pain in the hip and groin, stiffness, and reduced range of motion. * **Iliotibial band syndrome:** This presents with lateral knee pain, especially with activity, and tenderness over the lateral femoral epicondyle. * **Diabetic neuropathy:** Primarily presents with distal symmetrical neuropathy, numbness, tingling, or pain in the hands or feet.
539
What are the investigations for Meralgia Paraesthetica?
Clinical Diagnosis based on **Pelvic compression test examination** * **Ultrasound can be effective for diagnosis** * **Nerve conduction studies and electromyography (EMG):** To rule out other causes of neuropathy.
540
What is the conservative management of Meralgia Paraesthetica?
Rest Looser clothing (tight clothes such as belts may add pressure to the nerve) Weight loss (if appropriate) Physiotherapy
541
What is the medical management of Meralgia Paraesthetica?
Paracetamol NSAIDs Neuropathic analgesia (e.g., amitriptyline, gabapentin, pregabalin or duloxetine) Local injections of steroids or local anaesthetics
542
What is the surgical management of Meralgia Paraesthetica?
Decompression – removing pressure on the nerve Transection – cutting the nerve Resection – removing the nerve
543
Define Spinal Cord Compression (SCC)
A condition that results from processes causing compression or displacement of the arterial, venous, and cerebrospinal fluid spaces, as well as the spinal cord itself. This condition leads to various neurological symptoms and disturbances based on the level and severity of compression.
544
What is the epidemiology of SCC?
Common neurosurgical condition Often seen in patients with a history of malignancy
545
What is the Aetiology of SCC?
* **Trauma:** Can lead to fractures or dislocations that compress the spinal cord. * **Neoplasia:** Seen in 5-10% of cancer patients, it is a presenting complaint in 20% of these cases. * **Infection:** Particularly tuberculosis (TB) in at-risk patients can cause SCC. * **Disc Prolapse:** Protrusion of an intervertebral disc can compress the spinal cord. * **Epidural Haematoma:** Accumulation of blood in the epidural space can compress the spinal cord.
546
What are the clinical features of Spinal Cord Compression?
* **Upper motor neuron signs:** Such as hyperreflexia, spasticity, and a positive Babinski's sign. * **Sensory disturbance:** Typically below the level of the lesion. * Deep and localized back pain. * **Radicular sensory disturbance:** A stabbing sensation at the level of the lesion. * **Bladder and bowel involvement:** This can manifest as incontinence or retention.
547
What are some differential diagnoses for Spinal Cord Compression?
* **Multiple Sclerosis:** Presents with optic neuritis, limb weakness, sensory loss, ataxia, and bladder dysfunction. * **Transverse Myelitis:** Characterized by acute or subacute development of lower limb weakness, sensory disturbance, and sphincter dysfunction. * **Acute disseminated encephalomyelitis:** Presents with fever, malaise, headache, vomiting, ataxia, and changes in consciousness. * **Peripheral Neuropathy:** Symptoms include sensory loss, pain, and weakness in the limbs. * **Musculoskeletal back pain:** Presenting as severe pain, spasms with no significant weakness
548
What are the investigations for Spinal Cord Compression?
**Urgent whole spine MRI done in anyone with features suggestive of SCC or Cauda Equina Syndrome (CES) within 48 hours**
549
What is the management of SCC?
**Surgical decompression:** This should be performed urgently, typically **within 48 hours**. **Administration of dexamethasone:** Indicated in patients with demonstrated malignancy on MRI or those with high clinical suspicion, * Given at 16 mg daily in divided doses, along with proton pump inhibitors (PPI)
550
Define Cauda Equina Syndrome (CES)
Compression of the cauda equina, the "horse's tail" in Latin is a surgical emergency. The term describes the bundle of Lumbo-Sacral nerve roots that extend from the termination of the spinal cord at the L1 level and exit the spinal column in the lower lumbar and sacral regions.
551
What is the Aetiology of CES?
