Neurology

Question 1

What are the risk factors for developing Alzheimer’s disease?

Answer 1

Age
CVD
Depression
Low educational attainment
- Low social engagement and support
- head trauma, learning difficultie

Question 2

What is dementia?

Answer 2

• a state characterised by impairment of 2 or more cognitive domains
• persisting for more than 6 months
• severe enough to impact on ability to function
• not primarily attributed to another mental disorder

Question 3

what is mild cognitive impairment?

Answer 3

• a state characterised by impairment of one or more cognitive domains
• not severe enough to impact on ability to function
• synonymous with ‘prodromal’
  (preclinical –> prodromal –> symptomatic)
  (symptomatic is then alzheimer’s)

Question 4

What are the cognitive domains?

Answer 4

• memory: semantic (worldly, no context)/episodic (autobiographical)
• visuospatial: perception/contruction/praxis
• language: naming/fluent/structure/comprehensive
• executive function: planning/ working memory/ inhibition
• social cognition/behavioural: theory of mind/empathy/insight
• attention: speed/multitasking

Question 5

What genes put you at risk of AD?

Answer 5

APP and presenilin genes (PSEN1, PSEN2)
- APP on chromosome 21, so increased risk for people with down syndrome

alleles of apolipoprotein E4 = not as good at clearing beta plaques

Question 6

What are the amyloid beta plaques and how do they contribute to AD?

Answer 6

1. Senile plaques deposits of beta amyloid aggregate.
2. Amyloid precursor protein is found on neurons (helps it to grow and repair after injury).
3. If it is broken down normally by alpha secretase and gamma secretase it is soluble and easy to remove
4. However if it is broken by beta secretase the leftover fragment isn’t soluble and creates a monomer called beat amyloid.
5. These are sticky and can in between neurones and their signalling, They also increase inflammation which damages surrounding neurones

Question 7

What are neurofibrillary tangles and how do they contribute to AD?

Answer 7

Neurons held together by cytoskeleton = is made up of microtubules a protein ‘tau’ makes sure they stay together.
Beta amyloid build-up outside the neurones initiates pathways which leads to the activation of kinase.

This leads to the phosphorylation of tau

The tau protein changes shape, stops supporting the microtubules, and clumps up with other tau proteins, forming neurofibrillary tangles

Neurones with tangles and non-functioning microtubules can’t signal as well, and sometimes end up undergoing apoptosis.

As neurones die, the brain starts to atrophy.

Question 8

What are some of the symptoms of AD?

Answer 8

• Agnosia- can’t recognise things
• Apraxia can’t do basic motor skills
• Aphasia speech difficulties
• Cognitive impairment
• Agitation and emotional lability
• Depression and anxiety
• Sleep cycle disturbance:
• Motor disturbance: wandering is a typical feature of dementia

Question 10

What are the symptoms of episodic memory loss?

Answer 10

Repetitive in conversation
- struggles to follow plot of TV shows
- May forget appointments
- increasingly reliant on partner, lists
- limited knowledge of current affairs

signs
- poor retention of address
- cannot recall breakfast
- cannot recall pictures from naming test
- Ribbot’s phenomena = better remote long term memory than what they did yesterday

Question 11

What are causes of ‘rapid onset dementias’?

Answer 11

CJD
Vasculitis
Limbic encephalitis
SOl
seizures
infections
metabolic = Wernicke’s
Normal pressure hydrocephalus

usually treatable so want to diagnose quickly

Question 12

How can the activities of daily life be affected in AD?

Answer 12

Loss of independence: increasing reliance on others for assistance with personal and domestic activities:

Early stages: problems with higher level function (e.g. managing finances, difficulties at work)
Later stages: problems with basic personal care (e.g. washing, eating, toileting) and motor function (e.g. walking, transferring)

Question 13

What is the diagnostic criteria for AD?

Answer 13

Functional ability: inability to carry out normal functions

Impairment in 2 or more cognitive domains

Differentials excluded

Question 14

What imaging is used for AD?

Answer 14

CT/MRI:
exclude other diagnosis
help determine type of dementia;
will show medial temporal lobe atrophy

Question 15

What is the definitive diagnosis for AD

Answer 15

Brain biopsy after autopsy

Question 16

What are the two cognitive assessments that can be performed to assess AD?

Answer 16

Mini mental state examination (MMSE)
Montreal cognitive assessment scale (MoCA)

Primary care: 6 Cognitive Impairment Test (6CIT)

ACE II

Question 17

What is assessed in the cognitive assessments?

Answer 17

Attention and concentration
Recent and remote memory
Language
Praxis (planned motor movements)
Executive function
Visuospatial function

Question 18

What is the treatment for AD?

Answer 18

Non-pharmacological:
programmes to improve/maintain cognitive function

Mild to moderate AD:
acetylcholinesterase inhibitors e.g., Donepezil and rivastigmine

Moderate to severe AD:
N-methyl-d-aspartic acid receptor antagonist memantine (anti-glutamate transmitter)

Question 19

What is Parkinson’s disease (PD)

Answer 19

A neurodegenerative disorder characterised by the loss of dopaminergic neurons within the substantia nigra pars compacta (SNPC) of the basal ganglia.

Question 20

What are the risk factors for developing PD?

Answer 20

Age: prevalence is 1% in 60-70 and 3% in those above 80
Gender: men are 1.5 times more likely than females to develop PD
Family history

Question 21

Describe the pathophysiology of PD

Answer 21

progressive loss of dopamine-producing neurons meaning there is a reduction in the amount of dopamine produced at the substantia nigra
Loss of these neurons results in reduction in action of the direct pathway and a resultant increase in the antagonistic indirect pathway which has a restrictive action on movement.
Therefore bradykinesia and rigidity are key symptoms
There is also formation of protein clumps Lewy bodies

Question 22

What are the 3 key presentations of PD?

Answer 22

Bradykinesia
Tremor
Rigidity

PD symptoms usually start unilaterally and then become bilateral later in the disease course.

Question 23

What does the bradykinesia look like in PD?

Answer 23

Handwriting gets smaller
Only take small steps (shuffling gait)
Difficulty initiating movement
Difficulty turning around when standing
Reduced facial movements and expressions

Question 24

What does the tremor look like in PD?

Answer 24

• A unilateral resting tremor.
• Described as pill rolling tremor looks like they are rolling pill between thumb and finger
• The tremor is worse at resting and when they are distracted like using the other hand
• Frequency of 4-6 times a second

Question 25

What is the difference between an essential and Parkinson’s tremor?

Answer 25

Essential - active tremor + worsens with movement Parkinson’s - pill rolling, resting tremor

Question 26

What is the rigidity like in PD?

Answer 26

if you take their hand and passively flex and extend their arm at the elbow you will feel tension in their arm that gives way to movement in small increments (little jerks) Described as cogwheel

Question 27

What are some other symptoms of PD?

Answer 27

Depression Sleep disturbance and insomnia no REM Loss of sense of smell Postural instability Cognitive impairment and memory problems

Question 28

What are the differences between a PD tremor and a benign essential tremor?

Answer 28

PD= asymmetrical BET= symmetrical PD= frequency= 4-6 BET= 5-8 PD= worse at rest BET= better at rest PD= improves with intentional movement BET= worse PD= no change with alcohol BET= better with alcohol

Question 29

What is used to diagnose PD?

Answer 29

PD is a clinical diagnosis and should be suspected in a patient with bradykinesia and at least one of the following: - Tremor - Rigidity - Postural instability

Question 30

What is the management for PD?

Answer 30

Motor symptoms NOT affecting quality of life: A choice of one of the following: 1. Dopamine agonist (non-ergot derived) - Pramipexole, ropinirole 2. Monoamine oxidase B inhibitor - Selegiline, rasagiline - Stop breakdown of circulating dopamine 3. Levodopa Motor symptoms affecting the quality of life: 1. L.Dopa Synthetic dopamine levodopa given with a drug that stops it being broken down. These are peripheral decarboxylase inhibitors: - Carbidopa and benserazide. - Co-benyldopa (levodopa and benserazide) - Co-careldopa (levodopa and carbidopa)

Question 31

What is the prognosis for PD?

Answer 31

PD is a chronic and progressive condition with no cure. Overall, life expectancy is reduced with the mortality being 2-5 times higher for those aged 70-89 years old. Also, the risk of dementia is up to 6 times higher in PD patients.

Question 32

Management: Essential Tremor

Answer 32

The tremor is not harmful and does not require treatment if it is not causing functional or psychological problems. Medications that may improve symptoms are: - Propranolol (a non-selective beta blocker) - Primidone (a barbiturate anti-epileptic medication)

Question 33

What can cause vascular dementia?

Answer 33

Ischaemic stroke Small vessel disease Haemorrhage Other: cerebral amyloid, which is a cause of small vessel disease. Deposition of amyloid in small arteries. CADASIL, which is due to mutation in the NOTCH3 gene and leads to arterial thickening and occlusion.

Question 34

Describe the pathophysiology of VD?

Answer 34

1. Once blood supply to the brain falls below the demands to the tissue, it’ considered an ischaemic stroke. 2. The tissue damage is permanent because the dead tissue liquifies in a process called *liquefactive necrosis** 3. Brain tissue necrosis leads to a loss of mental function in the area where the loss has occurred

Question 35

How do the symptoms of VD appear?

Answer 35

They appear suddenly and the brain function decline is STEP-WISE e.g. it decreases with each stroke Symptoms will vary depending on which vessels are affected in stroke/atherosclerosis

Question 36

What is motor neurone disease?

Answer 36

Motor neurone disease is an umbrella term that encompasses a variety of specific diagnoses. They are neurodegenerative diseases that affect both upper and lower motor neurones but sensory neurons are spared

Question 37

What is the most common type of Motor Neurone Disease?

Answer 37

Amyotrophic lateral sclerosis (ALS) accounts for 50% of cases

Question 38

What are some other types of MND?

Answer 38

Progressive muscular atrophy: LMN only Primary lateral sclerosis: UMN only Progressive bulbar palsy: affects muscles of talking and swallowing (second most common) LMN only ALS: affects both UMN and LMN

Question 39

What gene is implicated in some cases of ALS?

Answer 39

SOD1

Question 40

What are the risk factors for MND?

Answer 40

Increasing age (over 60) Male Pesticides Heavy metals Rugby

Question 41

What are the general symptoms of MND?

Answer 41

- 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). - struggle to breathe especially at night due to only relying on diaphragm to breathe and it weakens earlier (wake up with headache)

Question 42

What are the signs of LMN disease?

Answer 42

Muscle wasting Reduced tone Reduced reflex Fasciculations Loss of power Babinski reflex negative

Question 43

What is the cause of LMN injury?

Answer 43

motor neurone - MND< spinal muscular atrophy, polio infection motor nerve roots - radiculopathy, guillain barre motor nerves - neuropathies Neuromuscular junction - myasthenia gravis Muscles disorder - myositis, myopathies

Question 44

What are the signs of a UMN lesion?

Answer 44

Increased tone (no UMN to stop actions of LMN) Brisk reflexes Rigidity + spasticity Babinski reflex positive

Question 45

What is never affected in MND?

Answer 45

Eye muscles (oculomotor movements) Sensory function and sphincters (onuf’s nucleus)

Question 46

What are the symptoms of MND?

Answer 46

Progressive weakness Fatigue - nocturnal respiratory insufficiency (so wake up in night when CO2 builds up) Falls Speech and swallow issues

Question 47

What are the investigations for MND?

Answer 47

MND is a clinical diagnosis Electromyography: in MND there will be evidence of fibrillation potentials Nerve conduction studies: may show modest reductions in amplitude MRI spine: imaging can help exclude spinal pathology which may mimic MND, such as cervical cord compression and myelopathy

Question 48

What are some differentials for MND?

