Neurology

Question 1

Epilepsy - pregnancy and breast feeding?

Answer 1

Take folic acid
Aim for mono therapy

Sodium valproate - associated with neural tube defects
Carbamazepine -often the least teratogenic
Phenytoin - associated with cleft palate
lamotrigine - congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy

Breast feeding - considered safe with anti-epileptics apart from barbiturates

It is advised that pregnant women taking phenytoin are given vitamin K in the last month of pregnancy to prevent clotting disorders in the newborn

Question 2

what is restless leg syndrome?

Answer 2

Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population.

Question 3

clinical features of restless legs syndrome?

Answer 3

uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest
paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)

Question 4

Causes and associations of restless legs syndromes?

Answer 4

there is a positive family history in 50% of patients with idiopathic RLS
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy

Question 5

Treatment of restless leg syndrome?

Answer 5

simple measures: walking, stretching, massaging affected limbs
treat any iron deficiency
dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
benzodiazepines
gabapentin

Question 6

Features of a third nerve palsy ?

Answer 6

eye is deviated ‘down and out’
ptosis
pupil may be dilated (sometimes called a ‘surgical’ third nerve palsy)

Question 7

Causes of 3rd nerve palsy?

Answer 7

diabetes mellitus
vasculitis e.g. temporal arteritis, SLE
false localizing sign* due to uncal herniation through tentorium if raised ICP
posterior communicating artery aneurysm
pupil dilated
often associated pain
cavernous sinus thrombosis
Weber’s syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes
other possible causes: amyloid, multiple sclerosis

Question 8

Clinical signs of Uncal herniation?

Answer 8

Signs of uncal herniation may include loss of consciousness, hypertension, bradycardia and Cheyne-Stokes respiration.

Question 9

what drug is contraindicated in absence seizures?

Answer 9

Carbamazepine

Question 10

Features of essential Tremor?
Management?

Answer 10

AD inheritance

Features
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor) - usually worse on movement and during stress - relieved by alcohol and sleep

Management
propranolol is first-line
primidone is sometimes used

Question 11

What is Spastic Paraparesis?

Answer 11

Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs

Question 12

causes of spastic paraparesis?

Answer 12

demyelination e.g. multiple sclerosis
cord compression: trauma, tumour
parasagittal meningioma
tropical spastic paraparesis
transverse myelitis e.g. HIV
syringomyelia
hereditary spastic paraplegia
osteoarthritis of the cervical spine

Question 13

where is wernicke’s area found?

Answer 13

Brodmann area 22 in the superior temporal gyrus

Question 14

treatment for MND?

Answer 14

Riluzole
prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis
prolongs life by about 3 months

Respiratory care
non-invasive ventilation (usually BIPAP) is used at night
studies have shown a survival benefit of around 7 months

Nutrition
percutaneous gastrostomy tube (PEG) is the preferred way to support nutrition and has been associated with prolonged survival

Prognosis
poor: 50% of patients die within 3 years

Question 15

what is the treatment for myoclonic seizures?

Answer 15

males: sodium valproate
females: levetiracetam

Question 16

what is the treatment for absence seizures?

Answer 16

first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures

Question 17

What is Myotonic Dystrophy?

Answer 17

Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.

Question 18

What are the genetics of myotonic dystrophy?

Answer 18

autosomal dominant
a trinucleotide repeat disorder
DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19
DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3

Question 19

What are the key difference between the two types of myotonic dystrophy?

Answer 19

DM1
- DMPK gene on chromosome 19
- Distal weakness more prominent

DM2
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
- Severe congenital form not seen

Question 20

Features of myotonic dystrophy?

Answer 20

General features
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria

Other features
myotonia (tonic spasm of muscle)
weakness of arms and legs (distal initially)
mild mental impairment
diabetes mellitus
testicular atrophy
cardiac involvement: heart block, cardiomyopathy
dysphagia

Question 21

what are the features of Miller Fisher syndrome?

Answer 21

ssociated 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 22

what antibodies are associated with Miller Fisher Syndrome?

Answer 22

anti-GQ1b antibodies are present in 90% of cases

Question 23

What is Anti-NMDA receptor encephalitis?

Answer 23

Anti-NMDA receptor encephalitis is a paraneoplastic syndrome, presenting as prominent psychiatric features including agitation, hallucinations, delusions and disordered thinking; seizures, insomnia, dyskinesias and autonomic instability

Question 24

What is associated with Anti-NMDA receptor encephalitis?

Answer 24

Ovarian teratomas are detected in up to half of all female adult patients, particularly prevalent in Afro-Caribbean patients

Question 25

How is Anti-NMDA receptor encephalitis managed?

Answer 25

Treatment of anti-NMDA encephalitis is based of immunosuppression with intravenous steroids, immunoglobulins, rituximab, cyclophosphamide or plasma exchange, alone or in combination. Resection of teratoma is also therapeutic.

Question 26

What is multiple system atrophy?

Answer 26

There are 2 predominant types of multiple system atrophy
1) MSA-P - Predominant Parkinsonian features
2) MSA-C - Predominant Cerebellar features

Shy-Drager syndrome is a type of multiple system atrophy.

Features
parkinsonism
autonomic disturbance
- erectile dysfunction: often an early feature
- postural hypotension
- atonic bladder
cerebellar signs

Question 27

how to differentiate between PSP, MSA and LBD?

Answer 27

Parkinson+cerebellar sign+autonomic dysfunction =Multiple system atrophy
Parkinson + vertical gaze palsy= PSP
Parkinson + visual hallucinations +deteriorated by antipsychotic =Lewy body dementia

Question 28

What is Lambert-Eaton Syndrome?

Answer 28

Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.

Question 29

What are the features of Lambert-Eaton Syndrome?

Answer 29

repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis)
in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease

limb-girdle weakness (affects lower limbs first)

hyporeflexia
autonomic symptoms: dry mouth, impotence, difficulty micturating

ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)

Question 30

what would an EMG show in Lambert-Eaton Syndrome?

Answer 30

incremental response to repetitive electrical stimulation

Question 31

What is the management of Lambert-Eaton Syndrome?

Answer 31

treatment of underlying cancer
immunosuppression, for example with prednisolone and/or azathioprine
3,4-diaminopyridine is currently being trialled
works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate
intravenous immunoglobulin therapy and plasma exchange may be beneficial