Neuro fifth yr

Question 1

Summary of Huntington’s?

Answer 1

inherited neurodegenerative condition. It is progressive and incurable

Typically results in death 20 years after initial symptoms develop. Death often due to respiratory disease. Suicide common.

AD
trinucleotide repeat disorder: repeat expansion of CAG
HTT gene on chromosome 4
Anticipation - where the disease is presents at an earlier age in successive generations, and increased severity of disease

results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia

Question 2

Features of Huntington’s?

Answer 2

typical develop after 35 years of age

cognitive, psychiatric or mood problems

chorea (involuntary, abnormal movements)

personality changes (e.g. irritability, apathy, depression) and intellectual impairment

dystonia

saccadic eye movements

Speech difficulties (dysarthria)

Swallowing difficulties (dysphagia)

Question 3

Diagnosis and management of Huntington’s?

Answer 3

Dx
made in a specialist genetic centre using a genetic test for the faulty gene
pre-test and post-test counselling regarding the implications of the results

Tx
no treatment options for slowing or stopping the progression of the disease
supporting the person and their family
MDT - OT, PT, psychology, SALT, genetic counselling, advanced directives, EoL care planning

Symptomatic relief:
Medications to suppress disordered movement - antipsychotics (e.g., olanzapine), BDZs, Dopamine-depleting agents (e.g., tetrabenazine)
Depression - antidepressants

Question 4

Types of migraines?

Answer 4

Migraine without aura
Migraine with aura (visual changes - blurring, lines, loss of visual fields)
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine (hemiplegia, ataxia, change in consciousness - mimic stroke)

Question 5

What are migraines?

Answer 5

Neurological condition that is a cause of primary headache.
Occur in attacks following typical pattern
more common in women

Question 6

Features of migraines?

Answer 6

a severe, unilateral, throbbing headache
associated with nausea, photophobia and phonophobia
attacks may last up to 72 hours
patients characteristically go to a darkened, quiet room during an attack
‘classic’ migraine attacks are precipitated by an aura. These occur in around one-third of migraine patients
typical aura are visual, progressive, last 5-60 minutes and are characterised by transient hemianopic disturbance or a spreading scintillating scotoma
formal diagnostic criteria are produced by the International Headache Society
5 stages - prodromal, aura, headache, resolution, recovery

Question 7

Triggers for migraines?

Answer 7

Tired/stress
Alcohol
COCP
Lack of food or dehydration
Food - cheese, chocolate, red wine, citrus fruits
Menstruation
Bright lights

Question 8

Acute treatment of migraine?

Answer 8

5-HT receptor agonists acutely
first-line: offer combination therapy with an oral triptan and an NSAID, or an oral triptan and paracetamol

for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan

if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide* or prochlorperazine and consider adding a non-oral NSAID or triptan

Question 9

Prophylaxis of migraines?

Answer 9

5-HT receptor antagonist in prophylaxis
prophylaxis should be given if patients are experiencing 2 or more attacks per month.

topiramate or propranolol - Propranolol should be used in preference to topiramate in women of childbearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives

if these measures fail NICE recommends ‘a course of up to 10 sessions of acupuncture over 5-8 weeks’

migraines triggered by menstruation - prophylaxis with NSAIDs (mefanamic acid) or triptans

Question 10

What is Brown-Sequard syndrome?

Answer 10

Caused by lateral hemisection of the spinal cord

Features - ipsilateral weakness below lesion, ipsilateral loss of proprioception and vibration sensation, contralateral loss of pain and temperature sensation

Question 11

What is Parkinson’s disease?

Answer 11

a condition where there is a progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement. Characteristicially asymmetrical, with one side more affected than the other.

Basal ganglia is responsible for coordinating habitual movements such as walking or looking around, controlling voluntary movements and learning specific movement patterns. Part of the basal ganglia called the substantia nigra produces a neurotransmitter called dopamine.

Question 12

Features of Parkinsons disease?

Answer 12

typical patient is an older aged man around the age of 70. Twice as common in men. Mean age of diagnosis is 65 yrs.

Triad - resting tremor, rigidity, bradykinesia

Unilateral tremor - 4-6Hz, pill rolling, pronounced when resting, improves on voluntary movement, worsens if distracted

Cogwheel rigidity

Bradykinesia - movements slower and smaller - handwriting, shuffling gait, difficulty initiating movement, difficulty turning, hypomimia

Depression
Sleep disturbance and insomnia
Anosmia
Postural instability
Cognitive impairment and memory problems

Question 13

Difference between Parkinsons tremor and benign essential tremor?