Answer 48

MS Polyneuropathies Myasthenia gravis GBS

Question 49

What is the management for MND

Answer 49

No treatment - large amount of neurones have died before symptoms present 1. Riluzole = prolongs survival by 2-4 months by protecting motor neuron damage form glutamate Respiratory support: patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months Supportive treatment: Antispasmodics: such as baclofen

Question 50

What is the main cause of death in motor neurones disease?

Answer 50

death 75-80% within 5 years - most commonly due to respiratory failure

Question 51

What is Multiple Sclerosis?

Answer 51

An autoimmune cell-mediated demyelinating disease of the central nervous system

Question 52

What are some risk factors for developing MS?

Answer 52

Vitamin D deficiency Family history: HLA-DR2 is implicated; 30% monozygotic twin concordance EBV infection: the virus with the greatest link to MS Smoking Obesity

Question 53

Describe the pathophysiology of MS

Answer 53

(don’t fully know the cause) T-cells get through the blood brain barrier and are activated by myelin. The T-cell then changes the BBB to allow more immune cells to get in the brain MS is a type IV hypersensitivity reaction: T-cells release cytokines and these recruit more immune cells whilst also damaging the oligodendrocytes (+ axones) B-cells will make antibodies that will destroy the myelin of the oligodendrocytes. leaving behind areas of plaque/sclera

Question 54

How does MS progress over time?

Answer 54

1. In early disease, re-myelination can occur and symptoms can resolve. (thin myelin forms so conduction reduced in heat) 2. In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent. 3. A characteristic feature of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time MS lesions change location over time is that they are

Question 55

What are the different types of MS?

Answer 55

Relapsing-remitting: Secondary progressive Primary progressive Progressive relapsing Clinically isolated syndrome (kind of counts)

Question 56

What is Relapsing-remitting: MS?

Answer 56

The most common pattern 85% of cases Episodic flare-ups separated by periods of remission. There isn’t full recovery after flare ups so disability increases over time. 60% will develop secondary within 15 years

Question 57

What is secondary progressive MS?

Answer 57

Initially, the disease starts with a relapsing-remitting course, but then symptoms get progressively worse with no periods of remission

Question 58

What is primary progressive MS?

Answer 58

Symptoms get progressively worse from disease onset with no periods of remission Accounts for 10% of cases and is more common in older patients

Question 59

What is Progressive relapsing MS?

Answer 59

One constant attack but there are bouts superimposed during which the disability increases even faster

Question 60

What is clinically isolated syndrome MS?

Answer 60

This describes the first episode of demyelination and neurological signs and symptoms. - MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”. - Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress to MS

Question 61

What are the signs and symptoms of MS?

Answer 61

1. Optic nerve affected: Optic neuritis Eye movement abnormalities- double vision VI nerve 2. Spinal cord affected: - Focal weakness (incontinence, limb paralysis, Bells palsy) - Focal sensory symptoms ( numbness, pins and needles, - Lhermitte’s sign is an electric shock sensation that travels down the spine and into the limbs when flexing the neck.) 3. Cerebellar white matter affected Ataxia 4. Cerebral hemispheres: - large variety but can be silent 5. Medulla + pons - dysarthria, double vision, vertigo, nysatgmus 6. Uhthoff’s phenomenon: worsening of symptoms following a rise in temperature, such as a hot bath

Question 62

What is optic neuritis?

Answer 62

Demyelination of the optic nerve which present with: - Unilateral reduced vision - Central scotoma (enlarged blind spot) - Pain on eye movement - Impaired colour vision (red)

Question 63

What is the primary investigation for MS and what would it show?

Answer 63

MRI of brain and spine: - Will show demyelinating plaques called Dawson’s fingers - High signal L2 lesions - Old lesions will not enhance with contrast, whereas newer lesions will. This provides evidence of dissemination of lesions in time and space which is required for a diagnosis of MS

Question 64

What other investigations are done for MS?

Answer 64

Lumbar puncture - can detect oligoclonal IgG bands in the CSF Nerve conduction studies - delayed conduction speeds Evoked Potentials (VEP) - measures speed of messages along nerves

Question 65

What is the diagnostic criteria used to diagnose MS?

Answer 65

McDonald criteria

Question 66

What is the McDonald criteria based on?

Answer 66

2 or more relapses and either: - Objective evidence of two or more lesions - Objective evidence of one and a reasonable history of a previous relapse ‘Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potentials

Question 67

What is used to treat a MS relapse?

Answer 67

Oral or IV methylprednisolone (reduce time to recover from illness but don’t treat illness) Plasma exchange: to remove disease-causing antibodies

Question 68

What is used for maintenance of MS?

Answer 68

Disease-modifying drugs Beta-interferon: decreases the level of inflammatory cytokines Monoclonal antibodies e.g. alemtuzumab (anti-CD52) and natalizumab (anti-α4𝛃1-integrin) Glatiramer acetate: immunomodulator drug which acts as a ‘decoy’ Fingolimod: a sphingosine-1-phosphate receptor modulator that keeps lymphocytes in lymph nodes so they can’t cause inflammation

Question 69

What is used to treat muscle spasticity in MS?

Answer 69

- baclofen - gabapentin

Question 70

Why is stem cell transplant used in MS?

Answer 70

- use chemotherapy to kill off all their immune cells and give them a stem cell transplant - MS is a inflammatory disease so new immune cells should not attack myelin - doesn’t improve demyelination as damage is already done but stops the disease progressing

Question 71

What are some complications of MS?

Answer 71

Genitourinary: urinary tract infections, urinary retention and incontinence Constipation Depression: offer mental health support if required Visual impairment Mobility impairment: offer physiotherapy, orthotics and other mobility aids Erectile dysfunction

Question 72

What is Huntington’s disease?

Answer 72

An autosomal dominant genetic neurodegenerative condition that causes a progressive a progressive deterioration in the nervous system.

Question 73

What causes HD?

Answer 73

- It is a trinucleotide repeat disorder that involves a genetic mutation in the HTT gene on chromosome 4 - There is a repeat of CAG which codes for glutamine 36 times in a row so patients have 36 glutamine in a row on the Huntington protein - These mutated proteins aggregate within neuronal cells of the caudate and putamen causing neuronal cell death. - This leads to decreased ACh and GABA synthesis. - This leads to dopamine increase leading to excessive movement

Question 74

What is anticipation and how is it linked to HD?

Answer 74

It is a feature of trinucleotide repeat disorders. When copying the HTT gene, DNA polymerase can lose track of which CAG it’s on and so add extra CAGs. This is called repeat expansion. This leads to successive generations having more repeats in the gene resulting in: - Early age of onset - Increased severity of the disease

Question 75

What are the symptoms of HD?

Answer 75

Patients will be asymptomatic until 30-50. Symptoms: - Begin with Cognitive, psychiatric or mood problems - Chorea (involuntary, abnormal movements) - Eye movement disorders - Dysarthria: speech difficulties - Dysphagia: swallowing difficulties - Dementia

Question 76

How is diagnosis of HD made?

Answer 76

Made at a specialist genetic centre that looks for the number of CAG repeats. This will involve pre and post test counselling

Question 77

What is the treatment for HD?

Answer 77

No treatment - supporting person and family - maintaining quality of life = OT + PT - speech and language therapy - genetic counselling - end of life care planning

Question 78

What medications are given for symptoms relief in huntington’s?

Answer 78

Medications that can suppress the disordered movement: Antipsychotics (e.g. olanzapine) Benzodiazepines (e.g. diazepam) Dopamine-depleting agents (e.g. tetrabenazine)

Question 79

What is meningitis?

Answer 79

Meningitis refers to inflammation of the pia and arachnoid mater. Micro-organisms infect the cerebrospinal fluid (CSF).

Question 80

What are the 3 main features of ‘meningism’?

Answer 80

Stiffness of the neck Photophobia Severe headache

Question 81

What other symptoms would present with bacterial or viral meningitis?

Answer 81

Fever Feel unwell Rash (characteristically haemorrhagic in meningococcal meningitis)

Question 82

What are the viral causes of meningitis?

Answer 82

80% viral - Enteroviruses (including Echo virus, Coxsackie virus) - Herpes simplex virus - Mumps virus - varicella zoster virus - Lymphocytic chorio meningitis virus And historically, Poliovirus (also an enterovirus)

Question 83

What are the main bacterial causes of meningitis for each age group?

Answer 83

Neonates = E.coli, Group B strep, Listeria monocytogenes Infants = N.meningitis + H.influenza, Strep pneumoniae Adults = N.meningitis + S.pneumoniae Elderly = S. pneumoniae + N.meningitis + listeris monocytogenes

Question 84

What are the causes of fungal meningitis?

Answer 84

Cryptococcus neoformans Candida

Question 85

What are some risk factors for developing meningitis?

Answer 85

Immunocompromised: such as being in the extremes of age, infection (HIV), and medication (Chemotherapy) Listeria monocytogenes M. Tuberculosis Non-immunised: at risk ofH. influenza, pneumococcal and meningococcal meningitis Crowded environments: students living in halls of residence are a commonly affected demographic

Question 86

What is encephalitis?

Answer 86

Refers to inflammation of the cerebral cortex

Question 87

What are the main clinical symptoms of encephalitis?

Answer 87

Lethargy + fatigue Decreased level of consciousness Fever Focal neurology Occasionally there may be fits

Question 88

What are the viral causes of encephalitis?

Answer 88

Herpes simplex virus Varicella zoster virus Parvoviruses HIV Mumps virus Measles virus

Question 89

Where are the signs of encephalitis?

Answer 89

Pyrexia (fever) Reduced GCS Aphasia Hemiparesis Cerebellar signs

Question 90

What are some behavioural changes that occur in encephalitis?

Answer 90

Memory disturbance Psychotic behaviour Withdrawal or change in personality

Question 91

What are some investigations for encephalitis?

Answer 91

Throat swab HIV serology MRI of head will show evidence of inflammation will be normal in 1/3 of cases Lumbar puncture and CSF investigation including a PCR for HSV

Question 93

What is the treatment for encephalitis?

Answer 93

Aciclovir = should be given in all cases where it is suspected Ganciclovirmay = be preferred in other herpesvirus infections, such as HHV-6

Question 94

What CSF cell count results would signify a bacterial cause of infection?

Answer 94

a neutrophil predominant leucocytosis a raised protein (from dying bacteria) reduced CSF glucose to serum glucose ratio (metabolically active bacteria, use up glucose as a source of energy)

Question 95

What are the two tests to look for meningeal infection?

Answer 95

Kernigs Test Brudzinski’s Test

Question 96

What is Kernigs Test?

Answer 96

Involves patient lying on their back and flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed. This causes the meninges to stretch A positive test will be where there is spinal pain or resistance to the movement

Question 97

What is Brudzinski’s Test?

Answer 97

Involves lying a patient on their back and gently using your hands to lift their head and neck off the bed and flexing their chin to their chest A positive test is when a patient involuntarily flexes their hips and knees

Question 98

What is another classic sign of meningococcus meningitis?

Answer 98

non-blanching rash that everybody worries about as it indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages. Other causes of bacterial meningitis do not cause this rash

Question 99

What is the empirical antibiotic therapy used against bacterial causes of CNS infection in hospital?

Answer 99

IV cefotaxime or ceftriaxone

Question 100

What medication should be prescribed alongside antibiotics for meningitis treatment?

Answer 100

IV dexamethasone (corticosteroids) - reduce risk of long term neurological complications from some causes of meningitis

Question 101

what medication should be given in primary care for meningitis?