The most common cause of cauda equina syndrome is **lumbar disc herniation at the L4/5 and L5/S1 levels**. **Other causes include:** * Neoplasms (metastatic or primary) * Abscesses * Trauma * Spondylolisthesis * Iatrogenic causes (e.g., manipulation, spinal anaesthesia, post-operative haematoma)
552
What are the clinical features of Cauda Equina Syndrome?
* Lower back pain * Lower motor neurone signs * Alternating or bilateral radicular (sciatic) pain * **Saddle anaesthesia,** often manifesting as an inability to feel toilet paper when wiping * **Bladder and bowel disturbances** which may manifest as either constipation/retention or incontinence
553
What areas do the nerves of the cauda equina supply?
* Sensation to the lower limbs, perineum, bladder and rectum * Motor innervation to the lower limbs and the anal and urethral sphincters * Parasympathetic innervation of the bladder and rectum
554
What are the key red flags to look out for suggestive of cauda equina?
* Saddle anaesthesia (loss of sensation in the perineum – around the genitals and anus) * Loss of sensation in the bladder and rectum (not knowing when they are full) * Urinary retention or incontinence * Faecal incontinence * Bilateral sciatica * Bilateral or severe motor weakness in the legs * Reduced anal tone on PR examination
555
What are some differential diagnoses for CES?
* **Spinal stenosis:** Characterised by lower back pain, radicular leg pain, neurogenic claudication, and in severe cases, bowel and bladder dysfunction. * **Sciatica:** Typically presents with lower back pain radiating along the sciatic nerve in the back of the leg, but usually without bowel and bladder disturbances. * **Discitis:** Back pain is a common feature but is typically accompanied by fever and elevated inflammatory markers rather than by radicular pain or bowel and bladder disturbances.
556
What is the **Gold standard** investigation for CES?
Urgent **Whole spine MRI** to identify the cause and location of the compression
557
What is the management of Cauda Equina Syndrome?
**Surgical decompression** within 48 hours **If malignancy is identified or clinically suspected then Dexamethasone 16mg daily in divided doses (with PPI cover)**
558
Define Anterior Cord Syndrome
Anterior cord syndrome is an incomplete cord syndrome that predominantly affects the anterior 2/3 of the spinal cord, characteristically resulting in motor paralysis below the level of the lesion as well as the loss of pain and temperature at and below the level of the lesion caused by ischaemia or infarction of the anterior spinal artery.
559
How can cauda equina syndrome be differentiated between metastatic spinal cord compression based on the clinical features?
Cauda equina presents with lower motor neuron signs (reduced tone and reduced reflexes). The nerves being compressed are lower motor neurons that have already exited the spinal cord. When the spinal cord is being compressed higher up by metastatic spinal cord compression, upper motor neuron signs (increased tone, brisk reflexes and upping plantar responses) will be seen.
560
What are the progression of symptoms in CES?
**Acute:** Sudden onset, rapidly progressing symptoms which worsen over several hours or days **Chronic:** insidious onset with slow progression of symptoms * Most patients will first notice lower back pain, this may present with or without sciatica. In many with slow onset CES, this may be the only symptom present for many days or weeks. Saddle anaesthesia often manifests soon after the back pain, but may not be noticed until the patient uses the bathroom and wipes that area. * Later symptoms of CES include lower limb weakness and urinary and bowel dysfunction, which can occur constantly or intermittently depending upon the cause of compression. Typically urinary retention and a reduced urge to urinate occurs first, with urinary overflow incontinence developing as a late sign of CES.
561
What are the different classifications of CES?
**Cauda equina syndrome with retention (CESR):** 50-60% of patients * Presents with established urinary retention and/or overflow incontinence **Incomplete cauda equina syndrome (CESI):** 40-50% of patients * Presents without urinary retention or overflow incontinence. Patients may have reduced bladder sensation, loss of desire to void and/or poor urinary stream * CESI has a better prognosis
562
What is an Anterior spinal artery infarction (ASAI)?
