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.

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2
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%)

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3
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.

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4
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.

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5
Q

What are some clinical features of Alzheimer’s Disease?

A

Memory Impairment

Language Impairment

Executive Dysfunction - impaired planning, organisation, and activities of tasks

Behavioural Changes

Psychological Symptoms

Disorientation

Loss of Motor Skills

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6
Q

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

A

Agitation
Aggression
Apathy
Mood swings
Irritability

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7
Q

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

A

Hallucinations
Delusions
Paranoia (in later stages)

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8
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

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9
Q

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

A

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

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10
Q

What are the lab tests involved in a confusion screen?

A

FBC.
U&E.
Calcium.
B12 and Folate.
TSH.
Glucose.
ECG

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11
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

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12
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

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13
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.

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14
Q

What is the epidemiology of Parkinson’s disease?

A

The second most common neurodegenerative disorder after Alzheimer’s disease

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15
Q

What is the aetiology of Parkinson’s disease?

A

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

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16
Q

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

A

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

Previous Head injury

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17
Q

What are some protective factors for Parkinson’s disease?

A

Smoking
Caffeine intake
Physical activity

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18
Q

What is the characteristic triad of Parkinson’s disease?

A

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

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19
Q

What are the clinical features of Parkinson’s disease?

A

Motor Features:

  • Asymmetrical Pin-rolling Tremor - worse on one side
  • Rigidity - “Cogwheel Rigidity”
  • Bradykinesia - Shuffling gait, Micrographia

Other Features:

  • Sleep Disturbance and insomnia
  • Anosmia (loss of sense of smell)
  • Autonomic Dysfunction - constipation and ED.
  • Psychiatric features - Depression, Hallucinations, Anxiety
  • Postural instability (a Late feature of Parkinson’s)
  • Hypomimia (lack of facial expression)
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20
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)
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21
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

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22
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

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23
Q

What are some Parkinson’s-Plus Syndromes?

A
  • Multiple System Atrophy - Presents with early autonomic dysfunction and postural instability compared to PD
  • Lewy Body Dementia - Early and prominent cognitive dysfunction before parkinsonism traits. (Visual Hallucinations)
  • Progressive Supranuclear Palsy
  • Corticobasal Degeneration
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24
Q

What are some differential Diagnoses for Parkinson’s Disease?

