Neuro Qs

Question 1

What is a seziure? What is epilepsy?

Answer 1

Seizure = convulsion or transient event caused by a paroxysmal discharge of cerebral neurones

Epilepsy = continued tendency to have seizures

Question 2

How is epilepsy treated?

Answer 2

Primary generalised - valprate or lamotrigine

Absence seizure - valproate or ethosuxamide

partial seizure - carbamazepine, lamotrigine

Question 3

How would you diagnose epilepsy? What specific sign would you see in a petit mal seizure?

Answer 3

Electroencephalography and CT/MRI (structural abnormalities?)

Genetical testing in Juvenile myoclonic epilepsy

3 Hz spike and wave for petit mal (absence seizures)

Question 4

Give a side effect for each anti-epileptic drug you may prescribe

Answer 4

Sodium valproate – nausea, diarrhoea, weight gain

Lamotrigine – Steven-Johnson syndrome, diplopia, blurred vision

Carbamazepine – Steven-Johnson syndrome - SEVERE SKIN REACTION, hyponatraemia, impaired balance

Question 5

What the triad of parkinson’s disease? Name 3 extra neuro symptoms

Answer 5

Triad = pill rolling tremor, bradykinesia, rigidity (postural instability and gait small shuffling)

Others= depression, anosmia, hallucinations, constipation, frequency

Question 6

What causes Parkinson’s disease (pathophysiology)

Answer 6

Progressive degeneration of dopaminergic neurons in the sunstantia nigra pars compacta of the basal ganglia, associated with the presence of Lewy bodies.
This causes a decrease in striatal dopamine levels (and melanin) leading to cell loss and akinesia

(smoking is protective!!)

Question 7

Describe the treatment of Parkinson’s disease, what side effects are associated?

Answer 7

1. Levodopa + decarboxylase inhibitor e.g. carbidopa
   (Levodopa is a precursor of dopamine and can cross the BBB, the decarboxylase inhibitor prevents peripheral conversion of levodopa which reduces SE of N/V, arrhythmias, alopecia and hypotension. There is reduced efficacy over time so L-dopa should only be started when other treatments are ineffective.
2. Dopamine agonists e.g. ropinirole
   SE: drowsy, nausea, hallucinations, compulsive behaviour
3. Monoamine oxidase B inhibitors (MAOB-I) e.g. selegiline, rasagiline - reduces dopamine breakdown
   SE: postural hypotension, AF
4. COMT inhibitors e.g. entacapone
   reduces breakdown of dopamine
   SE: can cause liver damage

Question 8

What is Lewy body dementia?

Answer 8

When dementia occurs prior to or at the same time as motor symptoms
If dementia occurs more than 1 year after motor symptoms occur then it is a feature of Parkinson’s disease

Question 9

What symptoms are seen in Huntington’s? What is diagnosis reached?

Answer 9

Mean onset is 30-50years

Symptoms: progressive, often begins with psychotic and behaviour symptoms, chorea (jerky movements, fidgidity movements, restlessness), lack of coordination, slow saccadic eye movements
Progressives to rigidity, dysphagia and behaviour change - aggressive, depression, self-neglet

Diagnosis: clinical, genetic, imaging - may show caudate nucleus atrophy and increase size of lateral ventricles

Question 10

What is the pathophysiology of Huntington’s disease?

Answer 10

AD mutation in chromosome 4- huntingtin gene leading to huntintin protein overexpression (CAG repeats)

Cerebral atrophy of caudate nucleus and putamen of basal ganglia
- loss of corpus striatum GABA-nergic and cholinergic neurons - decreased ACh synthesis and GABA in the striatum
(dopamine levels are normal, high somatostatin, reduced ACh synthesis, depleted GABA and ACE in corpus striatum)

Question 11

What is the treatment of Huntington’s disease?