An ischemic stroke occurring in the anterior two-thirds of the spinal cord, typically due to disruption in the blood flow from the anterior spinal artery.
563
What is the aetiology of an ASAI?
* **Damage to the aorta** * Aortic aneurysm repair * Aortic dissection * Atherosclerosis * Cardiac arrest * Cardiac emboli * Vasculitis * Shock
564
What is the epidemiology of an ASAI?
It typically affects the elderly, and the prevalence tends to be higher in individuals with atherosclerotic disease, arterial hypertension, diabetes mellitus, or those undergoing surgical procedures on the aorta.
565
What are the clinical features of an ASAI?
**Affects Lateral corticospinal tracts AND Lateral spinothalamic tracts** * Bilateral Loss of pain sensation (analgesia) * Bilateral Loss of temperature sensation (thermoanaesthesia) * Bilateral Motor function impairment (spastic paresis) * Loss of autonomic functions
566
What are some differential diagnoses for ASAI?
* **Transverse myelitis:** Presents with bilateral motor, sensory, and autonomic spinal cord dysfunction. Acute or subacute onset is common. * **Acute myelopathy:** It also presents with similar symptoms, but it usually has a rapid onset and may also show signs of brain involvement. * **Spinal cord compression:** Features include pain, progressive motor and sensory loss, and autonomic dysfunction, including bowel and bladder dysfunctions. * **Spinal cord tumours:** Usually present with localized pain, weakness, sensory loss, and bowel or bladder dysfunction.
567
What are the investigations for ASAI?
**MRI scanning:** This is the investigation of choice, typically showing **hyperintense signals on T2-weighted images** in the anterior two-thirds of the spinal cord.
568
What are some complications of an ASAI?
Persistent neurological deficits Chronic pain Urinary and bowel dysfunction Pressure sores from prolonged immobility
569
What is the management of an ASAI?
Management of ASAI is largely supportive and aims to treat the underlying cause of the infarction. This may involve surgical interventions in cases of aortic aneurysm or dissection. **Control of Complications:** * Pain control * Physiotherapy for motor function improvement * Management of autonomic dysfunctions * Skin care for preventing pressure sores
570
What is the length of the spinal cord?
From C1-L1/2
571
Where is the conus medullaris and cauda equina?
At L1/2
572
Define Hemiplegia?
Paralysis to one side of the body (usually due to a brain lesion)
573
Define Paraplegia?
Paralysis of both legs/lower body (usually due to a spinal cord lesion)
574
What does the DCML tract convey information for?
Ascending tract for fine touch, 2pt discrimination, vibration and proprioception.
575
What is the pathway for the DCML
Travels in dorsal route (Fasciculus Gracilis/Cuneatus) Decussates in the medulla
576
What does the spinothalamic tract convey information for?
Ventral: Crude Touch and pressure Lateral: Pain and temperature
577
What is the pathway for the spinothalamic tract?
Ascending: Enters at spinal level of nerve ascends 1-2 spinal levels and then decussates
578
What information does the corticospinal tract convey?
Upper motor neurons for movement. Decussates at the medulla
579
What are the nerve routes for the Knee jerk reflex?
L3/4
580
What are the nerve routes for the Big toe reflex?
L5
581
What are the nerve routes for the ankle Jerk reflex?
S1/2
582
What are the commonest causes of spinal cord injury?
Trauma Vertebral compression fractures Intervertebral disc disease - prolapse/herniation Tumours Infection
583
What are the common vertebral body neoplasms that can cause spinal compression?
Mets from: Lungs Breast RCC Melanoma
584
What are the different types of spinal cord injury?
Complete SC injury Anterior SC injury Posterior SC injury Central SC injury Brown-Sequard Syndrome
585
What is Spinal Cord Compression?
Compression of the spinal cord resulting in upper neurone signs and specific symptoms dependent on where compression is
586
What is the commonest cause of acute spinal cord compression?
Vertebral body neoplasms
587
What is the concern if there is sphincter involvement in spinal cord compression
This is a late and bad sign signalling a poorer prognosis
588
What would be the features of a complete spinal cord compression?