Answer 101

IM benzylpenicillin then admit the patient ASAP!!

Question 102

What is the management for meningitis?

Answer 102

1. Assess GCS 2. blood culture 3. broad spectrum antibiotics = Ceftriaxone or cefotaxime 4. Steroids = IV dexamethasone 5. lumbar puncture = definitive diagnosis

Question 103

What are the contraindications for a lumbar puncture?

Answer 103

Abnormal clotting (platelets/coagulation) Petechial rash Raised intracranial pressure GCS <9

Question 104

Where is a lumbar puncture usually taken from?

Answer 104

Between L3/L4 Since spinal cord ends L1/2

Question 105

When would you do a CT head before a lumbar puncture in meningitis?

Answer 105

Aged > 60 Immunocompromised History of CNS disease Seizures <1 week GCS <14 Focal neurological signs Papilloedema Atypical history

Question 106

What are close contacts of patients with meningitis offered?

Answer 106

Notified Antibiotic prophylaxis = single dose of ciprofloxacin

Question 107

What is the CSF count and culture characteristic for a viral meningitis infection?

Answer 107

Predominant lymphocyte response Only moderately raised protein CSF glucose more than 50% of the level of serum glucose

Question 108

What antimicrobial agent is used to treat a viral meningitis or encephalitis infection?

Answer 108

IV aciclovir

Question 109

How does an infection get into the brain?

Answer 109

1. extra-cranial infection e.g. nasopharynx, ear, sinuses 2. neurosurgical complications e.g. post op, infected shunts, trauma 3. via blood stream e.g. bacteraemic

Question 110

What is the pathophysiology of meningitis?

Answer 110

1. bacteria in blood 2. bacteria enter CSF and can be isolated from immune cells due to BBB 3. replication 4. blood vessels become leaky 6. so WBCs can enter the CSF, meninges and brain 7. meningeal inflammation +/- brain swelling

Question 111

What types of meningitis are acute and which are more commonly chronic?

Answer 111

Bacterial and viral meningitis are usually acute Fungal is more chronic

Question 112

What will a lumbar puncture show for bacterial meningitis?

Answer 112

high pressure cloudy high WBCs neutrophils low glucose high protein

Question 113

What will a lumbar puncture show for viral meningitis?

Answer 113

normal or elevated pressure clear increased lymphocytes high glucose low protein

Question 114

what will a lumbar puncture results show for fungal/TB meningitis?

Answer 114

elevated pressure cloudy/fibrin web Increased lymphocytes low glucose high protein

Question 115

What is spinal cord compression SCC?

Answer 115

Results form processes that compress or displace arterial, venous and cerebrospinal fluid spaces as well as the cord itself.

Question 116

What is the main cause of SCC?

Answer 116

Metastatic cancer lesions

Question 117

Where are the metastasis most commonly from the cause SCC?

Answer 117

Breast Lung Prostate Thyroid Kidney Myeloma Lymphoma

Question 118

What area of the spine is most commonly affected by metastasis in SCC?

Answer 118

Thoracic 60% Lumbar 30% Cervical 10%

Question 119

What are some other causes of SCC?

Answer 119

Disc herniation Disc prolapse Primary spinal cord tumour Infection Haematoma

Question 120

What are the symptoms of SCC?

Answer 120

Back pain Progressive weakness of legs with UMN signs Sensory loss 1-2 cord segments below level of lesion UMN signs below level of lesion LWN signs at the level of the lesion

Question 121

What is a late sign of SCC?

Answer 121

Bladder and anal sphincter involvement: hesitancy, frequency and painless retention

Question 122

What are the sensory ascending spinal tracts?

Answer 122

1. dorsal column medial lemniscus system - fine touch, vibration and proprioception 2. spinocerebellar tracts - posterior = proprioceptive from lower limb - cuneo = upper limbs - anterior = lower limb - rostral = upper ipsilateral 3. anterolateral systme - anterior spinothalamic tract = crude touch, pressure - lateral spinothalamic tract = pain + temperature

Question 123

What does injury to DCMl pathway result in?

Answer 123

loss of proprioception and fine touch - ipsilateral loss (if lesion in spinal cord) - decussation in medulla oblongata Gracillis = ground lower limbs Cunneatus = upper limbs

Question 124

What does injury to anterolateral system result in?

Answer 124

Impairment of: pain and temperature (lateral) Crude touch and pressure (anterior) - contralateral loss (decussate in spinal cord)

Question 125

What does injury to spinocerebellar tracts cause?

Answer 125

ipsilateral loss of muscle co-ordination (unconscious) - likely to be injury alongside descending motor tracts

Question 126

What are the motor descending spinal tracts?

Answer 126

Pyramidal tracts - originate in cerebral cortex, carrying motor fibres to the spinal cord and brain stem - voluntary control of musculature Extrapyramidal tracts - originate in brain stem, carrying motor fibres to the spinal cord - responsible for the involuntary and automatic control of all musculature No synapses = so all upper motor neurones

Question 127

What is the function of descending motor tracts?

Answer 127

Corticospinal tracts – supplies the musculature of the body. Corticobulbar tracts – supplies the musculature of the head and neck.

Question 128

What are the two types of spinal cord injury?

Answer 128

1. complete spinal cord injury - total loss of motor and sensory function below level of injury 2. Incomplete spinal cord injury - partial loss of motor and sensory function below level of injury

Question 129

What would the features of a complete spinal cord compression be?

Answer 129

Loss of all motor and sensory function below the level

Question 130

What are the types of incomplete spinal cord injury?

Answer 130

- anterior cord syndrome - central cord syndrome - Brown-Sequard syndrome - Mixed syndromes

Question 131

What would be the features of an anterior spinal cord compression?

Answer 131

Disruption of the anterior spinal cord: - Loss of motor function below the level - Loss of pain and temperature sensation - Preservation of fine touch and proprioception

Question 132

What would be the features of a posterior spinal cord compression?

Answer 132

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)

Question 133

What are the features of brown-sequard syndrome?

Answer 133

Hemi section of spinal cord: - Ipsilateral paralysis - Ipsilateral loss of vibration and proprioception - Contralateral loss of pain and temperature

Question 134

What is the primary investigation if suspecting a Spinal cord compression?

Answer 134

MRI of spinal cord Biopsy/surgical exploration

Question 135

What is the treatment for SCC?

Answer 135

Surgical decompression - Laminectomy: removal of the lamina/spongy tissue between the discs to relieve pressure - Microdiscectomy: removal of the herniated tissue from the disc

Question 136

What is bilateral sciatica a red flag for?

Answer 136

Cauda equina syndrome

Question 137

What is cauda equina syndrome?

Answer 137

It is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed

Question 138

What are the causes of Cauda Equina?

Answer 138

Lumbar disc herniation: the most common cause of CES Trauma Spinal tumour Lumbar spinal stenosis: narrowing of the spinal cord may result in CES. This can be congenital or acquired e.g. spinal osteoarthritis (spondylosis), rheumatoid arthritis, and a slipped vertebra (spondylolisthesis) Epidural abscess or haematoma

Question 139

What are the key presentations of Cauda Equina Syndrome?

Answer 139

Saddle anaesthesia = (loss of sensation in the perineum) can you feel wiping after poo Loss of sensation in bladder and rectum (not knowing when they’re full) Urinary retention or incontinence Faecal incontinence

Question 140

What are the investigations for CES?

Answer 140

Examinations: PR exam, knee and ankle reflexes MRI spine is gold standard Bladder ultrasound: to determine whether there us urinary retention

Question 141

What is the treatment for CES?

Answer 141

Emergency decompressive laminectomy: surgery should be performed within24-48 hoursof symptom onset. The incidence of thromboemboli in patients with CES is remarkably high, therefore patients require adequate thromboprophylaxis

Question 142

What is the main differential of CES?

Answer 142

Conus medullaris

Question 143

What is Broca’s and Wernicke’s area?

Answer 143

Broca - motor control of speech Wernicke - understanding of speech

Question 144

What are mononeuropathies?

Answer 144

Lesions of individual peripheral or cranial nerves - causes are usually local e.g. trauma or entrapment (tumour)

Question 145

What are the types of mononeuropathies?

Answer 145

most affect the upper limb - median nerve e.g. carpal tunnel syndrome - ulnar = compression at elbow - axillary neuropathy = shoulder dislocation/relocation - radial neuropathy = spiral fractures of the radius lower limb - L5 radiculopathy = foot drop

Question 146

Presentation: mononeuropathy

Answer 146

pain/paraesthesia/numbness in the distribution of the named nerve examination - majority of patients have no signs

Question 147

Investigations: mononeuropathy

Answer 147

EMG nerve conduction studies

Question 148

What is Myasthenia Gravis?

Answer 148

A chronic autoimmune disorder of the postsynaptic membrane at the neuromuscular junction of skeletal muscle

Question 149

What are the risk factors for developing Myasthenia Gravis?

Answer 149

Female 2x as common Autoimmune: linked to rheumatoid and SLE -Thymoma or thymic hyperplasia: 10-15% have a thymoma and 70% have thymic hyperplasia

Question 150

What is the main cause of disease in Myasthenia Gravis?

Answer 150

Acetylcholine receptor antibodies present (85%) These bind to the receptors on the post-synaptic membrane and prevent ACh from binding and causing muscle contraction The problem is worsened during exercise as more of the receptors become blocked up. So the more the muscles are used the weaker they get. Gets better with rest These antibodies also activate the complement system which damages cells further making the problem worse

Question 151

What are the two other antibodies that can cause MG and how do they do it?

Answer 151

1. Muscle specific kinase (MuSK) 2. low-density lipoprotein receptor-related protein 4 (LRP4). They are both proteins that are important for making the acetylcholine receptor. These antibodies lead to inadequate acetylcholine receptors causing MG

Question 152

What are the signs of MG?

Answer 152

Proximal muscle weakness: often affecting the face and neck Ptosis (drooping eyelid) Complex ophthalmoplegia: cannot be isolated to one cranial nerve Head drop: a rare sign due to weakness of cervical extensor muscles Myasthenic snarl: a ‘snarling’ expression when attempting to smile

Question 153

What are the symptoms of MG?

Answer 153

Muscle weakness that gets worse throughout the day Diplopia: double vision Slurred speech Fatigue in jaw while chewing

Question 154

What are the investigations for MG?

Answer 154

Look for presence of autoantibodies: - Acetylcholine receptor (ACh-R) antibodies (85% of patients) - Muscle-specific kinase (MuSK) antibodies (10% of patients) - LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%) CT/MRI of thymus gland to look for thymoma

Question 155

What is another test that can be performed if unsure about MG?

Answer 155

Edrophonium test: patients given IV Edrophonium chloride (neostigmine). Will prevent breakdown of ACh by cholinesterase enzymes and improve symptoms temporarily

Question 156

What are the treatments for MG?

Answer 156

First-line: Reversible acetylcholinesterase inhibitors (neostigmine/pyridostigmine) Second-line: Immunosuppressants: prednisolone/azathioprine Consider monoclonal antibodies (rituximab) Thymectomy can also improve symptoms.

Question 157

What is the main complication of MG?

Answer 157

Myasthenic crisis often triggered by another illness such as respiratory tract infection . Causes an acute worsening of symptoms and can lead to respiratory failure

Question 158

What is the treatment for a Myasthenic crisis?

Answer 158

Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation. Medical treatment of myasthenic crisis is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.

Question 159

What is the main differential for MG?

Answer 159

Lambert-Eaton myasthenic syndrome

Question 160

What drugs should be avoided in MG?