Answer 13

Parkinsons - asymmetrical, 4-6Hz, worse at rest, improves with intentional movement, other Parkinson features, no changes with alcohol

Benign essential tremor - symmetrical, 5-8Hz, improves at rest, worse with intentional movement, no other Parkinsons features, improves with alcohol

Question 14

Diagnosis of Parkinsons?

Answer 14

Clinical diagnosis - should be made by a specialist
NICE recommend using UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria.
Can use single photon emission computed tomography (SPECT) to differentiate between Parkinsons and benign essential tremor

Question 15

Management of Parkinsons?

Answer 15

Treatment is initiated and guided by a specialist, and is tailored to each individual patient and their response to different medications.
No cure - focus on controlling Sx and minimising SE

Levodopa - boost dopamine levels, usually combined with drug that stops levodopa being broken down in the body (peripheral decarboxylase inhibitors - carbidopa, benserazide) - so combination drugs include: co-benyldopa, co-careldopa. Levodopa is most effective but becomes less effective over time - so is saved for when other treatments aren’t managing Sx. Too much dopamine is SE - cause dyskinesias (e.g., dystonia, chorea, athetosis)

COMT inhibitors - catechol-o-methytransferase inhibitors, e.g., entacapone. Taken with levodopa to slow breakdown of levodopa in the brain.

Dopamine agonists - mimic dopamine in BG and stimulate dopamine receptors. Less effective than levodopa, and used to delay use of levodopa. Eg. bromocryptine, pergolide, Cabergoline. SE - pulmonary fibrosis.

Monoamine oxidase-B inhibitors - Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. The monoamine oxidase-B enzyme is more specific to dopamine and does not act on serotonin or adrenalin. Hence increases circulating dopamine. Delays use of levodopa. Eg. selegiline, rasagiline

Question 16

Examples of Parkinson-plus syndromes?

Answer 16

MSA
Dementia with Lewy bodies
Progressive Supranuclear Palsy
Corticobasal degeneration

Question 17

Features of drug-induced Parkinsonism?

Answer 17

motor symptoms are generally rapid onset and bilateral
rigidity and rest tremor are uncommon

Question 18

Summary of multi system atrophy?

Answer 18

2 types - MSA-P (predominant Parkinsonism features) and MSA-C (predominant cerebellar features)

Shy-Drager syndrome is a type of MSA

Features - Parkinsonism, autonomic disturbance (ED, postural hypotension, atonic bladder, constipation, sweating), cerebellar signs

Question 19

Summary of progressive supranuclear palsy?

Answer 19

Features - postural instability and falls, impairment of vertical gaze (pt may complain of difficulty reading or descending stairs as down gaze is worse than up gaze), Parkinsonism, cognitive impairment (primary frontal lobe dysfunction)

Management - poor response to L-dopa

Question 20

Summary of Lewy body dementia?

Answer 20

Accounts for 20% of cases

Alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas.

Features - progressive cognitive impairment, typically before Parkinsonism. Fluctuating cognition. Early impairments in attention and and executive function rather than just memory loss. Visual hallucinations.

Diagnosis - clinical. SPECT.

Management - acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine. Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism.

Question 21

Summary of corticobasal degeneration?

Answer 21

Between 50 - 70
Increasing numbers of brain cells becoming damaged or dying - due to build up of tau

Features - ‘useless hand’, muscle stiffness, tremors, dystonia, cerebellar Sx, slow and slurred speech, Sx of dementia, difficulty swallowing - usually one limb and then spreads to the rest of the body

Question 22

Conditions causing tremor?

Answer 22

Parkinsonism - resting, pill rolling

Essential tremor - postural tremor (worse if arms outstretched, improves with alcohol and rest, titubation (head tremor), strong FHx)

Anxiety

Thyrotoxicosis

Hepatic encephalopathy - Hx of CLD

CO2 retention - Hx of COPD

Cerebellar disease - intention tremor, past-pointing, nystagmus

Drug withdrawal - alcohol, opiates

Question 23

Management of essential tremor?

Answer 23

Propranolol first line
Primidone

Question 24

Pathway of facial nerve?

Answer 24

Exits brain stem at cerebellopontine angle. Passes through temporal bone and parotid gland/

Divides into 5 branches - temporal, zygomatic, buccal, marginal mandibular and cervical

Question 25

Function of facial nerve?