A
  • 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
  • Wilson’s Disease: May be associated with signs of liver disease
  • Dementia Pugilistica: Secondary to repeated head trauma
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25
What are some side effects of Levodopa?
**Central Side effects:** * Dyskinesia (Excessive motor activity; **Dystonia** **Chorea** **Athetosis**) * Hallucinations * Confusion * Psychosis **Peripheral Side effects:** * Postural Hypotension * Nausea and Vomiting
26
Give an example of a Catechol-o-methyltransferase (COMT) inhibitor?
Entacapone
27
Give an example of a Monoamine Oxidase B (MOA) inhibitor?
Selegiline Rasagiline
28
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**
29
Give Some examples of Dopamine Agonists and a key side effect?
Bromocriptine Pergolide Cabergoline **Side effect of Pulmonary Fibrosis in prolonged use**
30
Define Essential Tremor?
Benign essential tremor is a relatively common condition chronic neurological condition associated with older age. 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.
31
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.
32
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**
33
What is the Management of Parkinson's Disease?
Tx focused on Symptom control and minimising side effects: **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** **COMT Inhibitors** - Decarboxylase inhibitor to prevent breakdown of L-DOPA **MOA Inhibitors** - Inhibit the breakdown of neurotransmitters such as dopamine and serotinin. **Dopamine Agonists** - Mimic action of Dopamine
34
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**
35
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.
36
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.
37
What is the Management of Essential Tremor?
No definitive treatment for Essential Tremor Medications that may improve symptoms are: * Propranolol (a non-selective beta blocker) * Primidone (a barbiturate anti-epileptic medication) * Topiramate
38
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.
39
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**
40
What is the Aetiology of MND?
Associated with the misfolding of the **TDP-43 Protein** 2% of cases associated with a mutation in the **SOD-1 gene**
41
What are some risk factors for MND?
Increased age >60 yrs Male FHx Smoking RUGBY
42
What is the most common type of Motor neurone Disease?
Amyotrophic Lateral Sclerosis (ALS) **Accounts for 50% of cases**
43
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)**
44
What is the Pathophysiology of MND?
Degenerative condition selectively affecting motor neurons 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
45
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
46
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
47
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)
48
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).
49
What are the clinical signs of MND?
Mixed UMN and LMN signs Dysarthria / Dysphagia Fasciculations Split Hand Sign - Disproportionate wasting of thenar muscles compared to hypothenar muscles.
50
What are some differential Diagnosis of MND?
* **Thyrotoxicosis syndrome:** Characterised by symptoms like weight loss, heat intolerance, fatigue, and muscle weakness. * **Paraproteinaemias:** These disorders can manifest with neuropathy, fatigue, bone pain, and recurrent infections. * **Brainstem lesions:** Depending on the location, brainstem lesions can cause symptoms such as weakness, numbness, double vision, and coordination problems. * **Cervical spondylopathy:** Can present with neck pain, numbness or weakness in limbs, and coordination and reflex issues. * **Myasthenic syndrome:** Characterised by muscle weakness that worsens with activity and improves with rest. * **Chronic inflammatory demyelinating polyneuropathy:** This disorder presents with progressive weakness and impaired sensory function in the legs and arms. * **Multifocal mononeuropathy:** Characterised by weakness in the distribution of individual peripheral nerves, often asymmetrically.
51
How is MND Diagnosed?
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.
52
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.
53
What is the management of MND?
**No effective treatments for halting or preventing progression of MND** **Riluzole** can slow progression by a couple of months **NIV (non-invasive ventilation)** can be used to support breathing **Symptom Control:** * MDT input * **Baclofen** for muscle spasticity * Benzodiazepines for breathlessness due to anxiety
54
What is the prognosis of MND
Depending on the type of MND will have a different prognosis. * PMA has the **best prognosis** * PBP has the **worst prognosis** * Average prognosis is 2-3 years after diagnosis
55
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.
56
What is the pathophysiology of MS?
**Multiple sclerosis affects the central nervous system (the oligodendrocytes)** * Inflammation and immune cell infiltration cause damage to the myelin, affecting the electrical signals moving along the neurones. * When a patient presents with symptoms of an MS attack (e.g., an episode of optic neuritis), there are often other demyelinating lesions throughout the central nervous system * In early disease, re-myelination can occur, and the symptoms can resolve. * In the later stages of the disease, re-myelination is incomplete, and the symptoms gradually become more permanent. * A characteristic feature of MS is that lesions vary in location, meaning that the affected sites and symptoms change over time. **The lesions are described as “disseminated in time and space”.**
57
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
58
What is the Epidemiology of MS?
Predominantly affects females Young-Middle age (30 yrs)
59
What is characteristic about the symptom onset in MS?
* Symptoms usually progress over more than 24 hours. * 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.**
60
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 * Relative afferent pupillary defect
61
What are some other causes of Optic Neuritis?
Sarcoidosis Systemic lupus erythematosus Syphilis Measles or mumps Neuromyelitis optica Lyme disease
62
What are the clinical manifestations of MS?
* Optic Neuritis * Sensory or Cerebellar Ataxia * Transverse Myelitis **Focal Neurological Symptoms:** * Incontinence * Horner Syndrome * Facial Nerve Palsy * Limb Paralysis **Focal Sensory Symptoms:** * Trigeminal Neuralgia * Numbness and paraesthesia * Lhermitte's Sign (**Electric shock sensation traveling down the spine into limbs when flexing the neck**)
63
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
64
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.
65
What investigations are done in MS?
* Clinical History and Examination * MRI Scan - Demonstrates typical lesions * Lumbar Puncture - **IgG Oligoclonal Bands in CSF**
66
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**
67
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)
68
What is the Chronic management of MS?
Disease Modifying therapies: * **Beta Interferon** * Biologics: **Natalizumab, Alemtuzumab** Symptomatic Control: * Exercise to maintain strength and activity * Amitriptyline or gabapentin for neuropathic pain * Physiotherapy * **Baclofen**/Botox for spasticity * SSRIs for depression and fatigue
69
What may indicate a worse prognosis in MS?
Older age of Onset Male Gender Primary Progressive MS High relapse rate Smoking Comorbidities
70
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.
71
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.**
72
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.
73
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
74
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
75
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
76
What are the investigations for Muscular Dystrophy?
* **Genetic Testing is Gold standard for diagnosis** * Creatine Kinase Measurement - **First line screening as levels are significantly raised in muscular dystrophy** * Muscle Biopsy
77
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
78
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.**
79
Define Huntington's Chorea?
Huntington’s disease (also called Huntington’s chorea) is an autosomal dominant genetic condition that causes progressive neurological dysfunction.
80
What is the epidemiology of Huntington's disease?
It is one of the most common hereditary neurodegenerative disorders 1 in 10,000-20,000
81
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
82
Give some examples of other Trinucleotide repeat disorders?
Fragile X syndrome Spinocerebellar ataxia Myotonic dystrophy Friedrich ataxia
83
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**
84
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)
85
What is the presentation of Huntington's disease?
**Symptoms typically begin aged 30-50** * Huntington’s chorea presents with an insidious, progressive worsening of symptoms. * It typically begins with cognitive, psychiatric or mood problems, * Followed by the development of movement disorders
86
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)
87
What are some differential Diagnosis for Huntington's disease?
Parkinson's disease Sydenham's Chorea (Rheumatic Fever) Wilsons disease SLE Huntington's disease-like disorders Neuroacanthocytosis Syndromes
88
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
89
What is the management of Huntington's disease?
**No curative or preventative treatments** **Chorea Management**: Tetrabenazine **Depression Management**: SSRIs **Psychosis Management**: Antipsychotics (Haloperidol) **Supportive care**: MDT team
90
What is the prognosis for a patient with Huntington's disease?
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**
91
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.
92
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
93
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
94
What are the investigations for a Brain Abscess?
CT scan of the brain
95
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
96
Define Meningitis?
Inflammation of the meninges from both infective and non-infective causes. This is a notifiable condition to PHE
97
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
98
What is the most common cause of meningitis in children and adults?
**Neisseria meningitidis Streptococcus pneumonia**
99
What is the most common cause of meningitis in neonates?
Group B Streptococcus - **Streptococcus Pyogenes** E.coli Strep. pneumonia Listeria
100
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.
101
What is the most common cause of meningitis?
Viral infection (Most commonly **enteroviruses**) More common but less severe than bacterial causes.
102
What are the main risk factors for meningitis?
Extremes of age (Infant/elderly) Immunocompromised Pregnancy Travel Crowded environment - barracks/uni Non-vaccinated
103
What vaccines are available for meningitis coverage?
N. Meningitidis - Men B + Men C + Men ACWY S. pneumoniae - PCV Vaccine
104
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
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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.
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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.**
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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
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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)**
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What are some contraindications for a lumbar puncture?
Raised ICP GCS <9 Focal Neurological signs
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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
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Where is a lumbar puncture usually taken from?