All motor and sensory function below the SCI level
589
What would be the features of an anterior spinal cord compression?
Disruption of anterior spinal cord or anterior spinal artery Loss of motor function below the level Loss of pain and temperature sensation (anterior column) Preservation of fine touch and proprioception (posterior column)
590
What would be the features of a posterior spinal cord compression?
Disruption of posterior spinal cord or posterior spinal artery (rare) Loss of fine touch and proprioception (posterior column) Preservation of pain and temperature sensation (anterior column)
591
What would be the clinical features of brown-Sequard syndrome?
- (Ipsilateral Corticospinal) Ipsilateral weakness and loss of motor function below the lesion. - (Ipsilateral DCML) Ipsilateral loss of proprioception, 2-point discrimination and fine touch. - (Contralateral Spinothalamic) Contralateral loss of pain and temperature sensation 1-2 spinal segments below the lesion.
592
What is Sciatica?
Sciatica refers to the symptoms associated with irritation of the sciatic nerve.
593
What spinal nerves form the sciatic nerve? Where is the most common region of compression to cause Sciatica?
L4-S3 spinal roots Most common region of compression at L5/S1
594
What is the innervation of the sciatic nerve?
The sciatic nerve supplies sensation to the Posterior thigh, lateral lower leg and the foot. (below the knee) * Sciatic Nerve (L4-S3) innervates Muscles of the posterior thigh * Tibial Nerve (Posterior Divisions of Sacral plexus) innervates Posterior leg * Common Peroneal Nerve (Anterior Divisions of Sacral Plexus) innervates anterior/lateral leg * Sensory innervation of entire foot, leg, and posterior thigh.
595
What nerves are branches of the sciatic nerve?
Common peroneal nerve Tibial nerve
596
What are the Signs and Symptoms of Sciatica?
* Unilateral pain from the buttock radiating down the back of the thigh to below the knee or feet * Paraesthesia (pins and needles), numbness and motor weakness * Reflexes may be affected depending on spinal root affected **Signs:** * Unilateral * Weak plantar flexion * Absent right ankle jerk * Decreased sensation over lateral edge and sole of right foot
597
What are the main causes of Sciatica?
**Intervertebral Herniated/prolapsed disc** Tumours Piriformis Syndrome Spondylolisthesis Spinal stenosis
598
What is Bilateral sciatica a red flag for?
Cauda Equina syndrome
599
What are the diagnostic investigations for sciatica?
**Clinical Diagnosis generally:** Can't Do straight leg raise test without pain **Other Investigations:** * X-ray * CT scan * MRI - if cauda equina suspected
600
What is the main treatment for sciatica?
Physiotherapy + Analgesia: * NSAIDs first line **Neuropathic pain medications are possibly used later on** * Amitriptyline (TCA) * Duloxetine (SNRI)
601
What is Brown Sequard Syndrome?
Hemi-section of the spinal cord and therefore loss of sensations of pain temperature and touch and motor movement
602
What are the causes of Brown-Sequard Syndrome?
Space occupying lesions Intervertebral disc prolapses Vertebral bone fractures Trauma – gunshot wounds, knife wounds Infectious – HIV MS
603
What is the Investigation and Treatment for Brown Sequard Syndrome?
Ix - MRI Spine Tx - Supportive (physical/occupational therapy) and Steroids (Dexamethasone)
604
What are the signs of a Tibial Nerve Lesion?
L4-S3 (Posterior cord) Inability to: Plantarflexion Invert foot Flex toes Sensory loss over sole of foot
605
What are the signs of a Common Peroneal Nerve Lesion? What other signs would suggest a different differential?
L4-S2 (Anterior cord Foot drop Weak ankle dorsiflexion and eversion Sensory loss over dorsal foot If Ankle inversion/hip adduction affected it could be L5 radiculopathy
606
What are the side effects Amitriptyline?
constipation. Blurred vision/dizziness dry mouth. feeling sleepy. Confusion Urinary retention headache
607
Define the Radial Nerve
The radial nerve is a peripheral nerve that arises from the **posterior cord of the brachial plexus**, * Originating from **nerve roots C5-T1**. * It serves both motor and sensory functions in the arm.