Answer 160

Antibiotics; - aminoglycosides (e.g. gentamicin) - fluoroquinilones (e.g. ciprofloxacin) - Macrolides (e.g. azithromycin) - tetracyclines (e.g. doxycycline) - quinolones Neuromuscular blocking agents Magnesium sulfate penicillamine Cardiac drugs - BB neurological - lithium + phenytoin

Question 161

What is Guillain-Barré syndrome?

Answer 161

An acute paralytic polyneuropathy. It is an autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system

Question 162

What are the risk factors for developing Guillain-Barré syndrome?

Answer 162

Male Age 15-35 and 50-75 Malignancies Vaccines (flu) Infections

Question 163

What are the most common infections that trigger Guillain-Barré syndrome?

Answer 163

Campylobacter jejuni (most common) Cytomegalovirus EBV

Question 164

What causes Guillain-Barré syndrome?

Answer 164

A pathogenic antigen resembles myelin gangliosides in the peripheral nervous system. The immune system targets the antigen and attacks the myelin sheath of sensory and motor neurones It occurs in patches along the length of the axon so is called segmental demyelination

Question 165

What antibodies are found in 25% of people with Guillain-Barré syndrome?

Answer 165

Anti-ganglioside antibodies (anti-GMI)

Question 166

Describe the disease course of Guillain-Barré syndrome?

Answer 166

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Question 167

What are the signs and symptoms of Guillain-Barré syndrome?

Answer 167

infection 2 weeks prior Symmetrical ascending weakness (starting at feet and moving up the body) Reduced reflexes Loss of sensation and pain Cranial nerve involvement such as facial nerve weakness Autonomic features (sweating, raised pulse) Struggling to breathe

Question 168

What is used to diagnose Guillain-Barré syndrome?

Answer 168

A clinical diagnosis that is evidenced by progressive weakness and hyporeflexia in the weaker limbs. The Brighton criteria is used for diagnosis.

Question 169

What are the differentials of Guillain-Barré syndrome?

Answer 169

Myasthenia gravis Transverse myelitis Polymyositis

Question 170

What is the treatment for Guillain-Barré syndrome?

Answer 170

IV immunoglobulins IG or Plasma exchange (alternative to IV IG) Venous thromboembolism prophylaxis (PE is the leading cause of death)

Question 171

What is the prognosis for Guillain-Barré syndrome?

Answer 171

80% will fully recover 15% will be left with some neurological disability 5% will die

Question 172

What is Bell’s palsy?

Answer 172

Neurological condition that presents with rapid onset of unilateral facial paralysis

Question 173

Why is the top half of the face sometimes spared in Bell’s palsy?

Answer 173

The lower half of the faces only has contralateral innervation Top half has bilateral. forehead sparing

Question 174

How can you tell between an UMN and LMN Bell’s palsy?

Answer 174

UMN injured: means the contralateral side is weak but the forehead is not LMN - weakness of all the muscles on the ipsilateral side of the face

Question 175

Management: Bell's palsy

Answer 175

Prednisolone within 72 hrs of onset - can be combined with antiviral e.g. aciclovir Lubricating eye drops

Question 176

Define epilepsy?

Answer 176

A neurological disorder in which a person experiences recurring seizures. (needs to have at least 2 seizures to be diagnosed as epilepsy)

Question 177

What are the 2 different types of seizure?

Answer 177

Generalised:when both hemispheres are affected always a loss of consciousness Focal : when the affected area is limited to one half of the brain or sometimes even smaller like a single lobe can progress to bilateral

Question 178

What are the different subtypes of generalised seizure?

Answer 178

Tonic Atonic Clonic Tonic-clonic Myoclonic Absence

Question 179

What are the two types of focal seizure?

Answer 179

Simple (without impaired awareness) Complex (with impaired awareness)

Question 180

What are the general clinical manifestations of seizures for each stage?

Answer 180

Prodromal phase: Confusion, irritability or mood disturbances Early-ictal phase: Aura: warning felt before a seizure. These can include sensory, cognitive, emotional or behaviour changes. Ictal phase: Will vary depending on seizure type Post-ictal phase: Confused, drowsy and irritable during recovery

Question 181

What is a tonic, clonic and tonic-clonic seizure?

Answer 181

Tonic seizure: the muscles become stiff and flexed which will cause the patients to fall backwards Clonic seizures: violent muscle contractions (convulsions) Tonic-clonic: there is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking episodes). Typically the tonic phase comes before the clonic phase. (tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolongedpost-ictal periodwhere the person is confused, drowsy and feels irritable or depressed.

Question 182

What is the medication for generalised tonic-clonic seizures?

Answer 182

males: Sodium valproate female: Lamotrigine or levetiracetam

Question 183

What is an Atonic seizure?

Answer 183

Known as drop attacks. This is where the muscles suddenly relax and become floppy which can cause the patient to fall usually forward. They don’t usually last longer than 3 minuets. They typically begin in childhood. They may be indicative ofLennox-Gastaut syndrome.

Question 184

What is the management for a Tonic and Atonic seizure?

Answer 184

males: sodium valproate females: Lamotrigine

Question 185

What is a myoclonic seizure?

Answer 185

They present as sudden brief muscle contractions like a sudden jump. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy.

Question 186

What is the management for a myoclonic seizure?

Answer 186

males: sodium valproate females: levetiracetam

Question 187

What is an absence seizure?

Answer 187

Impaired awareness or responsiveness. Patient becomes blank and stares into space before returning to normal. Motor abnormalities are either absent or very minor e.g. eyelid flutters or repetitive lip smacking Common in children. Most patients (> 90%) stop having absence seizures as they get older.

Question 188

What is the management for an absence seizure?

Answer 188

First line: ethosuximide 2nd line: males = sodium valproate female = lamotrigine or levetiracetam

Question 189

What is a simple focal seizure (focal aware seizure)?

Answer 189

No loss of consciousness The patient is aware and awake Will have uncontrollable muscle jerking

Question 190

What is a complex focal seizure (focal impaired awareness seizure)?

Answer 190

There is loss of consciousness Patient is unaware

Question 191

What is the most common region of the brain affected in a focal seizure?

Answer 191

Temporal lobe

Question 192

What are the features of a temporal lobe seizure?

Answer 192

They affect hearing, speech, memory and emotions: - Hallucinations - Memory flashbacks - Déjà vu - Doing strange things on autopilot Can also include audio symptoms such as buzzing, ringing and vertigo

Question 193

What are the features of a frontal lobe seizure?

Answer 193

Motor symptoms: pelvic thrusting, bicycling and tonic posturing Bizarre behaviour Vocalisations Sexual automatisms

Question 194

What are the features of a parietal focal seizure?

Answer 194

Paraesthesia Visual hallucinations Visual illusions More subjective and difficult to diagnose than other areas

Question 195

What are the features of a Occipital focal seizure?

Answer 195

Visual hallucinations Transient blindness Rapid and forced blinking Movement of head or eyes to the opposite side

Question 196

What can happen if a focal seizure spreads?

Answer 196

Focal to bilateral tonic-clonic: a focal seizure may spread to affect a wider network of neurons involving both hemispheres. Traditionally termed a secondary generalised seizure.

Question 197

What is the medication for focal seizures?

Answer 197

1st line: Lamotrigine or levetiracetam 2nd line:  carbamazepine, oxcarbazepine or zonisamide

Question 198

What is the mechanism of Carbamazepine?

Answer 198

sodium channel blocker; prevents repetitive and sustained firing of action potentials

Question 199

What are the side effects of Carbamazepine?

Answer 199

- Reduce effect of Oral Contraceptive Pill (by CYP450 induction > faster metabolism) - leucopenia, thrombocytopenia, hydronatraemia + liver dysfunction

Question 200

Can you take Carbamazepine when pregnant?

Answer 200

DONT STOP - risk of harm from seizures is greater than risk of foetal malformations (very low risk) - immediately start taking folic acid

Question 201

What is the mechanism of sodium valproate?

Answer 201

increases the activity of GABA, which has a relaxing effect on the brain, teratogenic

Question 202

What are the investigations for seizures?

Answer 202

- urinalysis or urine cultures, blood cultures (exclude sepsis, encephalitis, or meningitis) - CT scan - Blood: blood glucose (rule out hypoglycaemia) - ECG (to exclude a heart problem) - EEG (only useful while having a seizure to track electrical patterns in brain, won't visualise much afterwards)

Question 203

What are the other treatments for epilepsy?

Answer 203

Vagus nerve stimulation = send electrical stimulations to left vagus nerve to calm down irregular electrical brain activity Surgery: Temporal lobe resection MC Corpus callosotomy = cut callosum to stop epilepsy spreading to other side of brain Lesionectomy Hemispherectomy or hemispherotomy Ketogenic diet - high fat, low carbs, controlled protein protein diet - brain use ketone bodies instead of glucose

Question 204

Which anti-epileptic medication is teratogenic?

Answer 204

sodium valproate - drugs which cause structural/functional malformations in a developing fetus

Question 205

What is required for a diagnosis of epilepsy?

Answer 205

Must have had 2 or more seizures more than 24 hours apart MRI/CT: examine the hippocampus look for underlying cause EEG: 3H2 wave absence Bloods: rule out metabolic/infection

Question 206

What are the different causes of seizures? (pneumonic)

Answer 206

VITAMIN DE - Vascular - Infection - Trauma - Autoimmune- SLE - Metabolic - Idiopathic - Neoplasms - Dementia and drugs (cocaine) - Eclampsia

Question 207

What is the medical emergency associated with epilepsy?

Answer 207

Status Epilepticus

Question 208

How do you treat status Status Epilepticus?

Answer 208

ABCDE 1. Give IV benzodiazepam e.g. lorazepam 4mg over 5 mins and repeat 5 minuets after if it doesn’t work 2. if no stopping after 15 mins give IV sodium valproate or levetiracetam (If seizure persist then give IV phenobarbital or phenytoin) 3. Anesthetist = anaesthetise to stop seizures

Question 209

What are the driving rules for a seizure?

Answer 209

Adults who present with an isolated seizure: Should stop driving for6 months, providing no cause is found on brain imaging and there is no epileptiform activity on EEG If the above conditions are not met or the patient has known epilepsy: Patients must be seizure-free for12 monthsbefore they may qualify for a driving license If seizure-free for 5 years, a ‘til 70 licence is usually reinstated

Question 210

What are febrile convulsions?

Answer 210

A type of seizure that occurs in children with a high fever - children aged 6 months to 5 yrs

Question 211

What are the 2 types of febrile convulsions?

Answer 211

1. simple febrile convulsions = generalised, tonic clonic seizures that last 15 mins and occur only once 2. Complex febrile convulsions = consist of partial or focal seizures, last more than 15 mins or occurs multiple times during the same febrile illness

Question 212

How do you diagnose febrile convulsions?

Answer 212

Child around 18months presenting with a seizure during a high fever rule out other diagnoses e.g. epilepsy, meningitis, intracranial space occupying lesion, trauma

Question 213

Management: febrile convulsion

Answer 213

1. manage source of infection and control fever with analgesia 2. if complex give parents advice on how to deal with seizures

Question 214

What is the cause of an extra-dural haemorrhage?

Answer 214

Traumatic – typically caused by bleeding from the meningeal arteries as a result of skull fracture that tears the dura Middle meningeal runs close to the pterion

Question 215

What is the clinical presentation of extra-dural haemorrhage?

Answer 215

Patient may have extensive traumatic injuries In other cases, patient sustains a head injury but appears to be OK for a while (‘lucid period’) Bleeding into the extradural space > rapid rise in intracranial pressure (ICP) > brain is compressed Headache, drowsiness, rapid neurological deterioration Death if not treated rapidly (neurosurgical emergency

Question 216

What is the imaging look like for an Extra-dural haemorrhage?