Between L3/L4 Since spinal cord ends L1/2
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What are some differential diagnoses for Meningitis?
* Encephalitis * Subarachnoid Haemorrhage * Brain Abscess * Sinusitis * Migraine
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What would the results of CSF analysis be in Bacterial, Viral and Fungal Meningitis?
Fungal: * Lymphocytosis * Increased Protein Concentration * Decreased Glucose Concentration
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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
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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
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What antibiotics should be used to treat bacterial meningitis in children?
Under 3 Months: **Cefotaxime and Amoxicillin** (covers for listeria) Over 3 Months: **Ceftriaxone**
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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.**
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What is the management of Viral Meningitis?
Usually only requires Supportive treatment **Acyclovir** can be used to treated suspected or confirmed HSV/VZV infections
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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
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Define Encephalitis?
Encephalitis is a pathological condition characterised by inflammation of the brain parenchyma, also known as the "encephalon".
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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
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What are the most common Viral causes of Encephalitis?
**Herpes Simplex Virus Type 1 (HSV-1)** Others: * HSV-2 * CMV * EBV * VZV * HIV
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What are the clinical features of Encephalitis?
**Altered Mental Status** Fever Flu-like prodromal illness Seizures Acute onset Focal neurological deficits Headaches Behavioural changes
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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**
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What may be seen on MRI in Encephalitis?
Temporal lobes affected Bilateral Multifocal Haemorrhage
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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
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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
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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
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Define Guillain-Barre Syndrome?
Guillain-Barré syndrome is an acute paralytic polyneuropathy that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms. It is usually triggered by an infection and is particularly associated with to **Campylobacter jejuni**, cytomegalovirus (CMV) and Epstein-Barr virus (EBV).
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What is the pathophysiology of GBS?
Guillain-Barré is thought to occur due to a process called molecular mimicry. The B cells of the immune system create antibodies against the antigens on the triggering pathogen. These antibodies also match proteins on the peripheral neurones. They may target proteins on the myelin sheath or the nerve axon itself.
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What are the clinical forms of GBS?
* Acute inflammatory demyelinating polyneuropathy * Acute motor axonal neuropathy * Acute Sensory and motor axonal neuropathy * Miller-Fisher variant
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What is the Aetiology of GBS?
Typically occurs **1-3 weeks following an infection** * Campylobacter jejuni * Cytomegalovirus * Epstein Barr Virus
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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
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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 * LMN signs (areflexia) * **Cranial Nerve Signs** - Ophthalmoplegia, facial nerve palsy. * **Autonomic Dysfunction** - Urinary retention, Ileus, Arrhythmias * **Potential respiratory distress in severe cases**
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What are some differential Diagnoses?
**Brainstem strokes** Polio Lyme Disease CMV HIV TB Transverse Myelitis Myasthenia Gravis
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What criteria is used to diagnose GBS?
Brighton Criteria
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What are the investigations for GBS?
* **Nerve Conduction studies** - reduced signal conduction * **Lumbar Puncture + CSF analysis** - raised protein with normal cell count and glucose Other: * Monitoring of FVC for respiratory muscle involvement * Serological - **Anti-ganglioside antibodies**
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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
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What is the leading cause of death in GBS?
**Pulmonary Embolism** hence the VTE prophylaxis
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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**
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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
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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.
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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**
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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
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What are the investigations for Bulbar Palsy?
* 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
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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**
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Define Cerebellar Syndrome?
A cluster of clinical manifestations resulting impaired function of the cerebellum.
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# 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 * **Metabolic:** Alcoholism * **Iatrogenic:** Phenytoin, Carbamazepine * **Neoplastic:** Primary and Secondary Tumours * **Congenital:** Friedrich's Ataxia, Spinocerebellar Ataxias (eg. SPG7)
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# 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
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What symptoms arise from cerebellar vermis lesions?
Truncal Ataxia Gait instability **Few cerebellar signs in the limbs**
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What symptoms arise from cerebellar hemisphere lesions?
VANISHED signs in **Ipsilateral Limb**
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What are some differential diagnoses for Cerebellar Dysfunction?
* Stroke * Multiple Sclerosis * Posterior Fossa Tumour * Alcoholism * Medication side effects: **Phenytoin, Carbamazepine** * Tumours * Hereditary conditions
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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
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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
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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.**
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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
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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
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What are some complications of Herpes Zoster Ophthalmicus?
* **Ocular:** Conjunctivitis, Keratitis, Episcleritis, Uveitis * Ptosis * Post herpatic Neuralgia
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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**
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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**
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What is a typical patient presenting with Ramsay Hunt Syndrome?
Patient with facial nerve palsy and a vesicular rash around their ear
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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
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Define Cerebral Palsy?
Permanent neurological movement problems resulting from damage to the brain around the time of birth. 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.
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What is the Epidemiology of Cerebral Palsy?
2/3 in 1000
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What are some causes of Cerebral Palsy?
**Antenatal:** * Maternal infections * Trauma during pregnancy **Perinatal:** * Birth asphyxia (**Hypoxic Ischemic Encephalopathy**) * Pre-term birth **Postnatal:** * Meningitis * Severe neonatal jaundice * Head injury
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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.**
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What are some clinical features of Cerebral Palsy?
**Motor Problems** * Abnormal tone early infancy * Delayed motor milestones * Abnormal gait - **Hemiplegic/Diplegic Gait** * Feeding difficulties.
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What are some non-motor problems associated with Cerebral Palsy?
* Learning Difficulties (60%) * Epilepsy (30%) * Squints (30%) * Hearing Impairments (20%)
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What are some differential diagnoses to Cerebral Palsy?
* Muscular Dystrophies * Metabolic Disorders * Hereditary spastic paraplegia * Juvenile idiopathic arthritis
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What are some investigations performed in suspected Cerebral Palsy?
* **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
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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)
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What are some complications of Cerebral Palsy?
* Learning disability * Epilepsy * Kyphoscoliosis * Muscle contractures * Hearing and visual impairment * Gastro-oesophageal reflux
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Define Hypoxic Ischaemic Encephalopathy (HIE)?
A form of brain damage that occurs due to antenatal or perinatal hypoxia
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What is the epidemiology of HIE?
1-2 per 1000 Incidence is higher in premature and low birth weight infants
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What are some causes of Hypoxic Ischaemic Encephalopathy?
**Pre-partum** * Placental Abruption **Intrapartum** * Cord compression **Post Partum** * Prolonged Respiratory Arrest
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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
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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
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What are some differential diagnoses for HIE?
Meningitis Metabolic Disorders Intracranial Haemorrhage Drug withdrawal
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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
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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
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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.**
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How is Malaria spread?
Through bites from the **Female Anopheles Mosquitoes** that carry the disease
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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