608
What is the epidemiology of radial nerve injuries?
Radial nerve injuries are relatively common, often resulting from fractures of the humerus or excessive pressure on the nerve. High-risk populations include individuals involved in heavy physical activities, sports, or those with specific medical conditions that make them more prone to fractures or nerve compression.
609
What is the aetiology of radial nerve injuries?
* Direct trauma or injury to the nerve * Compression or entrapment (e.g., in conditions such as Saturday night palsy) * Iatrogenic causes during surgical procedures * Systemic diseases that affect the peripheral nerves (e.g., diabetes mellitus)
610
What is the innervation of the radial nerve?
**Motor:** * Arm: Triceps Brachii, Brachioradialis, Extensor carpi radialis longus * Forearm: Muscles of the posterior compartment **Sensory:** * Lower lateral cutaneous nerve of arm = innervates the skin inferior to the insertion of the deltoid * Posterior cutaneous nerve of arm = innervates the skin on the posterior surface of the arm *Posterior cutaneous nerve of forearm = innervates the skin in the middle of the posterior forearm and dorsal surface of the hand
611
What are the clinical features of a radial nerve injury?
* Weakness or paralysis of the muscles innervated by the radial nerve (e.g., triceps brachii, brachioradialis, and extensor muscles of the forearm) **Wrist drop** * Numbness, tingling, or pain in the sensory distribution of the radial nerve (posterior forearm, lateral aspect of the dorsum of the hand, and dorsal surface of the lateral 3 1/2 digits)
612
What are some differential diagnoses for radial nerve injury?
* **Brachial Plexopathy:** Similar motor and sensory loss, but usually involves other nerves of the brachial plexus. * **Carpal Tunnel Syndrome:** Primarily causes sensory changes in the palmar aspect of the hand and motor weakness in the median nerve distribution. * **Ulnar Neuropathy:** Presents with sensory and motor deficits in the ulnar nerve distribution, including the 5th digit and medial half of the 4th digit. * **Cervical Radiculopathy:** Symptoms may overlap with radial nerve injury, but there may also be neck pain, and symptoms may be exacerbated by neck movements.
613
What are the investigations for a Radial nerve injury?
**Neurological examination:** to assess sensory and motor deficits **Electromyography (EMG) and Nerve Conduction Studies (NCS):** to evaluate nerve function **X-Ray** if history of trauma to rule out bone fractures or disclocations **MRI or CT scan:** to identify any anatomical causes of nerve injury such as a fracture or mass lesion
614
What is the management of radial nerve injuries?
**Depends on the underlying cause:** **Conservative measures:** including rest, physical therapy, and use of **splints** to prevent muscle contractures in cases of mild nerve injury **Pharmacological interventions:**such as analgesics for pain management **Surgical interventions:** in cases of severe nerve injury or where the cause is a correctable lesion such as a tumour or fracture
615
Define the Ulnar Nerve?
* The ulnar nerve is a peripheral nerve that arises from the **medial cord of the brachial plexus**, * Roots in the **C8-T1 nerve roots**. * It serves both motor and sensory functions in the forearm and hand.
616
What is the aetiology of ulnar neve injuries?
* Direct trauma or injury to the nerve * Compression or entrapment (e.g., in conditions such as cubital tunnel syndrome or Guyon's canal syndrome) * Iatrogenic causes during surgical procedures * Systemic diseases that affect the peripheral nerves (e.g., diabetes mellitus)
617
What is the innervation of the ulnar nerve?