Answer 25

Motor - supples muscles of facial expression, stapedius in inner ear, and posterior digastric, stylohyoid and platysma muscles in the neck. Sensory - carries taste from anterior 2/3 of tongue Parasympathetic - supply to the submandibular and sublingual salivary glands and the lacrimal gland (stimulating tear production).

Question 26

Summary of Bell's palsy?

Answer 26

acute, unilateral, idiopathic, facial nerve paralysis aetiology unknown - role of HSV has been investigated peak incidence is 20-40 years. common in pregnant women

Question 27

Features of Bells palsy?

Answer 27

LMNL facial nerve palsy - forehead affected, no sparing post-auricular pain - may precede paralysis altered taste dry eyes hyperacusis

Question 28

Management of Bells palsy?

Answer 28

oral prednisolone within 72 hours of onset of Bell's palsy - 50mg for 10 days debate about use of anti-virals eye care - artificial tears, eye lubricants, tape closed at night f the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT a referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months

Question 29

Summary of Ramsay-Hunt syndrome?

Answer 29

caused by the varicella zoster virus (VZV) unilateral lower motor neurone facial nerve palsy painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This rash can extend to the anterior 2/3 of the tongue and hard palate. Treatment should ideally be initiated within 72 hours. Treatment is with: Prednisolone Aciclovir Also - lubricating eye drops

Question 30

Causes of LMN facial nerve palsy?

Answer 30

Bells Ramsay Hunt Infection: Otitis media, Malignant otitis externa, HIV, Lyme’s disease Systemic disease: Diabetes, Sarcoidosis, Leukaemia, Multiple sclerosis, Guillain–Barré syndrome Tumours: Acoustic neuroma, Parotid tumours, Cholesteatomas Trauma: Direct nerve trauma, Damage during surgery, Base of skull fractures

Question 31

Summary of trigeminal neuralgia?

Answer 31

trigeminal nerve - 3 branches - ophthalmic, maxillary, mandibular syndrome characterised by severe unilateral pain. Majority idiopathic, but can be due to compression of trigeminal roots by tumours or vascular problems Features unilateral disorder - brief electric shock like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve pain evoked by light touch - washing, shaving, smoking, talking and brushing teeth, and frequently spontaenously trigger areas - nasolabial fold or chin pains usually remit for variable periods Red flags - sensory changes, deafness or other ear problems, history of skin or oral lesions that could spread perineurally, pain only in ophthalmic division of trigeminal nerve or bilaterally, optic neuritis, FHx of MS, age of onset before 40 Tx - carbamazepine, failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology, surgery to decompress or intentionally damage the trigeminal nerve is an option.

Question 32

What is Guillain-Barre syndrome?

Answer 32

acute paralytic polyneuropathy that affects peripheral nervous system immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni, also CMV, EBV) cross-reaction of antibodies with gangliosides in the peripheral nervous system - anti-ganglioside antibody. Due to molecular mimicry

Question 33

What is Miller-Fisher syndrome?

Answer 33

variant of Guillain-Barre syndrome associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome anti-GQ1b antibodies are present in 90% of cases

Question 34

Features of Guillain-Barre syndrome?

Answer 34

Symptoms usually start within 4 weeks of the preceding infection. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years. 65% of pt's have back/leg pain in the initial stages of the illness progressive, symmetrical weakness of all limbs - classically ascending (legs first) reflexes are reduced or absent sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs Hx of gastroenteritis respiratory muscle weakness CN involvement - diplopia, bilateral facial nerve palsy, oropharyngeal weakness is common autonomic involvement - urinary retention, diarrhoea less common - papilloedema (though to be secondary to reduced CSF resorption

Question 35

Ix of Guillain Barre syndrome?

Answer 35

lumbar puncture - rise in protein with normal WBC count nerve conduction studies - decreased motor nerve conduction velocity (due to demyelination)

Question 36

Management of Guillain Barre syndrome?

Answer 36

IV immunoglobulins Plasma exchange (alternative to IV IG) Supportive care VTE prophylaxis (pulmonary embolism is a leading cause of death) In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.

Question 37

Prognosis of Guillain Barre syndrome?

Answer 37

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

Question 38

What is motor neurone disease?

Answer 38

is a progressive, ultimately fatal condition where the motor neurones stop functioning. There is no effect on the sensory neurones and patients should not experience any sensory symptoms.