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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**
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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**
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What are some protective factors for malaria?
Sickle Cell Disease G6PD Deficiency HLA-B53 Absence of Duffy Antigens
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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
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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.
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What is the management of Malaria?
Anti-malarials: * **Riamet** is the usual first choice * **Quinine plus Doxycycline** * **Quinine plus clindamycin** **There are increasing rates of resistance to chloroquine**
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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
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What are some complications of P. falciparum Malaria?
* **Cerebral malaria** * Seizures * Reduced consciousness * Acute kidney injury * Pulmonary oedema * Disseminated intravascular coagulopathy (DIC) * Severe haemolytic anaemia * Multi-organ failure and death
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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**
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What are the different Primary Brain Tumours?
* Gliomas (Astrocytoma, Ependymoma, Oligodendroma, Glioblastoma * Meningioma * Schwannoma * Pituitary adenoma
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What is the most common Primary Brain Tumour in Adults and in Children?
Adults: Glioblastoma followed by Meningioma Children: Astrocytoma and Craniopharyngioma
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What is the most common form of brain tumour?
Metastatic brain cancer
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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
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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
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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)**
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What is a Glioblastoma Multiforme?
Glioblastoma (GBM), also referred to as a grade IV astrocytoma, is a fast-growing and aggressive brain tumour Often fatal within 1 year of Dx
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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
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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
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What are the differential Diagnoses of a brain tumour?
Aneurysm Abscess Cyst Haemorrhage Idiopathic intracranial hypertension
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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
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What is an Acoustic Neuroma?
**Vestibular Schwannoma** (previously termed acoustic neuroma) * A benign tumour arising from the auditory (vestibulocochlear) nerve often found at the cerebellopontine angle.
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Where do acoustic neuromas occur?
At the cerebellopontine angle (sometimes referred to as cerebellopontine angle tumours)
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What are the clinical features of an Acoustic Neuroma?
40-60 years presenting with gradual onset: * **Unilateral sensorineural Hearing loss** (often first symptom) * Unilateral Tinnitus * Dizziness or imbalance * **Sensation of fullness in the ear** * Facial nerve palsy - due to compression if the tumour grows large enough
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What condition is associated with vestibular schwannomas?
Neurofibromatosis type II **Bilateral acoustic neuromas**
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What are the investigations for an Acoustic neuroma?
**Audiometry:** pattern of sensorineural hearing loss **Neuroimaging:** CT or MRI to establish diagnosis and features of the tumour
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What may be seen on histology in an Acoustic neuroma?
Antoni A or B patterns Verocay bodies
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What is the treatment for an Acoustic Neuroma?
Observation (watch and wait) Radiotherapy to reduce growth Surgery to remove the tumour
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What are some complications of an Acoustic Neuroma?
* Vestibulocochlear nerve injury, with permanent hearing loss or dizziness * Facial nerve injury, with facial weakness
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Define Bell's Palsy?
Bell's palsy is an idiopathic syndrome that causes damage to the **Facial Nerve** characterized by unilateral, lower motor neuron facial weakness, without sparing the extraocular muscles and muscles of mastication.
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What is the Aetiology of Bell's Palsy?
Largely unknown **Viral infections have been implicated as a particular cause** * **Reactivation of HSV 1** * EBV * VZV
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What are the clinical features of Bell's Palsy?
**Acute (but NOT sudden; around 72 hours) onset of unilateral lower motor neurone facial weakness** * Involves the forehead and lower parts of the face on the affected side * Upper facial signs include the **inability to wrinkle forehead and close eye fully on the affected side** * Lower facial signs include the loss of the nasolabial labial fold, and **drooping of the mouth, which is more pronounced when the patient tries to smile** * The severity of the paralysis can be quantified by the **House-Brackmann facial paralysis scale**, which is graded from 1 (normal) to 6 (complete paralysis). * **Hyperacusis** * Pain in the ear and surrounding area (**Postauricular otalgia**) * Loss of taste in the anterior tongue in 35% of patients
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What are some differential diagnoses for Bell's Palsy?
**Ramsay-Hunt Syndrome:** * Prominent otalgia * Vesicular rash in the external auditory meatus, palate, or tongue **Lyme disease:** * Erythema migrans * Systemic symptoms (e.g., fever, fatigue, arthralgia) **Herpes zoster:** * Unilateral vesicular rash following a dermatomal distribution * Associated pain and sensory disturbances **Acute otitis media:** * Signs of middle ear infection (e.g., otalgia, fever, otorrhea) **Stroke:** * Sudden onset of upper motor neuron facial weakness (forehead-sparing) * Other neurological deficits **Guillain-Barré syndrome:** * Ascending symmetric weakness * Sensory abnormalities * Autonomic dysfunction
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What are the investigations for Bell's Palsy?
**Primarily a clinical diagnosis** * Full Blood Count * ESR and CRP * Viral Serology * Lyme serology * Otoscopy * EMG * MRI/CT
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What is the management of Bell's Palsy?
* Prompt administration 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
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What is the prognosis of Bell's Palsy?
**Complete Recovery:** 70-80% **Incomplete recovery:** some patients may experience residual weakness or persistent facial symptoms Increased age and severity are associated with increased risk of incomplete recovery.
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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**
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Define a seizure
Seizures are transient episodes of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
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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.
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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)
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What is a non-epileptic seizure?
Paroxysmal event in which changes in behaviour, sensation and cognitive function caused by mental processes associated with psychosocial distress
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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.
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What are some risk factors for Epilepsy?
Family History Head Trauma Premature babies Cerebral Infections Dementia (10x more likely) Drug use - cocaine Cerebrovascular events
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# VITAMIN DE List some causes of Epilepsy?
**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
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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
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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**
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What are the different types of a Generalised Seizure?
* **Absence seizures:** brief pauses for less than 10 seconds. * **Tonic-clonic 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.
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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.
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What is an Atonic Seizure?
* 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.**
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What is Todd's Paralysis?
Period after an epileptic seizure in which the patient experiences temporary paralysis
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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)
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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**, and **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.
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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 * Memory flashbacks * Déjà vu * Doing strange things on autopilot
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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**
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How are seizures classified?
Where the seizures began Level of awareness during the seizure Other features of the seizure e.g. motor
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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
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What are the characteristics of a non-epileptic seizure?
* Situational * Duration 1 – 20 mins * Dramatic motor phenomena or prolonged atonia * Eyes closed * Ictal crying and speaking * Surprisingly rapid or slow postictal recovery * History of psychiatric illness, other somatoform disorders
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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.
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What are the investigations for seizures and epilepsy?
* Detailed clinical History * Neurological Examination * Bloods: blood cultures (sepsis), blood glucose (hypos/DM) and U&Es (electrolyte imbalance) * Electroencephalogram (EEG): typically after the second simple tonic-clonic seizure * Neuroimaging: CT or MRI: to look for structural problems or tumours. Others: ECG: exclude cardiac problems Lumber puncture: exclude encephalitis or meningitis
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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
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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.
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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
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What is the management for Generalised Tonic-clonic seizures?