Answer 216

Acute (fresh) bleed appears hyperdense on CT (bright white) Convex; does not conform to surface of the brain as bleeding is limited by dural attachments to the skull (lemon shape) Compression of the brain – midline shift (falx cerebri, lateral ventricles) Skull fracture may be seen

Question 217

What is the cause of a sub-dural haemorrhage?

Answer 217

Usually caused by trauma – typically a fall leading to bleeding from dural bridging veins Low pressure bleeding Gradual rise in ICP (over several weeks or months) Most likely in patients with brain atrophy (elderly, dementia, history of excess alcohol intake) – bridging veins

Question 218

What is the clincal presentation for sub dural haemorrhage?

Answer 218

Typical picture is of gradual cognitive deterioration May be a history of a fall, maybe not Patient may have old bruising on their head (or elsewhere) suggesting frequent / recent falls

Question 219

What is the appearance of imaging for sub dural?

Answer 219

(banana shape) Chronic (old) bleed appears hypodense on CT (dark) Concave; conforms to surface of the brain as bleeding is not limited by dural attachments Compression of the brain – signs include midline shift (falx cerebri, lateral ventricles)

Question 220

What are the investigations for a SDH?

Answer 220

Immediate CT head

Question 221

WHat is the cause of a sub-arachnoid haemorrhage?

Answer 221

Usually spontaneous from rupture of an aneurysm on a cerebral artery Can be traumatic, but this is less common

Question 222

What is the management for a SDH?

Answer 222

Drainage: - small SDH are drained via a burr hole washout a small tube called - large SDH requires a craniotomy which is when part of the skull bone is removed

Question 223

What are the complications a the raised intercranial pressure in a SDH?

Answer 223

Supratentorial herniation: cerebrum is pushed against the skull or the tentorium, can compress the arteries that nourish the brain leading to an ischaemic stroke Infratentorial herniation: cerebellum is pushed against the brainstem, can compress the vital area in the brainstem that control consciousness, respiration, and heart rate

Question 224

What is the clinical presentation for subarachnoid?

Answer 224

Typically present with sudden onset severe ‘worst-ever headache’ (‘thunderclap’) Patient may: be conscious have reduced GCS be unconscious (poorer prognosis) - vomiting - blurred vision - cranial nerve deficits Can be rapidly fatal

Question 225

What is the appearacne of a sub-arachnoid bleed?

Answer 225

Acute (fresh) bleed is hyperdense on CT (bright white) Blood seen in fissures and cisterns +/- ventricles (not a large mass like SD and ED) Blood in the SA space: irritates the meninges irritates cerebral vessels and causes vasospasm > hypoxic injury may track back into the ventricular system > hydrocephalus (looks like spider)

Question 226

Investigations: SAH

Answer 226

1. CT head non-contrast 2. If postive - CT angiogram / MRI angiogram (can do weeks down the line to check for aneurysms) 3. If CT negative > Lumbar puncture 12hrs after onset (xanthochromia)

Question 227

Management: SAH

Answer 227

Initial 1. Surgery = coiling vs clipping 2. nimodipine to reduce vasospasm 3. systolic BP <160 4. Neuro-obs, HDU care 5. manage fluid balance

Question 228

Complications of SAH

Answer 228

- vasospasm - rebreeding - due to increase intra cranial pressure

Question 229

What is the cause of an intra-cerebral haemorrhage?

Answer 229

- Spontaneous due to aneurysm or vessel rupture - Small perforating vessels prone to rupture, especially if hypertensive - Clinical presentation determined by the size of the bleed and brain region affected E.g. Coma Weakness (facial, limbs) Seizure

Question 230

What is the appearance of a intra-cerebral bleed on imaging?

Answer 230

Acute (fresh) bleed is hyperdense on CT (bright white) Blood seen in the substance of the brain Mass effect seen if large (e.g. midline shift)

Question 231

What is an epidural haemorrhage?

Answer 231

Bleeding above the dura matter (same as extra dural haemorrhage)

Question 232

Who is an EDH most common in?

Answer 232

They usually occur in young adults

Question 233

What are the symptoms of a EDH?

Answer 233

Reduced GCS: loss of consciousness after the trauma due to concussion - There might be a lucid interval after initial trauma if there is a slower bleed. This is followed by rapid decline. Headaches Vomiting Confusion Seizures Pupil dilation if bleeding continues

Question 234

What are the differentials for a EDH?

Answer 234

Epilepsy Carotid dissection Carbon monoxide poisoning Subdural haematoma Subarachnoid haemorrhage

Question 235

What is the management for a EDH

Answer 235

Clot evacuation Craniotomy: part of the skull bone is removed in order to remove accumulated blood below. Followed by ligation of the vessel.

Question 236

Define: Giant Cell Arteritis

Answer 236

AKA temporal arteritis Type of systemic vasculitis affecting the medium and large arteries

Question 237

Presentation: Giant cell arteritis

Answer 237

Unilateral headache - scalp tenderness - jaw claudication - blurred or double vision - loss of visions if untreated Associated features of: - Symptoms of polymyalgia rheumatica (e.g., shoulder and pelvic girdle pain and stiffness) - Systemic symptoms (e.g., weight loss, fatigue and low-grade fever) - Muscle tenderness - Carpel tunnel syndrome - Peripheral oedema

Question 238

How to make a diagnosis of giant cell arteritis?

Answer 238

- Clinical presentation - Raised inflammatory markers, particularly ESR (usually more than 50 mm/hour) - Temporal artery biopsy (showing multinucleated giant cells) - Duplex ultrasound (showing the hypoechoic “halo” sign and stenosis of the temporal artery)

Question 239

Immediate Management: GCA

Answer 239

40-60mg prednisolone daily with no visual symptoms or jaw claudication 500mg-1000mg IV methylprednisolone daily with visual symptoms or jaw claudication

Question 240

Long term management: GCA

Answer 240

Once the diagnosis is confirmed and the condition is controlled, the steroid dose is slowly weaned over 1-2 years. Other medications include: - Aspirin 75mg daily decreases vision loss and strokes - Proton pump inhibitor (e.g., omeprazole) for gastroprotection while on steroids - Bisphosphonates and calcium and vitamin D for bone protection while on steroids

Question 241

What are red flags for headaches in regard to the features of the headache?

Answer 241

Sudden-onset reaching worse severity within 5 minuets (subarachnoid haemorrhage) New-onset over 50 (GCA/ space occupying lesion) Progressive/persistent headache or one that has changed dramatically (space-occupying lesion/ subdural haematoma)

Question 242

What are the causes of spinal fractures?

Answer 242

trauma - road traffic collisions - falls from height - sports injuries - violence or assault disease and degenerative changes - osteoporosis - cancer - infections - arthritis

Question 243

What are red flag precipitating factors for headaches?

Answer 243

Recent head trauma within 3 months (subdural haematoma) Headache worse lying down (raised ICP) Headache worse on standing (CSF leak) Household contacts with similar symptoms (CO poisoning)

Question 244

What are red flag associated symptoms of headaches?

Answer 244

Fever, photophobia or neck stiffness (meningitis or encephalitis) New neurological defect (raised ICP/stroke) Visual disturbance (GCA/ acute closure glaucoma) Vomiting (raised ICP, brain abscess and CO poisoning)

Question 245

What are the risk factors for spinal fracture?

Answer 245

Osteoporosis Cancer Trauma, such as a car accident or fall Age Tobacco use Alcohol abuse Chronic steroid use Poor nutrition

Question 246

What is an important investigation to carry out for a headache?

Answer 246

fundoscopy = (look at retina at back of eye) look for papilledema which indicates a raised intracranial pressure + idiopathic intracranial hypertension

Question 247

Presentation: spinal fracture

Answer 247

- Pain: in the back, neck or limbs - Paraesthesia: may indicate a spinal cord injury - Weakness/paralysis: may indicate a spinal cord injury - Impaired bowel and bladder function = indicate spinal cord injury

Question 248

What are the features of idiopathic intracranial hypertension?

Answer 248

females - headache, intermittent, generalised - worse lying down - visual obscurations - usually high BMI - papillodema I = normal CT T = modify risk factors = lose weight first acetazolamide/diuretics/topiramate

Question 249

Management: spinal fracture

Answer 249

1. maintain cervical spine immobilisation 2. treat life threatening injuries 3. perform 'log roll' examination of the back to asses the spine 4. neurological examination 5. C-spine radiograph and CT neck 6. analgesia 7. surgical intervention

Question 250

What are the risk factors for migraines?

Answer 250

Family history Female gender 3 times more common Obesity Triggers

Question 251

What are some triggers for migraines?

Answer 251

CH- Chocolate OC- Oral contraceptive OL- alcohOL A- anxiety T- travel E- exercise CHOCOLATE Other triggers can be red wine, bright lights and menstruation

Question 252

What are the different types of migraine?

Answer 252

Migraine without aura Migraine with aura Silent migraine (just the aura without the headache) Hemiplegic migraine

Question 253

What are the headache symptoms of a migraine?

Answer 253

Last between 4-72 hours: - Pounding or throbbing in nature - Usually unilateral (can be bilateral more common in children) - Photophobia - Phonophobia (loud noises) - Aura - Nausea and vomiting

Question 254

What is aura?

Answer 254

Aura is the term used to describe the visual changes associated with migraines symptoms can be: - Sparks in vision - Blurring vision - Line across vision - Loss of different visual fields

Question 255

What is a hemiplegic migraine?

Answer 255

They can mimic a stroke. Need to rule out if patient has symptoms: - typical migraine - Sudden onset - Hemiplegia - Ataxia - Change in consciousness

Question 256

What are the 5 stages of a migraine?

Answer 256

1. Prodromal stage = subtle symptoms such as yawning, fatigue or mood changes 2. Aura 3. headache 4. Resolution the headache can fade away and be relieved by vomiting or sleeping 5. Postdromal stage

Question 257

What is the diagnostic criteria for migraines with aura?

Answer 257

At least TWO headaches filling criteria: One or more typical fully reversible aura symptoms including: - Visual symptoms such as zigzag lines and/or scotoma visual aura is the most common type of aura. - Sensory symptoms such as unilateral pins and needles or numbness. - Speech and/or language symptoms such as dysphasia. At least three of the following: - At least one aura symptom spreads gradually over at least 5 minutes. - Two or more aura symptoms occur in succession. - Each individual aura symptom lasts 5-60 minutes. - At least one aura symptom is unilateral. - At least one aura symptom is positive. - The aura is accompanied, or followed within 60 minutes, by headache. (can be confused with stroke until headache starts)

Question 258

What is the diagnostic criteria for migraines without aura?

Answer 258

FIVE attacks fulfilling this criteria Duration = Headache lasting 4–72 hours in adults or 2–72 hours in adolescents. Headache with at least TWO of the following characteristics: - Unilateral location (more commonly bilateral in children). - Pulsating quality — may be described as ‘throbbing’ or ‘banging' in young people. - Moderate or severe pain intensity. - Aggravation by, or causing avoidance of, routine activities of daily life (for example walking or climbing stairs). Headache with associated symptoms including at least one of: - Nausea and/or vomiting. - Photophobia (sensitivity to light) and phonophobia (sensitivity to sound).

Question 259

What is the management for migraines?

Answer 259

Analgesia (avoid opioids) Oral triptan ( 500mg sumatriptan) as the headache starts

Question 260

What are triptans?

Answer 260

They 5HT (serotonin) receptor agonists and they cause: - smooth muscle contraction in arteries - Peripheral pain receptors to inhibit activation of pain receptors - Reduce neuronal activity in the central nervous system

Question 261

What are the medications used for migraine prophylaxis?