Muscular branch = innervates muscles of the anterior compartment: Flexor carpi ulnaris, Medial part of Flexor Digitorum Profundus Palmar cutaneous branch = innervates the medial half of the palm Dorsal cutaneous branch = innervates the dorsal surface of the medial 1 1/2 digits and associated dorsal hand area Superficial Ulnar nerve: innervates the palmar surface of the medial 1 1/2 digits Deep = intrinsic muscles of the hand **except LOAF**
618
What are the clinical features of an ulnar nerve injury?
**Damage at wrist** * 'claw hand' - hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits * wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals) * wasting and paralysis of hypothenar muscles * sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects) **Damage at elbow** * as above (however, ulnar paradox - clawing is more severe in distal lesions) * radial deviation of wrist
619
What are some differential diagnoses for ulnar nerve injury?
* **Brachial Plexopathy:** Similar motor and sensory loss, but usually involves other nerves of the brachial plexus. * **Carpal Tunnel Syndrome:** Primarily causes sensory changes in the palmar aspect of the hand and motor weakness in the median nerve distribution. * **Ulnar Neuropathy:** Presents with sensory and motor deficits in the ulnar nerve distribution, including the 5th digit and medial half of the 4th digit. * **Cervical Radiculopathy:** Symptoms may overlap with radial nerve injury, but there may also be neck pain, and symptoms may be exacerbated by neck movements.
620
What are the investigations for a suspected ulnar nerve injury?
**Neurological examination:** to assess sensory and motor deficits **Electromyography (EMG) and Nerve Conduction Studies (NCS):** to evaluate nerve function **MRI or CT scan:** to identify any anatomical causes of nerve injury such as a fracture or mass lesion
621
What is the management for an ulnar nerve injury?
**Depends on the underlying cause:** **Conservative measures:** including rest, physical therapy, and use of **splints** to prevent muscle contractures in cases of mild nerve injury **Pharmacological interventions:**such as analgesics for pain management **Surgical interventions:** in cases of severe nerve injury or where the cause is a correctable lesion such as a tumour or fracture
622
Define Carpal Tunnel Syndrome
Carpal Tunnel Syndrome is a condition characterized by median nerve compression as it traverses the narrow carpal tunnel from the forearm to the hand. **It is the most common mononeuropathy**, often associated with repetitive wrist activities, systemic diseases, and anatomical variations.
623
What is the epidemiology of Carpal Tunnel Syndrome?
Most common mononeuropathy
624
# HODPARAR What is the aetiology of Carpal Tunnel Syndrome?
Factors that **increase pressure within or decrease the size of the carpal tunnel** **Mostly Idiopathic** **H**ypothyroidism **O**besity **D**iabetes **P**regnancy **A**cromegaly **R**heumatoid Arthritis **A**myloidosis **R**epetitive Strain Injury
625
What are the clinical features of Carpal Tunnel Syndrome?
* Pain and paraesthesia to the lateral 3.5 digits due to the impingement of the palmar digital branch of the median nerve. * Wasting of the **thenar eminence** as a result of the compromise of the recurrent branch of the median nerve. * Symptoms are often worse at night or after activities involving wrist flexion.
626
What are some differential diagnoses for Carpal Tunnel Syndrome?
* **Cubital Tunnel Syndrome:** Presents with pain, numbness, and tingling in the ring and little fingers, and weakness in hand grip. * **Thoracic Outlet Syndrome:** Symptoms may include pain, numbness, and weakness in the arm, along with potential neck, shoulder, and hand discomfort. * **Radial Tunnel Syndrome:** Characterized by fatigue or dull, aching pain at the top of the forearm with forearm rotation. * **Ulnar Neuropathy:** Symptoms can include numbness, tingling, or pain in the arm, hand, and fingers, especially the ring and little fingers.
627
What are the investigations for Carpal Tunnel Syndrome?
* **Clinical Examination:** * Phalen's Test * Tinel's Test * Compression test * **Electromyography (EMG):** Motor and Sensory prolongation * **Nerve Conduction Studies (NCS):** to measure the speed and strength of signals traveling through the median nerve. Additional tests, such as MRI or X-ray, may be used in some cases to rule out other conditions or explore the possibility of structural abnormalities.