**1st Line:** Sodium Valproate **2nd Line:** Lamotrigine, Levetiracetam **Lamotrigine or Levetiracetam is first line in women of childbearing age who can have children**
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What is the management of an Generalised Absence Seizure?
**1st Line**: Ethosuximide **2nd Line:** Sodium Valproate
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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
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What are some notable side effects of sodium valproate?
**Teratogenic, so patients need careful advice about contraception** Liver damage and hepatitis Hair loss Tremor
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What are some notable side effects of Carbamazepine?
Agranulocytosis Aplastic anaemia Induces the P450 system so there are many drug interactions
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What are some notable side effects of Phenytoin?
Folate and vitamin D deficiency Megaloblastic anaemia (folate deficiency) Osteomalacia (vitamin D deficiency)
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What are some notable side effects of Ethosuximide?
Night terrors Rashes
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What are some notable side effects of Lamotrigine?
* Stevens-Johnson Syndrome or DRESS syndrome: Life threatening skin rashes * Leukopenia
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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.
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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.
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What is the DVLA guidance on epilepsy?
**You must stop driving straight away** * You can reapply for your licence after 6 months for a 1 off seizure or 1 year after multiple
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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**
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What is the management of Status Epilepticus?
* Secure the airway * Give high-concentration oxygen * Assess cardiac and respiratory function * Check blood glucose levels * Gain intravenous access (insert a cannula) * **IV lorazepam 4mg Bolus**, repeated after 10 minutes if the seizure continues * If seizure is persistent then IV **Phenobarbital or Phenytoin**
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What are some medical options for management of status epilepticus in the community?
Buccal midazolam Rectal diazepam
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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
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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.
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What is the definition of an Ischaemic stroke?
A sudden onset focal neurological deficit of vascular aetiology. With symptoms lasting >24 hours (or with evidence of infarction on imaging).
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What are the main causes of an Ischaemic Stroke?
* Large Vessel Atherosclerosis (50%) * Intracranial small vessel atherosclerosis (25%) * Cardio-embolic (AF) (20%)
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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
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What are the clinical features of a stroke?
* Limb weakness * Facial weakness * Dysphasia (speech disturbance) * Visual field defects * Sensory loss * Ataxia and vertigo (posterior circulation infarction) Stroke symptoms are usually asymmetrical
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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)
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What is the most sensitive test for confirming an ischamic stroke?
Diffusion Weighted MRI Only done if the diagnosis is unclear
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What are some post-acute ischaemic stroke investigations?
* Carotid ultrasound (critical carotid artery stenosis) * CT/MR angiography (intracranial and extracranial stenosis) * Echocardiogram (if a cardio-embolic source is suspected) * Vasculitis and Thrombophilia screens (in young patients)
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What are some post-acute Haemmorhagic stroke investigations?
Serum toxicology screen (sympathomimetic drugs (e.g. cocaine) are a strong risk factor)
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What is the acute management of an ischaemic stroke?
Once a head CT has ruled out haemorrhage: **Thrombolysis with Alteplase** (tissue plasminogen activator) if patients present within **4.5 hrs of symptom onset.** (Assuming there are no contraindications) **Mechanical Thrombectomy** in patients with an anterior circulation stroke (+ selected posterior circulation strokes) providing they have good baseline function. **300mg Aspirin for 2 weeks** started immedicately if thrombolysis not offered. Or 24hrs after thrombolysis.
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Give some contraindications for thrombolysis
* recent head trauma * GI or intracranial haemorrhage * recent surgery * Acceptable BP, platelet count, and INR
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# HALTSS What are the key steps for secondary stroke prevention?
* **Hypertension** - Anti-hypertensive therapy should be initiated 2 weeks post-stroke. * **Antiplatelet therapy** - Clopidogrel (75 mg once daily) or warfarin (in patients with a stroke seccondary to AF); started 2 weeks post-stroke. * **Lipid-lowering therapy** - High dose Atorvostatin (20-80mg) once nightly. * **Tobacco** - smoking cessation * **Sugar** - Diabetes Screen * **Surgery** - Carotid endarectomy in patients with ipsilateral carotid artery stenosis
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What are some stroke differential diagnoses?
* Hypoglycaemia * Drugs / Alcohol * Seizure * Migraine * Bell's Palsy * Tumour / Space occupying lesion * Meningitis / Encephalitis
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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.
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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.
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What are the risk factors for a haemmorhagic stroke?
* Age * Male sex * Family history of haemorrhagic stroke * Haemophilia * 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
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What is the clinical presentation of a Sub-arachnoid Haemmorhage?
* Severe "Thunderclap" Headache * Neck Stiffness and Photophobia * Kernig's sign * Brudinski's sign
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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
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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
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What is the management for a haemmorhagic stroke?
After a head CT: **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)
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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.
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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
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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
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What is Amaurosis Fungax?
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.
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What are some differential diagnoses for a TIA?
Ischaemic Stroke Haemorrhagic Stroke Migraine with Aura Focal Motor Seizures
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What is the main diagnostic investigation for a TIA?
MRI Scan
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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)
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What is the management for a TIA?
Immediate assessment (ideally within 24 hrs of symptoms) **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 Dyslipidaemia (start statin e.g. simvastatin 40mg) **Antiplatlet Therapy** Clopidogrel 300mg Aspirin 300mg **No driving until seen by specialist**
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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.
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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.
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What is a Lacunar Infarct defined by?
It can be any of: * A pure motor stroke * Pure sensory stroke * Sensorimotor stroke * Ataxic hemiparesis * Dysarthria-clumsy hand syndrome. A LACI affects small deep perforating arteries, typically supplying internal capsule or thalamus.
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What is a Posterior Circulation Infarct (POCI) defined by?
* Cerebellar dysfunction, OR * Conjugate eye movement disorder, OR * Bilateral motor/sensory deficit, OR * Ipsilateral cranial nerve palsy with contralateral motor/sensory deficit, OR * Cortical blindness/isolated hemianopia. A POCI involves the vertebrobasilar arteries and associated branches (supplying the cerebellum, brainstem, and occipital lobe).
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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) * **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
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Define Locked in Syndrome
It's Quadriparesis with preserved consciousness and ocular movements. Its caused by Basillar Artery Occlusion
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What is the clinical presentation of Wallenberg's Syndrome?
* Ipsilateral Horner's syndrome * Ipsilateral loss of pain and temperature sensation on the face * Contralateral loss of pain and temperature sensation over the contralateral body.
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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.
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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.
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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.
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What is the clinical presentation of Weber's Syndrome?
It causes an ipsilateral oculomotor nerve palsy and contralateral hemiparesis.
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Define Haemorrhage?
An active ongoing bleed
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Define Haematoma
A bleed that has mostly clotted and hardened
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Where can intracranial haemorrhages occur?
Extradural Subdural Subarachnoid Intracerebral
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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
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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.
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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.
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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
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What is the most common artery damaged in an EDH?
**Middle Meningeal Artery** due to damage to the temporoparietal region
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What are some rarer non-traumatic causes of an EDH?
Haemorrhagic tumour Coagulopathy Infection Vascular Malformation
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What are the risk factors for an EDH?
Younger age (20-30) Male Anticoagulant usage High contact/dangerous sports
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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.
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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
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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.