Answer 261

Propranolol Topiramate (don’t use in pregnancy as it is teratogenic and can cause cleft lip) Amitriptyline

Question 262

What are the non-pharmacological treatments for migraines?

Answer 262

Acupuncture: if both propranolol and topiramate are ineffective or unsuitable Riboflavin (vitamin B2): **may be effective in some people, but avoid in pregnancy

Question 263

What is a tension headache?

Answer 263

The most common form of a primary headache - Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)

Question 264

What are the causes of tension headaches?

Answer 264

Missed meals Stress Overexertion Lack of sleep Depression

Question 265

What are the symptoms of tension headaches?

Answer 265

Bilateral with a pressing/tight sensation of mild-moderate intensity Nausea or vomiting Photophobia Phonophobia

Question 266

What is the main risk factor for a tension headache?

Answer 266

stress

Question 267

What are cluster headaches?

Answer 267

Severe unilateral headaches often periorbital that come in clusters of attacks

Question 268

What is the typical presentation for cluster headaches?

Answer 268

A typical patient with cluster headaches in your exams is a – 50 year-old male smoker. - Attacks can be triggered by things like alcohol, strong smells and exercise.

Question 269

How long can a cluster headache last?

Answer 269

Patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years. Attacks last between 15 minutes and 3 hours.

Question 270

What are the symptoms of a cluster headache?

Answer 270

Severe and intolerable pain unilateral Red swollen watering eye Pupil constriction Eyelid dropping Nasal discharge Facial sweating

Question 271

What is the acute management of cluster headaches?

Answer 271

Triptans (6mg sumatriptan subcut) High flow oxygen 100%

Question 272

What are the prophylaxis for cluster headaches?

Answer 272

Verapamil (CCB) Lithium Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)

Question 273

What are the risk factors for Trigeminal Neuralgia?

Answer 273

females 50-60 increases with age unilateral MS

Question 274

What are the causes of Trigeminal Neuralgia?

Answer 274

Normally due to compression of the trigeminal nerve by a vascular loop often the superior cerebellar artery

Question 275

What are triggers for TGN?

Answer 275

Light touch Washing Shaving Talking Cold weather

Question 276

What is the headache like in Trigeminal Neuralgia?

Answer 276

Electric Shock Pain that lasts for seconds to minutes across the face 90% unilateral 10% bilateral

Question 277

What is the diagnostic criteria for trigeminal neuralgia?

Answer 277

Clinical Dx 3 or more attacks with characteristic unilateral facial pain and Symptoms MRI - exclude secondary causes/other pathology

Question 278

What is the treatment for TGN?

Answer 278

- First line: Carbamazepine - Second line: microvascular decompression or ablative surgery may be considered in refractory patients

Question 279

What is Horner's syndrome?

Answer 279

- lesion of the sympathetic chain supplying the eye. can arise as an isolated injury or as a manifestation of a systemic disease process.

Question 280

Presentation: Horner's syndrome

Answer 280

1. Miosis (constriction of the pupil), 2. Ptosis (drooping of the upper eyelid) 3. Anhidrosis (absence of sweating of the face) on ipsilateral side of face

Question 281

What is the classification of the location of Horner's syndrome?

Answer 281

classified according to the site of the lesion sympathetic chain involved in Horner's consists of 3 consecutive neurones 1. central lesion = Anhidrosis of face, arm and trunk 2. preganglionic lesion = Anhidrosis of face 3. post ganglionic lesion = No anhidrosis

Question 282

What are the causes of Horner syndrome based on the location of the lesion?

Answer 282

Central lesions: sentral S – Stroke S – Multiple Sclerosis S – Swelling (tumours) S – Syringomyelia (cyst in the spinal cord) Pre-ganglionic lesions: Torso T – Tumour (Pancoast tumour) T – Trauma T – Thyroidectomy T – Top rib (a cervical rib growing above the first rib and clavicle) Post-ganglionic lesions: Cervical C – Carotid aneurysm C – Carotid artery dissection C – Cavernous sinus thrombosis C – Cluster headache

Question 283

Investigations: Horner's syndrome

Answer 283

1. eye drops - apraclonidine = reverse pupillary constriction (cause dilation in Horner's) - hydroxyamphetamine = will dilate a constricted Horner's pupil if preganglionic lesions (and not a postganglionic lesion)

Question 284

What are mononeuropathies?

Answer 284

Lesions of individual peripheral or cranial nerves - causes are usually local e.g. trauma or entrapment (tumour)

Question 285

What are the features of a median nerve lesion in the wrist and anterior interosseous?

Answer 285

C6-T1 = nerve of precision grip + LOAF Wrist = carpal tunnel syndrome, weakness of abductor pollicis brevis; sensory loss over the radial 3 1/2 fingers and palm Anterior interosseous nerve lesions = weakness of flexion of the distal phalanx of the thumb and index finger Proximal lesions = may show combined effects

Question 286

What is the presentation of an ulnar nerve palsy/lesion?

Answer 286

C7-T1 Claw hand (4th + 5th fingers 'claw' up) - sensory loss over medial 1 1/2 fingers and ulnar side of the hand - wasting of hypothenar eminence

Question 287

What is the treatment for a ulnar nerve palsy?

Answer 287

Splint for elbow simple analgesia rest + avoid pressure on nerve

Question 288

What is the presentation of a radial nerve palsy?

Answer 288

C5-T1 Wrist drop Muscles involved = BEAST Brachioradialis Extensors Abductor pollicis longus Supinator Triceps

Question 289

What is the treatment for a radial nerve palsy?

Answer 289

Splint and simple analgesia

Question 290

What are the causes of brachial plexus lesion?

Answer 290

- Trauma** - **Radiotherapy** - **Prolonged wearing of heavy rucksack** - **Cervical rib** - **Thoracic outlet compression** - **Neuralgic amyotrophy**

Question 291

What are the signs of a brachial plexus lesion?

Answer 291

Pain/ paraesthesia and weakness in the affected arm in a variable distribution

Question 292

What are the causes of a phrenic nerve lesion?

Answer 292

C3,4,5 keeps the diaphragm alive - **Lung cancer** - **TB** - **Paraneoplastic syndrome** - **Myeloma** - **Thymoma** - **Cervical spondylosis/ trauma** - **Thoracic surgery** - **Infections e.g. HZV, HIV, Lyme disease** - **Muscular dystrophy**

Question 293

What are the signs of a phrenic nerve lesion?

Answer 293

- **Orthopnoea** - **Raised hemi-diaphragm** on chest x-ray

Question 294

What nerve causes a 'foot drop'?

Answer 294

Common peroneal nerve L4-S1

Question 295

What are the effects of a tibeal nerve lesion?

Answer 295

L4-S3 - inability to stand on tiptoes, invert foot and flex the toes - sensory loss over the sole

Question 296

What is mononeuritis mulitplex? And causes?

Answer 296

- A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes - Electromyography helps define the anatomic site of lesion

Question 297

What are the causes of mononeuritis multiplex?

Answer 297

WARDS PLC - Wegner’s granulomatosis - AIDS - Rheumatoid arthritis - Diabetes - Sarcoidosis - Polyarteritis nodosa - Leprosy - Carcinoma

Question 298

What are the signs of cranial nerve 1 lesion?

Answer 298

- Reduced taste and smell - Ammonia taste remains as it stimulates the pain fibres carried in the trigeminal nerve

Question 299

What are the causes of a cranial nerve lesion?

Answer 299

- Trauma - Frontal lobe tumour - Meningitis - fracture - Raised ICP - Diabetes - hypertension

Question 300

What are the signs of cranial nerve 2 lesion?

Answer 300

1. Visual field defects: - Start as small areas of visual loss (scotomas). - Monocular severe sight impairment: lesions of one eye or optic nerve - Bilateral severe sight impairment - Bitemporal hemianopia - Homonymous hemianopia: loss of the same half (left or right) of the visual field of both eyes, on the opposite side to the lesion (eg, a right side lesion causes loss of the left side of the visual field of both eyes). 2. Pupillary abnormalities 3. Optic neuritis - Pain on moving the eye - Loss of central vision - Afferent pupillary defect - Papilloedema 4. Optic atrophy - Pale optic discs - Reduced acuity

Question 301

What is diabetic neuropathy?

Answer 301

a complication of diabetes that results in nerve damage that develops gradually and is caused by long term high blood sugar levels

Question 302

Presentation: diabetic neuropathy

Answer 302

- sensitivity to touch - loss of sense of touch - difficulty with coordination when walking - numbness or pain in your hands or feet - burning sensation in feet, especially at night - muscle weakness or wasting - bloating or fullness - nausea, indigestion, or vomiting - diarrhea or constipation - dizziness when you stand up - excessive or decreased - sweating - bladder problems such as incomplete bladder emptying - vaginal dryness - erectile dysfunction

Question 303

What are the different types of diabetic neuropathy?

Answer 303

1. peripheral neuropathy - affects feet and legs = numbness, tingling, muscle weakness 2. autonomic neuropathy - digestion problems - sexual and bladder problems - cardiovascular problems 3. proximal neuropathy - affects hips, buttocks, thighs 4. focal neuropathy - damage to one specific nerve, causing weakness in that area

Question 304

What causes diabetic neuropathy?

Answer 304

high blood sugar levels sustained over a long period of time - damage to the blood vessels caused by high cholesterol levels - mechanical injury such as injuries caused by carpal tunnel syndrome - lifestyle factors such as smoking or alcohol use

Question 305

Define: radiculopathy

Answer 305

compression of nerve roots as they exit the spinal cord and spinal column, leading to motor and sensory symptoms - usually cervical or lumbar

Question 306

Presentation: radiculopathy

Answer 306

- radiating limb pain - sharp shooting in character - some have sensory loss nerve roots usually affected in upper limb = C6 and C7 Nerve root affected in lower limb = L5 and S1

Question 307

What is the myotome, dermatome and reflex of C6?

Answer 307

Myotome = thumb Dermatome = biceps Reflex = biceps jerk

Question 308

What is the myotome, dermatome and reflex of C7?

Answer 308

myotome = middle finger dermatome = triceps reflex = triceps jerk

Question 309

What is the myotome, dermatome and reflex of L5?

Answer 309

Myotome = dorsal of foot/big toe Dermatome = dorsiflexion (stand on heels) Reflex - N/A

Question 310

What is the myotome, dermatome and reflex of S1?

Answer 310

myotome = ankle, lateral aspect of foot/sole of foot/little toe dermatome = plantar flexion (stand on toes) reflex = ankle jerk

Question 311

What are types of muscular dystrophy?

Answer 311

- Duchennes muscular dystrophy - Beckers muscular dystrophy - Myotonic dystrophy - Facioscapulohumeral muscular dystrophy - Oculopharyngeal muscular dystrophy - Limb-girdle muscular dystrophy - Emery-Dreifuss muscular dystrophy

Question 312

What is Duchenne’s muscular dystrophy?

Answer 312

A genetic condition that cause gradual weakening and wasting of muscles.

Question 313

What is the inheritance pattern of Duchenne’s muscular dystrophy?

Answer 313

X-linked recessive

Question 314

What causes Duchenne’s muscular dystrophy?

Answer 314

caused by a defective gene for dystrophin on the X chromosome. Dystrophin is a protein that helps hold muscles together at the cellular level

Question 315

What will a muscle biopsy in Duchenne’s muscular dystrophy show?

Answer 315

Biopsy of the tissue show changes in the muscle itself but not in the nerves which is used to distinguish it between neuropathies

Question 316

What are the symptoms of Duchenne’s muscular dystrophy?