Answer 11

SYMPTOMATIC
(Counselling/genetic testing)

1. Benzodiazepams - diazepam
2. Sodium valproate - anticonvulsant
3. Tetrabenazine - used in hyperkinetic movement, promotes the degradation of dopamine, helps chorea
4. Mood stabilisers e.g. carbamazepine
5. Antidepressant e.g. amitriptyline

Also: speech therapy, physio, occupational therapy, pschyo therapy etc

Question 12

What may trigger a tension headache? (3)
How may a patient describe the headache? (2)
Describe the management (2)

Answer 12

Triggers: stress, anxiety, noise, fumes, concentrated visual efforts
Headache is: bilateral, ‘tight band like sensation’
+- tenderness (no vomiting, aura)

Management: clinical diagnosis, life style: exercise, avoid triggers, NSAIDS, acupuncture
Consider amitriptyline

Question 13

What may trigger a migraine? (3)
How may a patient describe the headache? (2)
Describe the management (2)

Answer 13

Triggers: cheese, caffeine, alcohol, menstruation, oral contraceptives, anxiety, travel, exercise (F>M, onset < 40yrs)

5+ Headaches: unilateral, lasts 4-72hrs, pulsatile/throbbing, aggravated by movement, nausea +- vomiting, photophobia/phonophobia

Management: avoid triggers, oral triptan (e.g. sumatriptan), NSAIDS, anti-emetic if needed e.g. metoclopramide
Prophylaxis - BB - e.g. propanolol
Consider amitriptyline

Question 14

Clinical features of cluster headache? (2)
Name 2 features specific to the eye (2)
Name an acute and a chronic/prophylactic treatment

Answer 14

Clinical features: rapid onset, rises on crescendo over minutes and lasts 15-160 mins, very severe, occurs in clusters (1/2 x a day), +- vomiting

Eye symptoms: Lacrimation, miosis, transient ipsilateral Horner’s, lid swelling, unilateral eye pain, may awake pt from sleep +- ptosis

Tx

acute: 100% oxygen, sumatripitan (s/c) at onset
chronic: verapamil CCB, avoid alcohol, prednisolone

Question 15

3 clinical features of giant cell arteritis (GCA) (3)

Answer 15

GCA: temporal pulsating headache, scalp tenderness, artery has reduced pulsating and is tender/pulseless, jaw claudication, amaurosis fungax - typically in one eye
Systemic: fatigue + fever, breathless, myalgia, morning stiffness

Question 16

3 causes of spinal cord compression

What is the most common

Answer 16

Most common - disc herniation at L4/L5

Secondary malignancy – breast, lung, prostate, thyroid, kidney
TB – Pott’s disease = destruction of vertebral bodies and disc spaces
Epidural haemorrhage or haematoma
Prolapsed disc – usually L4/5 or L5/S1

MYELOPATHY = CORD COMPRESSION = UMN
RADICULOPATHY = ROOT COMPRESS = LMN

Question 17

What occurs in Brown Sequard syndrome?

Answer 17

Hemisection of spinal cord results in..

Contralateral loss of pain and temperature
Ipsilateral loss of proprioception, vibration and power

Question 18

What occurs in Cauda Equina syndrome?

What investigation is gold standard? And what should you do?

Answer 18

Nerve root compression caudal to the termination of the spinal cord - usually large central disc herniation L4/L5, OR L5/S1
Generally S1-S5 = important in bladder function

WITHIN 24 HOURS
MRI – refer to neuro - surgery (epidural steroid injection)

Question 19

What symptoms are in cauda equina syndrome?

Answer 19

Bilateral/unilateral pain in legs
Variable leg weakness
Saddle anaesthesia  (loss of sensation around buttock, perineum, inner thigh)
Poor anal tone
Erectile dysfunction
Bladder/bowel dysfunction

Question 20

Name 4 causes of TIA

Answer 20

Mostly due to micro emboli - Atrial fibrillation, atherosclerosis thrombi, vasculitis, antiphospholipid syndrome, thrombophilias, sickle cell, polycythaemia

Question 21

Describe the management of TIA

iv and rx

Answer 21

Brain imaging: MRI
Carotid doppler
ECG - AF?
ABCDD - age > 60, BP > 140/90, clinical weakness = 2, speech impairment = 1, duration 10-59mins = 1, >60mins = 2, DM?

TX: 2 weeks of aspirin than life long clopidogrel
Start satin, and don’t drive for 4 weeks.
Life style: stop smoking, diet, exercise, HTN control etc

Question 22

Describe the Oxford/Bamford classification of strokes, which vessels are affected?