Question 39

Pathophysiology of motor neurone disease?

Answer 39

progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared. There is a genetic component and many genes have been linked with an increased risk of developing the condition. increased risk with smoking, exposure to heavy metals and certain pesticides.

Question 40

Symptoms of MND?

Answer 40

typical patient is a late middle aged (e.g. 60) man, possibly with an affected relative insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. often first noticed in the upper limbs increased fatigue when exercising may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria). Doesn't affect external ocular muscles No cerebellar signs Abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

Question 41

Signs of motor neurone disorder?

Answer 41

Signs of LMNL - muscle wasting, reduced tone, fasciculations, reduced reflexes Signs of UMNL - increased tone or spasticity, brisk reflexes, upping plantar responses

Question 42

Diagnosis of MND?

Answer 42

Based on clinical presentation and excluding other conditions Should be made by specialist Unfortunately, the diagnosis is often delayed, which causes considerable anxiety and stress. Normal motor conduction - show normal motor conduction and can help exclude a neuropathy. EMG - shows a reduced number of action potentials with increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy

Question 43

Management of MND?

Answer 43

No effect treatments for heating or reversing the progression of the disease MDT approach Riluzole slow progression of the disease and extend survival by a few months in ALS - initiated by a specialist Edaravone - used in USA NIV used at home to support breathing at night improves survival PEG for nutrition End of life care planning

Question 44

Types of MND?

Answer 44

Amyotrophic lateral sclerosis (50% of patients) typically LMN signs in arms and UMN signs in legs in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase Primary lateral sclerosis UMN signs only Progressive muscular atrophy LMN signs only affects distal muscles before proximal carries best prognosis Progressive bulbar palsy palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei carries worst prognosis

Question 45

What is multiple sclerosis?

Answer 45

chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system

Question 46

Epidemiology of multiple sclerosis?

Answer 46

F>M 3:1 aged 20-40 more common at higher latitudes

Question 47

Subtypes of multiple sclerosis?

Answer 47

Relapsing-remitting disease most common form, accounts for around 85% of patients acute attacks (e.g. last 1-2 months) followed by periods of remission Secondary progressive disease describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis gait and bladder disorders are generally seen Primary progressive disease accounts for 10% of patients progressive deterioration from onset more common in older people

Question 48

Features of multiple sclerosis?

Answer 48

non-specific features - lethargy visual - optic neuritis, optic atrophy, Uhthoff's phenomenon (worsening of vision following rise in body temperature), internuclear ophthalmoplegia Sensory - pins/needles, numbness, trigemintal neuralgia, Lhermitte's syndrome (paraesthesiae in limbs on neck flexion) Motor - spastic weakness (commonly seen in legs) Cerebellar - ataxia (seen in acute relapse), tremor Others urinary incontinence sexual dysfunction intellectual deterioration

Question 49

Diagnosis of multiple sclerosis?

Answer 49

made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time determination of "lesions disseminated in space and time” - multiple parts of the CNS and over course of time

Question 50

Ix of multiple sclerosis?

Answer 50

MRI high signal T2 lesions periventricular plaques Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum CSF oligoclonal bands (and not in serum) increased intrathecal synthesis of IgG Visual evoked potentials delayed, but well preserved waveform

Question 51

Management of acute relapse of multiple sclerosis?

Answer 51

High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse. It should be noted that steroids shorten the duration of a relapse and do not alter the degree of recovery (i.e. whether a patient returns to baseline function)

Question 52

Management of multiple sclerosis?

Answer 52

MDT - neurologist, specialist nurses, PT, OT, pt education DMARDs - reduce risk of relapses indication - relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided) options - natalizumab ocrelizumab fingolimod beta-interferon glatiramer acetate fatigue - exclude (anaemia, DM, depression) - trial of amantadine, mindfulness, CBT spasticity - baclofen and gabapentin are first line. PT, cannabis, botox bladder dysfunction - may take the form of urgency, incontinence, overflow etc, US to assess bladder emptying, if significant residual volume → intermittent self-catheterisation if no significant residual volume → anticholinergics may improve urinary frequency oscillopscia - visual fields oscillate - gabapentin

Question 53

What is Erb-Duchenne paralysis?

Answer 53

Damage to C5/6 roots Winged scapula May be caused by breech presentation

Question 54

What is Klumpke's paralysis?