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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
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What does this show?
CT head showing an Extradural Haemorrhage
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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
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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
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Define a subdural haemorrhage (SDH)?
An accumulation of venous blood in the potential space between the dura mater and arachnoid mater of the brain.
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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.
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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**
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What blood vessel is the common culprit in a subdural haemorrhage?
Tearing of bridging veins due to shearing or deceleration injury
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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
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What is the clinical presentation of a subdural haemorhage?
Clinical presentation of a subdural haematoma varies but may include: * Headache * Nausea or vomiting * Confusion * Diminished eye, verbal, or motor response (Reduced GCS) * Focal neurological signs indicating the haematoma site. The presentation is **typically sub-acute (within 3 days to 3 weeks) or chronic (>3 weeks).**
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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.
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What is the diagnostic investigation for a subdural haemorrhage?
**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.
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What does this show?
A Subdural Haemorrhage
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What percentage of Subdural haemorrhages are bilateral in nature?
15% in adults 80% in infants
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What is the management of a Subdural Haemorrhage?
The management of a subdural haematoma depends on its stage: * **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.
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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.
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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
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Who do SAHs more commonly affect?
Black patients Female patients Age 45-70
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What is the commonest cause on an SAH?
80% of SAH is due to a ruptured **berry aneurysm** without trauma
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What are possible causes of an SAH?
* **Berry aneurysm rupture** * Idiopathic * AVM * Trauma
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What are some risk factors for SAH?
* Hypertension * Adult polycystic kidney disease * Increased Age >50yrs * Family History * Excessive alcohol consumption * Smoking **Strong associations with cocaine use and sickle cell disease**
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Where do berry aneurysms often occur?
At junctions of arteries within the Circle of Willis Most commonly the **Anterior Communicating Artery**
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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)** * Retinal haemorrhages as seen with ophthalmoscope examination * Localising focal neurological signs depending on the location of the bleed
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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
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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
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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
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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.
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What is the first line investigation for an SAH?
* **CT head** is the first-line investigation due to its high sensitivity, especially within the first 24 hours of symptom onset. * **Star shaped sign** * Diagnostic with 100% sensitivity if performed within 6 hours of onset.
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What are the investigations for an SAH?
* **CT head is the first-line investigation** * **Lumbar puncture** is used when CT head scan results are not definitive, with **CSF examination for xanthochromia. Only performed after 12 hours** of symptom onset if ICP is normal. * **CT Angiogram** is the next line to look for evidence of aneurysms or other vascular abnormalities
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What does this show?
Subarachnoid Haemorrhage
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What is the management of an SAH?
**1st line - Neurosurgery Referral:** * 1st - Endovascular coiling * 2nd - Surgical clipping * 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
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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
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What are some complications of an SAH?
Re-bleeding Cerebral ischaemia - due to vasospasm Hydrocephalus – due to blockage of arachnoid granulations Hyponatraemia - due to urinary salt loss
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What is the Cushing's Triad?
Hypertension (Wide Systolic Pulse Pressure) Bradycardia Respiratory irregularity
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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
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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
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Where do intracerebral haemorrhages occur?
* Lobar intracerebral haemorrhage * Deep intracerebral haemorrhage * Intraventricular haemorrhage * Basal ganglia haemorrhage * Cerebellar haemorrhage
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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.
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What is the epidemiology of GCA?
* GCA is the most common type of primary vasculitis. * It affects adults over the age of 60 and has a female preponderance. * Caucasian individuals have the highest incidence of disease. * Often associated with Polymyalgia Rheumatica
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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.**
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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
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What are some complications of GCA?
Permanent monocular blindness Stroke Aortic aneurysms
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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
355
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
356
What is the management of GCA?
**Immediate High dose Steroids** * **40-60mg prednisolone OD**: if no visual symptoms * **500mg-1000mg Methylprednisolone** if visual symptoms * Taper steroids gradually over 1-2 years once symptoms resolved **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
357
What are some complications of GCA?
Steroid-related complications (e.g., weight gain, diabetes and osteoporosis) Visual loss Cerebrovascular accident (stroke)
358
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.
359
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.
360
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
361
What condition is shown here?
Horner's Syndrome: * Ptosis * Miosis * Anhidrosis
362
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.
363
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
364
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.
365
What are the primary headaches?
**No underlying causes associated:** * Tension Headache * Migraine * Cluster Headache * Trigeminal Neuralgia
366
What are some causes of secondary headaches?
**Due to an underlying cause:** * Medication Overuse Headache * Vasculitis (Giant Cell Arteritis) * Sinusitis * Head Injury * Hormones
367
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%.
368
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
369
What are some potential triggers for Tension Headaches?
Stress Depression Alcohol Skipping meals Dehydration
370
What is the management of Tension Headaches?
* Reassurance * Simple Analgesia: **Paracetamol, Ibuprofen** * Addressing potential triggers such as stress
371
What medication may be used if Tension headaches are frequent or chronic?
Amitriptyline
372
What are Cluster Headaches?
Cluster headaches are severe and unbearable unilateral headaches, usually centred around the eye.
373
What is the epidemiology of Cluster Headaches?
Typically affect 30-50 year old males Smokers
374
What are some potential triggers for Cluster Headaches?
Alcohol Strong Smells Exercise
375
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
376
What is the management of Cluster Headaches?
* Avoid Triggers * **Acute Attacks:** **15L 100% oxygen** via a non-rebreathable mask and **Triptans** (Subcutaneous or nasal) * **Prophylaxis:** Verapamil
377
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.
378
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.
379
What is the Aetiology of Migraines?
Not fully understood. Likely combination or genetic and environmental triggering factors
380
# 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
381
What are the different types of Migraine?
Migraine without aura Migraine with aura Silent migraine (migraine with aura but without a headache) Hemiplegic migraine
382
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
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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.
384
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
385
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).
386
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**
387
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.
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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)
389
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**
390
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.
391
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
392
What are medication overuse headaches?
Medication overuse headache is associated with long-term use of analgesics.
393
What is the management of Medication overuse headaches?
The cornerstone of management involves discontinuation or reduction of the overused medication.
394
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.
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What is the Epidemiology of Trigeminal Neuralgia?
* Trigeminal neuralgia typically affects adults over the age of 50 * Higher prevalence in women.
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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
397
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.
398
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.
399
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.
400
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
401
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
402
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.
403
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.
404
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.
405
What are the different categories of diabetic peripheral neuropathy?
* Distal Symmetrical Sensory Neuropathy * Small-fibre Predominant Neuropathy * Diabetic Amyotrophy * Mononeuritis Multiplex * Autonomic Neuropathy