Answer 22

Total anterior circulation stroke (TACS): middle cerebral artery, anterior cerebral artery

1. Unilateral weakness (and/or sensory deficit) of face, arm, leg
2. Homonymous hemianopia
3. Higher cerebral function - dysphasia, visuospatial

Posterior anterior circulation stroke: middle cerebral artery and anterior cerebral artery, 2 of the three for TACS

Posterior circulation syndrome (POCS), one of: cerebellar or brainstem syndromes

1. Loss of consciousness
2. Isolated homonymous hemianopia

Lacunar syndrome (LACS): subcortical (midbrain, internal capsule) one of…

1. Unilateral weakness (and/or sensory deficit) of face and arm, arm and leg, or all three
2. Pure sensory loss
3. Ataxic hemiparesis (Cerebellar and motor syndrome)

Question 23

What are the 5 types of lacunar stroke?

Answer 23

1. Motor
2. Ataxia hemiparesis
3. Sensory
4. Mixed
5. Dysarthria

Question 24

Describe the management of a cerebrovascular accident

Answer 24

URGENT CT - to direct treatment - an infarction is seen as a low density lesion

1. WITHIN 4.5 hrs - THROMBOLYSIS - IV tissue plasminogen activator e.g. alteplase
2. AFTER 24 hrs - ANTIPLATELET e.g. clopidogrel

(If after 4.5 hours then give aspirin for 2 days, then clopidogrel)

Question 25

Subarchnoid haemorrhage 1. Clinical features 2. Cause? 3. Ix? 4. Tx?

Answer 25

1. Sudden onset thunderclap headache, neck stiffness, Kernig's sign, altered level of consciousness, drowsiness, collapse, seizure, papilledema 2. Most often berry aneurysm (HTN) - anterior communicating artery 3. CT - star shaped LP - bloody then becomes yellow - XANTHOCHROMIC due to Hb break down to Hb 4. Dexamethasone to decrease oedema, refer to neuro for clipping or coiling urgent. Nimodipine to reduce risk of vasospasm.

Question 26

What is Kernig's sign?

Answer 26

Stiffness of hamstrings causes an inability to straighten the leg when hip is at 90 degrees

Question 27

Subdural haemorrhage 1. Cause? 2. Clinical features 3. Who? 4. Ix? 5. Tx?

Answer 27

1. Usually due to head injury, there is a latent stage when the tearing of bridging veins causes a haematoma to form, increasing ICP and shifting the midline structures - lucid period days/weeks/months 2. raised ICP = headache, nausea, vomiting, raised BP, confusion, seizures, focal neurology (GCS), cognitive decline, personality change 3. More common in elderly and alcoholics due to cerebral atrophy, physical abuse in infants 4. CT - hyperdense crescent shape/sickle shape (becomes isodense then hypodense) 5. Surgery - craniotomy - mannitol if raised ICP

Question 28

Extradural haemorrhage 1. Cause? 2. Clinical features 3. Who? 4. Ix? 5. Tx?

Answer 28

1. Head trauma - most commonly due to fracture of the temporal or parietal bone - laceration of middle meningeal artery (blood accumulates between bone and dura) 2. Brief post-traumatic loss of consciousness Lucid period for several hours/days, followed by altered consciousness. Severe headache, nausea and vomiting, confusion, seizures, neuro deficit - may lead to rapid increase in ICP 3. Often YP - trauma 4. CT - hyperdense biconcave/egg shaped (XR may show fracture) 5. Surgery - craniotomy - mannitol if raised ICP

Question 29

What is the cauda equina?

Answer 29

A bundle of spinal nerves and spinal nerve roots that consist of L2-5, S1-5 and coccygeal nerve, they innervate the lower limbs and pelvic organs. They also supply the sensory innervation to the perineum and parasympathetic supply to the bladder

Question 30

Describe the pathophysiology of MS

Answer 30

Inflammatory process in the white matter of the brain and cord mediated by CD4 T cells In active lesions, there is an increase in inflammatory cells, active myelin degradation and phagocytosis; it heals incompletely causing relapsing and remitting Plaques are not seen in peripheral nerves Acute relapses are caused by focal inflammatory demyelination

Question 31

What clinical features are seen in MS? Which signs?