Answer 54

Damage to T1 Loss of intrinsic hand muscles Due to traction

Question 55

Criteria for brain stem death testing?

Answer 55

Deep coma of known aetiology Reversible causes excluded No sedation Normal electrolytes

Question 56

How to test for brain stem death?

Answer 56

Fixed pupils which do not respond to sharp changes in the intensity of incident light No corneal reflex Absent oculo-vestibular reflexes - no eye movements following the slow injection of at least 50ml of ice-cold water into each ear in turn (the caloric test) No response to supraorbital pressure No cough reflex to bronchial stimulation or gagging response to pharyngeal stimulation No observed respiratory effort in response to disconnection of the ventilator for long enough (typically 5 minutes) to ensure elevation of the arterial partial pressure of carbon dioxide to at least 6.0 kPa (6.5 kPa in patients with chronic carbon dioxide retention). Adequate oxygenation is ensured by pre-oxygenation and diffusion oxygenation during the disconnection (so the brain stem respiratory centre is not challenged by the ultimate, anoxic, drive stimulus) Done by 2 appropriately experienced Drs on 2 separate occasions - both experienced in brain stem death + 5 years post-grad experience + one must be consultant

Question 57

Predictive factors in SAH?

Answer 57

conscious level on admission age amount of blood on CT head

Question 58

Complications of SAH?

Answer 58

re-bleeding, hydrocephalus, vasospasm, hyponatraemia, seizures

Question 59

Tx of SAH?

Answer 59

supportive (bed rest, analgesia, VTE prophylaxis, discontinuation of antithrombotics) nimodipine for vasospasm intracranial aneurysms - risk of rebleeding so treated with coil or clipping

Question 60

Tx of hydrocephalus?

Answer 60

external ventricular drain - right lateral ventricle Ventriculoperitoneal shunt - long-term CSF diversion technique drains from ventricles to peritoneum Obstructive - remove pathology

Question 61

Ix for hydrocephalus?

Answer 61

CT, MRI, LP

Question 62

Causes of non-obstructive hydrocephalus?

Answer 62

- imbalance of CSF production absorption: increased CSF production (choroid plexus tumour) or more commonly a failure of reabsorption at the arachnoid granulations (e.g. meningitis or post-haemorrhagic).

Question 63

Causes of obstructive hydrocephalus?

Answer 63

tumour, acute haemorrhage, developmental abnormalities

Question 64

Types of head injuries?

Answer 64

diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons intra-cranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact

Question 65

Subdural bleed blood vessel?

Answer 65

convex - middle meningeal artery

Question 66

Extra dural bleed blood vessel?

Answer 66

bridging veins concave

Question 67

Bells palsy - ipsilateral or contralateral?

Answer 67

ipsilateral

Question 68

How to differentiate between AICA and PICA strokes?

Answer 68

AICA strokes can be differentiated from PICA strokes as AICA causes ipsilateral facial paralysis and deafness, but PICA does not.

Question 69

Summary of T1 nerve root lesion?

Answer 69

wasting of intrinsic muscles of hand weakness of finger abduction and adduction and thumb adduction finger flexion normal altered touch along ulnar border of forearm reflex normal

Question 70

What to give for SCC?

Answer 70

dexamethasone

Question 71

CSF findings of bacterial meningitis?

Answer 71

High pressure, raised protein, excess neutrophils

Question 72

When to start anti-epileptic Tx?

Answer 72

anti-epileptic treatment should not be started after the first episode of seizure, unless in the following circumstances: * the presence of a neurological deficit * structural abnormality on brain imaging * unequivocal epileptic activity on the EEG * the patient, family or carers consider the risk of having a further seizure is unacceptable

Question 73

Ankle reflex?

Answer 73

S1-S2

Question 74

Knee reflex?

Answer 74

L3-L4

Question 75

Biceps reflex

Answer 75

C5-C6

Question 76

Triceps reflex?

Answer 76

C7-C8

Question 77

How does tetanus present?

Answer 77

Trismus - lock jaw Facial spasms - grimace expression, forced grinning Back pain Increased tone Dysphagia Spasms Temperatures Injecting drug habits is a RF for tetanus infection

Question 78

How does botulism present?

Answer 78

Flaccid paralysis (not spasms) Usually symmetrical and descending - muscles of neck, shoulders, upper extremities first

Question 79

CN6 palsy?

Answer 79

Defective abduction Horizontal diplopia (could be first sign of brain mets) Lateral rectus