406
What is the most common type of diabetic peripheral neuropathy?
Distal Symmetrical Sensory Neuropathy
407
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.
408
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.
409
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.
410
How does Mononeuritis Multiplex present?
* Typically painful. * Defined as neuropathies involving two or more distinct peripheral nerves.
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How does Autonomic Neuropathy present?
Presents with postural hypotension, gastroparesis, constipation, urinary retention, arrhythmias, and erectile dysfunction.
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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.
413
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.
414
What are some complications of diabetic peripheral neuropathy?
* Foot ulcers Cardiac, GI and genitourinary disturbances due to autonomic neuropathy
415
What is the management of Diabetic peripheral neuropathy?
**Control of blood glucose levels to slow disease progression** * Pain control: **Gabapentin, Pregabalin**
416
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.
417
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.
418
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.
419
What is the main differential diagnosis to rule out in Charcot Arthropathy?
Osteomyelitis Will typically also cause systemic symptoms and increase inflammatory markers
420
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.
421
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.
422
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.
423
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.
424
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
425
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.
426
What is the epidemiology of Meniere's disease?
* Individuals between 30-60 * Predominantly affects only one ear
427
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.
428
What is the classical triad of symptoms caused by Meniere's disease?
Hearing loss Vertigo Tinnitus
429
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
430
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
431
What are the investigations for Meniere's disease?
**Clinical Diagnosis usually made by an ENT specialist** * Audiometric testing * Other imaging or laboratory tests may be used to rule out other potential causes of symptoms
432
What is the management for Meniere's Disease?
* Prophylactic use of **betahistine** 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.
433
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.
434
What is the epidemiology of Delirium?
The prevalence increases with age, severity of illness and the presence of pre-existing cognitive impairment.
435
# DELIRIUMS What are the main causes of Delirium?
**Drugs and Alcohol** (Anti-cholinergics, opiates, anti-convulsants, recreational) **Eyes, ears and emotional disturbances** **Low Output state** (Myocardial Infarction, Acute Respiratory Distress Syndrome, Pulmonary Embolism, Congestive Heart Failure, Chronic Obstructive Pulmonary Disease) **Infection** **Retention** (of urine or stool) **Ictal** (related to seizure activity) **Under-hydration/Under-nutrition** **Metabolic disorders** (Electrolyte imbalance, thyroid disorders, Wernicke's encephalopathy) **Subdural hematoma, Sleep deprivation**
436
What is the clinical presentation of Delirium?
* Disorientation * Hallucinations * Inattention * Memory problems * Change in mood or personality * Disturbed sleep Patients can be hypoactive (sedated) or hyperactive (very agitated), and these presentations can fluctuate over time.
437
What are some differential diagnoses of Delirium?
Dementia Psychosis Depression Stroke
438
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
439
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
440
What pharmacological management can be used for delirium?
Small doses of haloperidol or olanzapine. To be used with caution and only in patients who are extremely agitated and present a danger to themselves or others
441
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)
442
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.
443
What gender is more commonly affected by CFS?
Women more than men
444
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
445
What are the clinical features of CFS?
* Extreme fatigue * Post-exertional malaise * Sleep disturbances * Cognitive impairment
446
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.
447
What are the investigations for CFS?
**Clinical diagnosis** based on the patients history and symptomatology * Blood tests: to rule out inflammation, infection, blood cell abnormalities * Thyroid function tests: Exclude hypothyroidism
448
What is the management of CFS?
There is no curative treatment and management strategies focus on symptom relief * **Graded exercise therapy (GET):** A tailored exercise program that gradually increases in intensity. * **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.
449
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.**
450
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.
451
What are the clinical features of Wernicke's Encephalopathy?
Characteristic Triad of: * **Ataxia:** Unsteady and uncoordinated movements * **Confusion:** Disorientation and difficulty with attention * **Ocular abnormalities:** This can include gaze-evoked nystagmus, spontaneous upbeat nystagmus, and horizontal or vertical ophthalmoplegia.
452
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.
453
What are the investigations for Wernicke's Encephalopathy?
**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. **Blood tests:** Although not definitive, they can reveal low thiamine levels and other signs of malnutrition or alcohol abuse.
454
What is the management of Wernicke's Encephalopathy?
**IV Pabrinex** (High dose thiamine replacement)
455
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
456
Define Narcolepsy
A chronic neurological disorder of the sleep boundary which affects the control of sleep and wakefulness with REM sleep intrusion into the wake state.
457
What is the pathophysiology of Narcolepsy?
Loss of hypocretin producing neurons in the hypothalamus leads to the dysregulation of wakefulness and sleep.
458
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
459
What is the Aetiology of Narcolepsy?
Unknown CNS pathology such as head trauma, stroke, CNS infections, MS have been correlated to lead to Narcolepsy syndromes.
460
What are the clinical features of Narcolepsy?
Tetrad of: * **Excessive Daytime Sleepiness:** 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
461
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
462
What are the investigations for Narcolepsy?
**1st Line:** * Acitgraphy and sleep diary * Overnight Polysomnography * Multiple Sleep Latency Tests (MSLT)
463
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
464
What is the conservative management of Narcolepsy?
Strict sleep schedule Avoid sleep deprivation Avoid alcohol and smoking Avoid late night exercise
465
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/SSRIs/TCAs
466
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.
467
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
468
What type of Hypersensitivity reaction is Myasthenia Gravis?
Type 2
469
What are the risk factors for MG?
Females FHx Autoimmunity Thymoma/ Thymic Hyperplasia
470
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.
471
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**
472
Give some examples of drugs that can exacerbate Myasthenia Gravis?
Beta blockers Lithium Penicillamine Gentamicin Quinolones Phenytoin
473
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.
474
What are the primary investigations for Myasthenia Gravis?
**Bedside tests:** Ice-pack test **Blood tests:** Testing for **serum acetylcholine receptor antibody and muscle-specific tyrosine kinase antibodies** **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
475
What is the management of Myasthenia Gravis?
Regular Medical Reviews MDT involvement. **Medical management:** * Acetylcholinesterase inhibitors: **Pyridostigmine, Neostigmine** * Immunosuppressive therapy: **Prednisolone** * Acute cases may require intravenous immunoglobulin (IVIG) or plasmapheresis in severe, steroid-refractory, cases. **Surgical management:** * Thymectomy may be considered in patients with thymic hyperplasia or thymoma.
476
What is the main complication of MG?
Myasthenic Crisis: Severe acute worsening of Sx Often Triggered by another illness (URTI) Severe Respiratory weakness
477
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**.
478
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.
479
What is the cause of Lambert Eaton Syndrome?
Unclear but likely a paraneoplastic syndrome: Typically occurs in Px with Small Cell Lung Cancer (SCLC)
480
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
481
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
482
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
483
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.
484
What are the different types of Neurofibromatosis?
Type 1 (NF1): Due to a loss of function mutation in the neurofibromin gene on chromosome 17 Type 2 (NF2): Due to a lose of function mutation in the Schwannomin (Merlin) tumour suppressor gene on chromosome 22
485
What is the epidemiology of Neurofibromatosis?
Type 1 is more common than type 2 NF1 is also known as **Recklinghausen's Disease**
486
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 * **Learning difficulties** * **Hypertension** * **Gastrointestinal issues:** Bleeding or obstruction due to tumours in the bowel. * **Epilepsy:** Due to tumours in the brain.
487
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**
488
What condition is this?
Neurofibromatosis
489
What is this cutaneous sign and what condition does it suggest?
Cafe-au-lait spot Suggests neurofibromatosis **May also suggest other conditions such as Legius syndrome or McCune-Albright syndrome**
490
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.
491
What condition is particularly associated with neurofibromatosis type 2?
Acoustic neuromas. **A patient with Bilateral acoustic neuromas almost certainly has NF2**
492
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.
493
How is Neurofibromatosis diagnosed?
**Clinical diagnosis** Genetic testing may be used to confirm the diagnosis
494
# CRABBING What is the Diagnostic criteria for a clinical diagnosis of Neurofibromatosis type 1?
**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
495
What is a neurofibroma?
* They are skin-coloured, raised nodules or papules with a smooth, regular surface. * A single skin neurofibroma without other features does not indicate neurofibromatosis. * **Two or more are significant.** * A plexiform neurofibroma is a larger, irregular, complex neurofibroma containing multiple cell types. * **A single plexiform neurofibroma is significant.**
496
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.
497
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)
498
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.**
499
What is the Epidemiology of NPH?
More common in older adults Considered one of the potentially reversible causes of dementia
500
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
501
# Wet, Wacky, Wobbly What are the clinical features of NPH?
Classical triad: * **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.
502
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.
503
What are the investigations for NPH?
**Neuroimaging:** CT/MRI shows dilated lateral ventricles and enlarged 4th ventricle **Lumbar Puncture**
504
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.
505
What are some complications of a Ventriculoperitoneal shunt?