Answer 31

Brainstem – diplopia, vertigo, facial numbness/weakness, dysarthria or dysphagia Spinal cord – spastic paraparesis, urinary symptoms Lhermitte’s sign By bending the head forward, the patient feels an electrical sensation down the back and limbs – due to lesion of dorsal columns

Question 32

What is McDonald Criteria?

Answer 32

MS - Uses frequency of attacks and lesions (MRI - disseminated in time and space) to diagnose MS e.g. 1 attack + 1 lesion + MRI of lesions disseminate in time and space or 2 attacks + 2 lesions - more than one potential relapse

Question 33

What investigations for MS?

Answer 33

1. Electrophysiology: delayed nerve conduction studies 2. MRI - periventricular lesions - discrete white matter abnormalities 3. LP - increased protein levels, oligoclonal IgG bands

Question 34

Treatment for MS

Answer 34

Relapse: oral methylprednisolone for 5 days OD or IV DMARDS: Alemtuzumab - CD52 monoclonal antibody , dimethyl fumarate, natalizumab Symptom management: Spasticity (Physio, baclofen, benzodiazepines), Botox injection and intermittent self catheterisation- for urgency and frequency, incontience - anticholinergic drugs e.g. doxizosin

Question 35

Courses of MS (4)

Answer 35

Relapsing and remitting Secondary progressive – so starts as R+R Primary progressive Progressive relapsing

Question 36

Myasthenia gravis is an autoimmune condition, explain how the pathophysiology works

Answer 36

There is an autoimmune reaction producing antibodies to nicotinic acetylcholine receptors which interfere with neuromuscular transmission by depleting receptor sites. Immune complexes of anti-acetylcholine receptor IgG and complement are deposited at the post- synaptic membranes. There is a second group of antibodies against muscle specific receptors, tyrosine kinase (anti-MUSK) Associated with thyroid disease, RA, pernicious anaemia and SLE

Question 37

What treatment causes a transient form of the condition?

Answer 37

Penicillamine (used in Wilson's disease as a chelating agent)

Question 38

Describe the clinical features of Myasthenia gravis

Answer 38

1. Weakness and fatiguability - ask pt to count to 50 - voice becomes less audible 2. Begins in proximal muscles 3. Also affects extra ocular muscles, facial expression and mastication, speech, fatiguable weakness - diplopia, ptosis 4. Acute resp failure due to weakness of resp muscles * no muscle pain, sensory change, normal tone, no wasting, normal reflexes - but may be fatiguable - does not affect peripheral muscles * Ask to look up high to look at finger - eyes will drop after a few seconds * worsened by pregnancy/infection/BB

Question 39

Investigations for Myasthenia gravis

Answer 39

Ix 1. Serum antibodies - anti-AChR or anti-MuSK (muscle-specific receptor tyrosine kinase) 2. CT of thymus - hyperplasia in 70% - may due to a tumour 3. Tensilon test - IV edrophonium given (short acting anti cholinesterase inhibitor) and power increases within seconds - rarely used for diagnosis 4. Neurophysiology - reduced evoked muscle action potentials during repeated nerve stimulation

Question 40

Treatment of Myasthenia gravis

Answer 40

1. Symptom control: oral acetyl cholinesterase inhibitors, e.g. pyridostigmine, neostigmine – duration of action 3-4 hours, prolong the action of ACh. Have cholinergic side effects: increased salivation, lacrimation, sweats, vomiting, diarrhoea, miosis 2. Immunosuppression can be used to treat relapses or if there is no response to pyridostigmine – reducing regimen of prednisolone 3. Azathioprine or methotrexate may also be given as the disease becomes more general 4. Thymectomy if onset <50 years and disease not easily controlled, or if a thymoma is found on CT. 5. Plasmapheresis or IVIg may be of use during severe exacerbation.

Question 41

What syndrome mimics myasthenia gravis and what is it caused by?

Answer 41

Lambert – Eaton syndrome, paraneoplastic manifestation of small cell bronchial carcinoma due to defective Ach release at neuromuscular junction. The difference is there are absent reflexes and autonomic involvement - dry mouth, constipation, impotence. Manage with immunoglobulins..