628
What is the management for Carpal Tunnel Syndrome?
**Conservative measures:** Wrist splinting, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroid injections, and modifying activities that exacerbate symptoms. **Surgery:** This is considered for severe or persistent cases that are unresponsive to conservative treatment. Surgical release of the transverse carpal ligament is the most common procedure. Physical therapy is often employed post-surgery to regain strength and mobility.
629
Define Common Peroneal Nerve Injury?
The common peroneal nerve, also known as the common fibular nerve, is part of the sciatic nerve and is particularly susceptible to injury at the neck of the fibula. Damage to this nerve often occurs due to direct trauma around the knee, with common peroneal nerve injury resulting in specific motor and sensory deficits in the affected leg.
630
What is the Aetiology of Common Peroneal Nerve Injuries?
* Direct trauma to the lateral aspect of the knee * Fractures or dislocations involving the knee joint or fibular head * Compression injury, often due to prolonged immobilisation or positioning * Iatrogenic injury during surgery * Certain systemic diseases like diabetes or polyneuropathies can predispose to nerve injury
631
What are the clinical features of Common Peroneal Nerve Injury?
* **'Foot drop':** Due to paralysis of the foot extensors, including the tibialis anterior, extensor digitorum longus, and extensor hallucis longus muscles * **Foot inversion:** Occurs as the common peroneal nerve also innervates the foot evertor muscles * Potential sensory loss or paresthesia in the distribution of the nerve
632
What are some differential diagnoses for Common Peroneal Nerve Injury?
* **L5 radiculopathy:** Typically presents with pain radiating down the leg, weakness of the dorsiflexors and evertors of the foot, and sensory loss along the lateral leg and dorsum of the foot * **Sciatic nerve injury:** Marked by hamstring weakness, decreased Achilles reflex, and sensory loss in the sciatic nerve distribution * **Muscular dystrophies:** Characterised by progressive muscle weakness and atrophy affecting various muscle groups * **Stroke:** Acute onset of unilateral weakness, often accompanied by other neurological deficits
633
What are the investigations for Common Peroneal Nerve Injury?
**Electromyography (EMG) and nerve conduction studies:** These can help establish the diagnosis and severity of the nerve injury **MRI:** Can aid in identifying space-occupying lesions, nerve sheath tumours, or other structural anomalies **Ultrasound:** Useful for visualising the course of the nerve and potential sites of entrapment
634
What is the Management of Common Peroneal Nerve Injury?
**Conservative treatment:** Physical therapy and orthotic devices can help maintain foot position and prevent secondary contractures **Surgical intervention:** If no improvement is observed in neurological function after 2-3 months, surgical decompression or nerve grafting might be considered
635
Define Mononeuritis Multiplex?
A type of peripheral neuropathy which is characterized by **simultaneous or sequential involvement of individual non-contiguous nerve trunks**, either partially or completely, evolving over days to years and typically presenting with acute or subacute loss of sensory and motor function of individual nerves.
636
What are the clinical features for Mononeuritis Multiplex?
* The pattern of involvement is asymmetric. * However, as the disease progresses, deficits becomes more confluent and symmetrical, making it difficult to differentiate from polyneuropathy. Therefore, attention to the pattern of early symptoms is important. * Mononeuritis multiplex may also cause pain, which is characterized as deep, aching pain that is worse at night and frequently in the lower back, hip, or leg. * In people with diabetes mellitus, mononeuritis multiplex is typically encountered as acute, unilateral, and severe thigh pain followed by anterior muscle weakness and loss of knee reflex.
637
# WARDS PLC What is the aetiology of Mononeuritis Multiplex?
* Wegener’s granulomatosis (**Vasculitis**) * Aids/Amyloid * Rheumatoid arthritis (Other immune mediated diseases) * Diabetes mellitus * Sarcoidosis * Polyarteritis nodosa * Leprosy (Other infections: Lyme, parvovirus, HIV) * Carcinoma
638
# DAVIDE What are some causes of Peripheral Nerve Disease?
**D**iabetes **A**lcohol **V**itamin B12 Deficiency **I**nfective - Guillain Barre/Charcot Marie Tooth **D**rugs - isoniazid **E**very vasculitis
639
Give some examples of Mononeuropathies?