Infection Blockage Excessive drainage **Intraventricular haemorrhage** during shunt related surgery
506
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**
507
What is the epidemiology of Meralgia Paraesthetica?
* Middle age (30-60 years) * No significant gender predilection
508
What are some risk factors for Meralgia Paraesthetica?
Anything that increases pressure on the grain area: * Obesity * Pregnancy * Diabetes * Wearing tight belts or clothing
509
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
510
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
511
What are the clinical features of Meralgia Paraesthetica?
* **Neuropathic pain, numbness, paraesthesia** in the distribution of the lateral femoral cutaneous nerve, primarily the outer aspect of the thigh. * Symptoms may be exacerbated by standing, walking, or hip extension. **Sensory loss may occur but motor function is unaffected.**
512
What are some symptoms a patient may describe in Meralgia Paraesthetica?
Burning Numbness Pins and Needles Cold sensation Loss of sensation Localised hair loss **Symptoms worsen when walking or standing for long duration or with extension of the hip**
513
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.
514
What are the investigations for Meralgia Paraesthetica?
**Clinical Diagnosis based on patient history and examination** * **Nerve conduction studies and electromyography (EMG):** To rule out other causes of neuropathy. * **Imaging studies (CT or MRI):** To rule out other causes of compressive neuropathy if suspected.
515
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
516
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
517
What is the surgical management of Meralgia Paraesthetica?
Decompression – removing pressure on the nerve Transection – cutting the nerve Resection – removing the nerve
518
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.
519
What is the epidemiology of SCC?
Common neurosurgical condition Often seen in patients with a history of malignancy
520
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.
521
What are the clinical features of SCC?
* **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.
522
What are some differential diagnoses for SCC?
* **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
523
What are the investigations for SCC?
**Urgent whole spine MRI done in anyone with features suggestive of SCC or Cauda Equina Syndrome (CES) within 48 hours**
524
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)
525
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 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.
526
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)
527
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
528
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
529
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
530
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.
531
What is the **Gold standard** investigation for CES?
Urgent **Whole spine MRI** to identify the cause and location of the compression
532
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)**
533
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.
534
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.
535
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.
536
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
537
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.
538
What is the aetiology of an ASAI?
* **Damage to the aorta** * Aortic aneurysm repair * Aortic dissection * Atherosclerosis * Cardiac arrest * Cardiac emboli * Vasculitis * Shock
539
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.
540
What are the clinical features of an ASAI?
* Loss of pain sensation (analgesia) * Loss of temperature sensation (thermoanaesthesia) * Motor function impairment * Loss of autonomic functions
541
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.
542
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.
543
What are some complications of an ASAI?
Persistent neurological deficits Chronic pain Urinary and bowel dysfunction Pressure sores from prolonged immobility
544
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
545
What is the length of the spinal cord?
From C1-L1/2
546
Where is the conus medullaris and cauda equina?
At L1/2
547
Define Hemiplegia?
Paralysis to one side of the body (usually due to a brain lesion)
548
Define Paraplegia?
Paralysis of both legs/lower body (usually due to a spinal cord lesion)
549
What does the DCML tract convey information for?
Ascending tract for fine touch, 2pt discrimination and proprioception.
550
What is the pathway for the DCML
Travels in dorsal route (Fasciculus Gracilis/Cuneatus) Decussates in the medulla
551
What does the spinothalamic tract convey information for?
Ventral: Crude Touch and pressure Lateral: Pain and temperature
552
What is the pathway for the spinothalamic tract?
Ascending: Enters at spinal level of nerve ascends 1-2 spinal levels and then decussates
553
What information does the corticospinal tract convey?
Upper motor neurons for movement. Decussates at the medulla
554
What are the nerve routes for the Knee jerk reflex?
L3/4
555
What are the nerve routes for the Big toe reflex?
L5
556
What are the nerve routes for the ankle Jerk reflex?
S1/2
557
What are the commonest causes of spinal cord injury?
Trauma Vertebral compression fractures Intervertebral disc disease - prolapse/herniation Tumours Infection
558
What are the common vertebral body neoplasms that can cause spinal compression?
Mets from: Lungs Breast RCC Melanoma
559
What are the different types of spinal cord injury?
Complete SC injury Anterior SC injury Posterior SC injury Central SC injury Brown-Sequard Syndrome
560
What is Spinal Cord Compression?
Compression of the spinal cord resulting in upper neurone signs and specific symptoms dependent on where compression is
561
What is the commonest cause of acute spinal cord compression?
Vertebral body neoplasms
562
What is the concern if there is sphincter involvement in spinal cord compression
This is a late and bad sign signalling a poorer prognosis
563
What would be the features of a complete spinal cord compression?
All motor and sensory function below the SCI level
564
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)
565
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)
566
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.
567
What is Sciatica?
Sciatica refers to the symptoms associated with irritation of the sciatic nerve.
568
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
569
What is the innervation of the sciatic nerve?
The sciatic nerve supplies sensation to the lateral lower leg and the foot. (below the knee) It supplies motor function to the posterior thigh, lower leg and foot. (Hamstring muscles and all muscles below the knee)
570
What nerves are branches of the sciatic nerve?
Common peroneal nerve Tibial nerve
571
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
572
What are the main causes of Sciatica?
**Intervertebral Herniated/prolapsed disc** Tumours Piriformis Syndrome Spondylolisthesis Spinal stenosis
573
What is Bilateral sciatica a red flag for?
Cauda Equina syndrome
574
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
575
What is the main treatment for sciatica?
Physiotherapy + Analgesia: Amitriptyline (TCA) Duloxetine (SNRI)
576
What is Brown Sequard Syndrome?
Hemi-section of the spinal cord and therefore loss of sensations of pain temperature and touch and motor movement
577
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
578
What is the Investigation and Treatment for Brown Sequard Syndrome?
Ix - MRI Spine Tx - Supportive (physical/occupational therapy) and Steroids (Dexamethasone)
579
What are the signs of a Tibial Nerve Lesion?
L4-S3 (originals from sciatic nerve above the knee) Inability to: Plantarflexion Invert foot Flex toes Sensory loss over sole of foot
580
What are the signs of a Common Peroneal Nerve Lesion?
L4-S1 (Originates from sciatic nerve just above the knee) Foot drop Weak ankle dorsiflexion and eversion Sensory loss over dorsal foot If Ankle inversion/hip adduction affected it could be L5 radiculopathy
581
What are the side effects Amitriptyline?
constipation. Blurred vision/dizziness dry mouth. feeling sleepy. Confusion Urinary retention headache
582
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.
583
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.
584
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)
585
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
586
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)
587
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.
588
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 **MRI or CT scan:** to identify any anatomical causes of nerve injury such as a fracture or mass lesion
589
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
590
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.
591
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)
592
What is the innervation of the ulnar nerve?
Muscular branch = innervates muscles of the anterior compartment 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**
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What are the clinical features of an ulnar nerve injury?
* Weakness or paralysis of the muscles innervated by the ulnar nerve (e.g., most of the intrinsic hand muscles and two muscles in the forearm) * Numbness, tingling, or pain in the sensory distribution of the ulnar nerve (skin of the medial 1.5 digits and associated palm area)
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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.
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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
596
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
597
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.
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What is the epidemiology of Carpal Tunnel Syndrome?
Most common mononeuropathy
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# 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
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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.
601
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.
602
What are the investigations for Carpal Tunnel Syndrome?
* **Clinical Examination:** * Phalen's Test * Tinel's Test * Compression test * **Electromyography (EMG):** to assess the electrical activity of the muscles at rest and during contraction. * **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.
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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.
604
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.
605
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
606
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
607
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
608
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
609
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
610
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.
611
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.
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# 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
613
# 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
614
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)
615
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.
616
What is the most common Radiculopathy?
Lumbar radiculopathy (L5 radiculopathy)
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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.**
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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. * Muscle spasms.
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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
620
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.