Answer 316

Symptoms usually present at around 3-5: - Weakness is muscles around pelvis - Walking late - Waddling gait - Enlarged calves

Question 317

What is Gower’s sign?

Answer 317

Children with proximal muscle weakness use a specific technique to stand up from a lying position. This is called Gower’s sign. To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect. This is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.

Question 318

What are the investigation for DMD?

Answer 318

- High creatinine kinase level - Genetic testing looking for dystrophin mutations (can be diagnostic) - Muscle biopsy - Electromyogram: distinguish between neuropathic and myopathic pathology

Question 319

What are the treatments for DMD?

Answer 319

- Occupational therapy - Oral steroids = have been shown to slow the progression of muscle weakness - Creatine supplementation can give an improvement in muscle strength

Question 320

What are the complications of DMD?

Answer 320

- Respiratory failure because of a weak diaphragm - Scoliosis - Dilated cardiomyopathy and arrhythmias: dystrophin protein is also expressed in heart muscle.

Question 321

How is Beckers' muscular dystrophy different to Duchenne's?

Answer 321

Dystrophin gene is less severely affected and maintains some of its function - clinical course is less predictable and symptoms only present around 8-12 yrs

Question 322

What are the features of myotonic dystrophy?

Answer 322

Myotonic dystrophy is a genetic disorder that usually presents in adulthood. Typical features are: - Progressive muscle weakness - *Prolonged muscle contractions - Cataracts - Cardiac arrhythmias

Question 323

Define: neurofibromatosis

Answer 323

Genetic condition that causes nerve tumours to develop throughout the nervous system - benign tumours - 2 types (type 1 is MC)

Question 324

What is neurofibromatosis type 1 gene?

Answer 324

Found on chromosome 17 - codes for a protein called neurofibromin = tumour suppressor protein - autosomal dominant

Question 325

What is the diagnostic criteria for neurofibromatosis type 1?

Answer 325

CRABBING” mnemonic: C – Café-au-lait spots (more than 15mm diameter is significant in adults) R – Relative with NF1 A – Axillary or inguinal freckling BB – Bony dysplasia, such as Bowing of a long bone or sphenoid wing dysplasia I – Iris hamartomas (Lisch nodules), which are yellow-brown spots on the iris N – Neurofibromas = can be on skin as well G – Glioma of the optic pathway

Question 326

Management: neurofibromatosis

Answer 326

Diagnosis is based on the diagnostic criteria. Genetic testing can be helpful. There is no treatment for the underlying disease process. Management involves monitoring, managing symptoms and treating complications.

Question 327

What are the complications of neurofibromatosis type 1?

Answer 327

Migraines Epilepsy Renal artery stenosis, causing hypertension Learning disability Behavioural problems (e.g., ADHD) Scoliosis of the spine Vision loss (secondary to optic nerve gliomas) Malignant peripheral nerve sheath tumours Gastrointestinal stromal tumour (a type of sarcoma) Brain tumours Spinal cord tumours with associated neurology (e.g., paraplegia) Increased risk of cancer (e.g., breast cancer and leukaemia)

Question 328

What is neurofibromatosis type 2?

Answer 328

- chromosome 22 - codes for merlin = tumour suppressor protein important in Schwann cells - lead to schwannomas - autosomal dominant (associated with acoustic neuromas)

Question 329

What are the different types of brain tumour?

Answer 329

Gliomas Meningiomas Pituitary Hemangioblastoma Acoustic neuroma Accounts for less than 2% of all malignant tumours but 20% of childhood cases

Question 330

Presentation: brain tumour

Answer 330

- progressive focal neurological symptoms e.g. change in behaviour, personality - vision change - raised intracranial pressure

Question 331

What are the causes of increased intracranial pressure?

Answer 331

Brain tumours Intracranial haemorrhage Idiopathic intracranial hypertension Abscesses or infection

Question 332

What headache symptoms indicate intracranial hypertension?

Answer 332

Constant headache Nocturnal (occurring at night) Worse on waking Worse on coughing, straining or bending forward Vomiting Papilloedema on fundoscopy

Question 333

What are the features of papilloedema on fundoscopy?

Answer 333

- Blurring of the optic disc margin - Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation) - Loss of venous pulsation - Engorged retinal veins - Haemorrhages around the optic disc - Paton’s lines, which are creases or folds in the retina around the optic disc

Question 334

What are gliomas?

Answer 334

Tumours of the glial cells in the brain or spinal cord and surround neurones - Glial cells include astrocytes, oligodendrocytes and ependymal cells grade 1 to 4 in severity: - Astrocytoma (the most common and aggressive form is glioblastoma) - Oligodendroglioma - Ependymoma (least malignant)

Question 335

What are meningiomas?

Answer 335

Tumours growing from the cells of the meninges - usually benign - take up space causing raised intracranial pressure

Question 336

What cancers most often spread to the brain?

Answer 336

Lung Breast Renal cell carcinoma Melanoma

Question 337

What are acoustic neuromas?

Answer 337

Benign tumours of the Schwann cells that surround the auditory nerve that innervates the inner ear - unilateral

Question 338

Presentation: acoustic neuroma

Answer 338

- CN 8: Unilateral sensorineural hearing loss (often the first symptom) - Unilateral tinnitus - Dizziness or imbalance - Sensation of fullness in the ear -CN 5: absent corneal reflex - CN 7: Facial nerve palsy (if the tumour grows large enough to compress the facial nerve)

Question 339

Investigations: acoustic neuroma

Answer 339

Refer urgently to ENT MRI of cerebellopontine angle Audiometry

Question 340

Management: acoustic neuroma

Answer 340

Refer urgently to ENT Conservative: management with monitoring may be used if there are no symptoms or treatment is inappropriate Surgery: remove the tumour (partial or total removal) Radiotherapy: to reduce the growth

Question 341

Why do brain abscesses form?

Answer 341

when an area of cerebral inflammation becomes necrotic and encapsulated by glial cells and fibroblasts - oedema around abscess may cause raised intracranial pressure

Question 342

What are the causes of brain abscess?

Answer 342

- direct extension of cranial infections - head wounds - hematogenous spread e.g in bacterial endocarditis - unknown causes

Question 343

Presentation: brain abscess

Answer 343

- headache - nausea - vomiting - lethargy - seizures - personality changes - papilledema - fever, chills and leukocytosis may develop before infection is encapsulated

Question 344

Investigations: brain abscess

Answer 344

MRI contrast enhanced CT

Question 345

Management: brain abscess

Answer 345

- antibiotics = ceftriaxone or cefotaxime plus metronidazole - CT guided aspiration or surgical drainage - corticosteroids, anti seizure drugs

Question 346

Define: narcolepsy

Answer 346

rare condition in which the brain loses its normal ability to regulate the sleep-wake cycle 2 types

Question 347

What is the cause of narcolepsy?

Answer 347

Orexin (hypocretin) is a neurotransmitter involved in the regulation of sleep, wakefulness and appetite Loss of orexin secreting neurons in the hypothalamus result in narcolepsy Due to to autoimmunity

Question 348

Presentation: narcolepsy

Answer 348

- Excessive daytime sleepiness (EDS): exclude other causes. - Disrupted nighttime sleep and/or vivid dreams. - Cataplexy: “conscious collapse” caused by muscle atonia, often in response to sudden emotion such as laughter or surprise; rarely seen by the clinician so diagnosis often based on the characteristic description. - Hypnagogic/hypnopompic hallucinations: dream-like hallucinations at the point of emerging from/entering REM sleep. - Sleep paralysis: paralysis while awake and conscious, again at the transition between REM sleep and wakefulness.

Question 349

Investigations: narcolepsy

Answer 349

Polysomnography Multiple sleep latency testing (sleep latency = amount of time it takes to fall asleep) CSF orexin levels

Question 350

Management: narcolepsy

Answer 350

- good sleep hygiene - scheduled naps - CNS stimulants e.g. methylphenidate - antidepressants (for cataplexy) e.g. clomipramine, SSRI - sodium oxybate = potent sedative to improve nocturnal sleep quality

Question 351

Define: bulbar palsy

Answer 351

Refers to a set of signs and symptoms linked to the impaired function of the lower cranial nerves (4-7)

Question 352

What are the causes of bulbar palsy?

Answer 352

- brainstem strokes and tumours - degenerative disease = ALS - autoimmune diseases = Guillain-barre syndrome - genetic diseases

Question 353

Presentation: bulbar palsy

Answer 353

- dysphagia - reduced/absent gag reflex - slurred speech - aspiration of secretions - dysphonia - dysarthria - drooling - difficulty chewing - nasal regurgitation

Question 354

Management: bulbar palsy

Answer 354

- no known treatment - symptom management: medications (drooling), feeding tube, speech and language therapy - condition specific treatments

Question 355

What is ataxia?

Answer 355

problems with balance and coodination - heterogenous group of disorders - many different causes - majority permanent and progressive

Question 356

What are the symptoms of cerebellar dysfunction?

Answer 356

- dizzy - falls, stumble - difficulty focusing/double vision/'oscillopsia' ( - slurred speech - problems with swallowing - tremor - problems with dexterity/ fine motor skills

Question 357

What are the clinical signs of cerebellar dysfunction?

Answer 357

DANISH Dysdiadochokinesis Ataxia Nystagmus Intention tremor Scanning speech, disarthria Hypotonia, hyporeflexia

Question 358

What are the investigations for ataxia?

Answer 358

Blood = FBC, LFTs, ESR, CRP, Gluten, B12, HbA1c, folate HLA typing for DQ2/DQ8 MRI brain = demonstrate cerebellar atrophy CT if MRI contraindicated

Question 359

What is the management of cerebellar ataxia?

Answer 359

no cure so tailored to the individual patient - specialist services

Question 360

Define: cerebral palsy

Answer 360

A non-progressive, permanent neurological condition commonly affecting normal movement and posture

Question 361

What is the cause of cerebral palsy?

Answer 361

Damage to the developing brain which can occur while the baby is in utero, during birth or in the neonatal period - caused by hypoxia, haemorrhage or infection

Question 362

What are the risk factors for cerebral palsy?

Answer 362

antenatal RF - multiple gestation - chorioamnionitis - maternal TORCH infections Perinatal RF - prematurity - low birth weight - birth asphyxia - neonatal sepsis postnatal RF - meningitis - severe hyperbilirubinaemia (jaundice)

Question 363

Presentation: cerebral palsy

Answer 363

delayed motor milestones not sitting by 8 months not walking by 18 months hand preference before 12 months - tone abnormalities (floppies or stiffness) - abnormal movement - feeding problems - persistent toe walking No regression in milestones

Question 364

What are the types of cerebral palsy?

Answer 364

- spastic cerebral palsy = velocity dependent hypertonia (spasticity) and hyperreflexia - Dyskinetic cerebral palsy = involuntary, uncontrolled, recurring movements, fluctuating muscle tone and persistent primitive reflexes - Ataxic cerebral palsy = loss of muscular coordination resulting in ataxia and tremor

Question 365

Investigations: cerebral palsy

Answer 365

clinical diagnosis - MRI scan

Question 366

Management: cerebral palsy

Answer 366

conservative - physiotherapy - occupational therapy - speech and language therapy - dietician input medical - Hyoscine hydrobromide or glycopyrronium bromide: excess drooling - Diazepam: pain - Baclofen: hypertonia - Botulinum toxin type A injections: used if spasticity is severely affecting function or causing significant pain Surgical - hip replacement

Question 367

What are the complications of cerebral palsy?