* Carpal tunnel syndrome (medial nerve) – most common * Radial neuropathy (entrapment at the cubital tunnel or radial tunnel) * Ulnar neuropathy (entrapment at the cubital tunnel) * Peroneal neuropathy (entrapment at the fibular head) * Cranial mononeuropathies (III or VII cranial nerve palsy)
640
Define a Radiculopathy?
Commonly referred to as a pinched nerve, radiculopathy is injury or damage to nerve roots in the area where they leave the spine.
641
What is the most common Radiculopathy?
Lumbar radiculopathy (L5 radiculopathy)
642
What is the aetiology of Radiculopathy?
* **Damage to a disk in the spine:** The damaged disk may then press on nearby nerve roots. * **Degeneration from wear and tear, and aging:** This can lead to narrowing (stenosis) of the openings between the vertebrae. The narrowed openings press on nerve roots as they leave the spinal canal. * **Unstable spine:** This is when a vertebra slips forward. It can then press on a nerve root. * Other, less common things can put pressure on nerves in the low back. These include **diabetes, infection, or a tumour.**
643
What are the clinical features of Lumbar radiculopathy?
* Pain in the low back. * Pain, numbness, tingling, or muscle weakness that travels into the buttocks, hip, groin, or leg. * Similar features of Common peroneal nerve lesion **plus weakness of hip abduction** * Muscle spasms.
644
What is the management for Radiculopathy?
* Analgesia: Paracetamol or NSAIDs * Limits on positions and activities that increase pain. **But lying in bed or avoiding all movement is only recommended for a short period of time.** * **Physical therapy**, including exercises and stretches. This helps decrease pain and increase movement and function. * **Steroid injections** into the lower back. This may help relieve symptoms for a time. * **Weight-loss program**. If you are overweight, losing extra pounds may help relieve symptoms. * Surgery is a possibility
645
What are the clinical features of L5 Radiculopathy?
* L5 radiculopathy is usually associated with **numbness down the side of the leg and into the top of the foot.** * S1 radiculopathy typically results in numbness down the back of the leg into the outside or bottom of the foot.
646
Define Functional Neurological Disorder?
Functional Neurological Disorder (FND) is a condition in which patients experience neurological symptoms such as weakness, movement disorders, sensory symptoms, and blackouts. As a functional disorder, there is by definition no known disease process affecting the structure of the body, yet the person experiences symptoms relating to their body function.
647
What is the aetiology of FND?
No clear cause as there is not a pathological process leading to FND. **Associated with:** * Childhood neglect * Depression and Anxiety * Other neurological condition such as Epilepsy.
648
What are the clinical features of FND?
* **Limb weakness or paralysis** * **Blackouts** (also called dissociative or non-epileptic seizures/attacks) – these may look like epileptic seizures or faints * Movement disorders including tremors, dystonia (spasms), myoclonus (jerky movements) * Visual symptoms including loss of vision or double vision * Speech symptoms including dysphonia (whispering speech), slurred or stuttering speech * **Sensory disturbance including hemisensory syndrome** (altered sensation down one side of the body) * Dizziness and balance problems
649
What are the investigations for FND
Clinical examination and history No results on imaging or blood tests leading to other underlying pathology.
650
What are some differential Diagnoses for FND?
* **Multiple Sclerosis:** Presents with many similar features of FND however there will be evidence of MS lesions on imaging. * **Epilepsy:** FND patients may present with blackouts and seizures that mimic epileptic seizures however there is no underlying disease process.
651
What is the management of FND?
* Physiotherapy * Occupational Therapy **Medications for complications:** * Sleeping tablets * Gabapentin/Pregabalin for neuropathic pain * Anti-epileptic/anticonvulsants * SSRIs for depression.