Answer 367

Problems with feeding and aspiration Drooling Constipation Visual and hearing impairment Epilepsy Learning disability Speech difficulty Osteopenia and osteoporosis (especially if non-mobile) Sleep disturbance

Question 368

define: hypoxic-ischaemic encephalopathy (HIE)

Answer 368

Occurs in neonates as a result of hypoxia during birth hypoxia = lack of oxygen Ischaemia = restriction in blood flow encephalopathy = malfunctioning of the brain HI can lead to permanent damage = cerebral palsy

Question 369

What are the causes of HIE?

Answer 369

- Maternal shock - Intrapartum haemorrhage - Prolapsed cord, causing compression of the cord during birth - Nuchal cord, where the cord is wrapped around the neck of the baby

Question 370

What are the grades of HIE?

Answer 370

Sarnat staging Mild - poor feeding, generally irritability and hyper-alert - resolves within 24 hrs - normal prognosis Moderate - poor feeding, lethargic, hypotonic and seizures - can take weeks to resolve - up to 40% develop cerebral palsy Severe - reduced consciousness, apnoeas, flaccid, and reduced or absent reflexes - up to 50% mortality - up to 90% develop cerebral palsy

Question 371

Management: HIE

Answer 371

Supportive neonatal care - neonatal resuscitation - optimal ventilation - circulatory support - nutrition - acid base balance - treatment of seizures - therapeutic hypothermia

Question 372

What is therapeutic hypothermia?

Answer 372

Babies near or at term considered to have HIE - actively cooling the core temperature of the baby - baby is transferred to a neonatal ICU and actively cooled using cooling blankets and a cooling hat (temp 33-34) - continued for 72 hrs and baby is gradually warmed over 6 hrs

Question 373

What is the purpose of therapeutic hypothermia?

Answer 373

- reduce inflammation and neurone loss after the acute hypoxic injury

Question 374

Define: meniere's disease

Answer 374

Long term inner ear disorder that causes recurrent attacks of vertigo - due to excessive buildup of endolymph in the labyrinth of the inner ear - causes a higher pressure than normal and disrupting sensory signals

Question 375

Presentation: Meniere's disease

Answer 375

- 40-50 years old - unilateral - vertigo episodes (last 20 mins to few hrs) - hearing loss (fluctuates at first and them becomes permanent, affects low frequencies first) - tinnitus (again temporary episodes then become permanent) - sensation of fullness in the ear - unexplained falls without loss of consciousness - imbalance - spontaneous nystagmus during an acute attack (unidirectional)

Question 376

Investigations: meniere's disease

Answer 376

clinical based on signs and symptoms - ENT specialist - audiology assessment

Question 377

Management: meniere's disease

Answer 377

Management involves: - Managing symptoms during an acute attack - Prophylactic medication to reduce the frequency of attacks For acute attacks, short-term options for managing symptoms include: - Prochlorperazine - Antihistamines (e.g., cyclizine, cinnarizine and promethazine) Prophylaxis is with: - Betahistine

Question 378

Define: chronic fatigue syndrome

Answer 378

AKA Myalgic Encephalomyelitis - chronic illness that causes extreme fatigue that cannot be explained by any other medical condition

Question 379

Presentation: Chronic fatigue syndrome

Answer 379

- post exertion malaise = symptoms get worse after exercise or mental effort and don't improve after sleep or rest (can last 24hrs or a few days) - start suddenly or gradually over months/years - persistent and profound fatigue - thinking problems e.g. memory loss, poor concentration - sleeping difficulties - headaches - muscle pain, joint pain - GI changes e.g. constipation, diarrhoea - sensitivities to light or noise - problems with temp regulation

Question 380

What causes chronic fatigue syndrome?

Answer 380

- infection e.g. COVID - environmental toxins - physical trauma e.g. surgery - genetics - physical or emotional stress

Question 381

How to diagnose chronic fatigue syndrome?

Answer 381

- no test - rule out other organic causes - must have symptoms for 6 months +

Question 382

Management: chronic fatigue syndrome

Answer 382

No cure 1. Pacing and rest = breaks down your activity into short bursts interspersed with rest so you don't trigger symptoms 2. Stepwise symptom management = ranking symptoms and starting with most problematic first - go to bed at same time each night - avoid caffeine, alcohol, nicotine - make time to rest - keep dairy to notice your triggers

Question 383

Define: normal pressure hydrocephalus

Answer 383

Abnormal buildup of CSF within the brain and spinal cord - it is either over production of CSF or a problem with draining or absorbing CSF

Question 384

What are the causes of hydrocephalus?

Answer 384

- Aqueductal stenosis = MC leading to insufficient drainage of CSF (in cerebral aqueduct between 3rd and 4th ventricle) - arachnoid cysts - Arnold-chiari malformation = cerebellum herniates downwards through the foramen magnum clocking outflow of CSF - chromosomal abnormalities and congenital malformations

Question 385

Presentation: hydrocephalus

Answer 385

cranial bones in babies are not fused so babies will have an enlarged and rapidly increasing head circumference - bulging anterior fontanelle - poor feeding and vomiting - poor tone - sleepiness

Question 386

Management: hydrocephalus

Answer 386

Ventriculoperitoneal shunt - drains CSF from the ventricles into another body cavity (often peritoneal cavity)

Question 387

What are the complications of a ventriculoperitoneal shunt?

Answer 387

- Infection - Blockage - Excessive drainage - Intraventricular haemorrhage during shunt related surgery - Outgrowing them (they typically need replacing around every 2 years as the child grows)

Question 388

Define: herpes zoster virus

Answer 388

aka Shingles Acute unilateral, self limiting inflammatory disease of cerebral ganglia, posterior nerve roots and peripheral nerves in a segmented distribution, caused by varicella zoster virus (chickenpox)

Question 389

Presentation: herpes zoster

Answer 389

- rash in single dermatome distribution - pruritus - dyesthesia

Question 390

How is herpes zoster caused?

Answer 390

Caused by reactivation of the varicella zoster virus within the dorsal root or cranial nerve ganglia 1. chickenpox virus migrates from skin lesions to spinal and cranial sensory ganglia where it becomes dormant 2. reactivation occurs when cell mediated immunity wanes 3. once reactivated the virus spreads through the affected sensory nerve, causing neuronal damage and reaches the dermatome in the skin where a vesicular rash develops

Question 391

Management: shingles

Answer 391

antiviral drug commenced within 72hrs of rash onset 1. treat if >50 years (post herpatic neuralgia rare if <50 years) and within 72 hours of rash or if 1 of the following: - active ophthalmic; - Ramsey Hunt; - eczema; - non-truncal involvement; - moderate or severe pain; - moderate or severe rash 2. shingles treatment if not within 72 hours: consider starting antiviral drug up to 1 week after rash onset if: - high risk of severe shingles or continued vesicle formation; - older age; - immunocompromised; - or severe pain medication used - acyclovir (IV for immunocompromised) - famciclovir - valaciclovir

Question 392

What are the complications of herpes zoster?

Answer 392

- post herpetic neuralgia = pai persisiting 3 or more months after resolution of rash - temporal paralysis = follows dermatome involvement and may affect bladder and bowel function - disseminated HZ (mainly in immunocompromised patients) - neurologic complications of HZ e.g. encephalitis, myelitis, meningitis, retinitis

Question 393

Glasgow Coma Scale

Answer 393

Eyes 1 to 4 (remember glasses = 4 eyes) None = 1 Open to pain = 2 open to voice = 3 spontaneous opening = 4 Voice (Roman numeral V = 5) 1 to 5 None sounds = 2 words = 3 Confused = 4 orientated = 5 Motor 1 to 6 none abnormal extension = 2 (decerebrate posturing = head, arms and legs extend and internally rotate, rigid = brain stem damage ) abnormal flexion = 3 (arm flexes in and draws in one chest = decorticate posturing) withdraws to pain = 4 (flexes arm away > cerebral hemisphere damage) localises pain = 5 (hand brought above clavicle) obeys commands = 6 lowest = 3 < 8 = coma

Question 394

Investigations: coma

Answer 394

- depends on suspected cause - basic blood including glucose - ABG - imaging = CT head, MRI head - lumbar puncture - EEG - toxicology and alcohol levels

Question 395

What are the features of brown sequard syndrome?

Answer 395

lateral hemisection of spinal cord - Same sided weakness and proprioception/vibration loss - Loss of pain/temp on opposite side 


Question 396

Define: myelopathy

Answer 396

syndrome for pathology involving the spinal cord - MC cause = disc-osteophyte cord compression

Question 397

Presentation: myelopathy

Answer 397

- loss of fine finger movements - difficulty walking, legs feel 'not my own' signs: long tract signs/upper motor neurone signs

Question 398

What are the upper motor neuron signs?

Answer 398

1. Spastic gait 2. Hypertonia 3. Hyper-reflexia 4. Babinski 5. Clonus 6. Cross-adductors 7. Hoffman's sign 8. Loss of fine finger movements (‘treacle hands’) 9. Deltopectoral reflex

Question 399

define: Venous sinus thrombosis

Answer 399

severe sudden onset headache

Question 400

Define: normal pressure hydrocephalus

Answer 400

Reversible cause of dementia seen in elderly patients - secondary to reduced CSF absorption at the arachnoid villi - changes may be secondary to head injury, SAH or meningitis

Question 401

What is the classical triad presentation in normal pressure hydrocephalus?

Answer 401

1. urinary incontinence 2. dementia and bradyphrenia 3. gait abnormality (may be similar to Parkinson's disease)

Question 402

Investigations: normal pressure hydrocephalus

Answer 402

CT head hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement

Question 403

Management: normal pressure hydrocephalus

Answer 403

- ventriculoperitoneal shunting around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages

Question 404

Presentation: B12 deficiency

Answer 404

extreme tiredness a lack of energy pins and needles (paraesthesia) a sore and red tongue mouth ulcers muscle weakness disturbed vision psychological problems, which may include depression and confusion problems with memory, understanding and judgement

Question 405

What are causes of B12 or folate deficiency?

Answer 405

1. pernicious anaemia = immune cells attack stomach preventing body from absorbing 2. lack of vitamins in diet 3. medication

Question 406

Define: subacute combined degeneration of the cord

Answer 406

vitamin B12 deficiency resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts

Question 407

Presentation: subacute combined degeneration of the spinal cord

Answer 407

1. dorsal column involvement - distal tingling/burning/sensory loss is - symmetrical = legs >arms - impaired proprioception and vibration sense 2. lateral corticospinal tract involvement - muscle weakness, - hyperreflexia - spasticity - upper motor neuron signs - typically develop in the legs first - brisk knee reflexes - absent ankle jerks - extensor plantars 3. spinocerebellar tract involvement - sensory ataxia → gait abnormalities - positive Romberg's sign

Question 408

What causes foot drop?

Answer 408

L5 nerve lesion Common perineal nerve lesion

Question 409

Treatment ladder: neuropathic pain

Answer 409

1. Amitryptylline - duloxetine - gabapentin - pregabalin 2. Tramadol 'rescue drug' 3. topical capsaicin = localised neuropathic pain

Question 410

What is the difference in presentation between CN3 nerve palsy and Horner's syndrome?

Answer 410

CN 3 - ptosis and dilated pupil (mydriasis) Horner's - ptosis + constricted pupil (miosis)

Question 411

Define: wernicke's encephalopathy

Answer 411

Thiamine deficiency - seen MC in alcoholics

Question 412

What is the triad for wernicke's?

Answer 412

1. ophthalmoplegia/nystagmus 2. ataxia 3. confusion

Question 413

What are some other symptoms of wernicke's?

Answer 413

CAN OPEN Confusion Ataxia Nystagmus Ophthamoplegia PEripheral Neuropathy

Question 414

Presentation: Korsakoff syndrome

Answer 414

- anterograde amnesia -retrograde amnesia - confabulation