Exam 3

Question 1

Cause of myasthenia gravis

Answer 1

Auto-Ab against the nicotinic acetylcholine receptor

Question 2

Associations of myasthenia gravis

Answer 2

Thymoma or thymic hyperplasia

Question 3

Distinguishing features of the muscle weakness in myasthenia gravis (4)

Answer 3

Fatiguable and mainly proximal

Question 4

Treatment of myasthenia gravis (3)

Answer 4

Pyridostigmine- acetylcholinesterase inhibitor
Thymectomy
Prednisolone

Question 5

Cause of Lambert-Eaton myasthenic syndrome?

Answer 5

Antibodies to pre-synaptic voltage gated calcium channels

Question 6

Lambert-Eaton is associated with what disease?

Answer 6

Small cell lung cancer

Question 7

Management of myasthenic crisis

Answer 7

IVIG

Question 8

Feature of LEMS which may distinguish it from MG?

Answer 8

Signs of autonomic dysfunction

Question 9

Commonest form of motor neurone disease and associated symptoms

Answer 9

Amytrophic lateral sclerosis. Mix of UMN and LMN signs

Question 10

Subtypes of MND:

a) dyarthria, dysphagia, wasted fasciculated tongue
b) UMN signs exclusively
c) LMN signs exclusively, best prognosis

Answer 10

a) progressive bulbar palsy
b) primary lateral sclerosis
c) progressive musclar atrophy

Question 11

Motor neurone disease never affects…(4)

Answer 11

sensation
sphincters
cerebellum
eye muscles

Question 12

Impairment of cognitive function seen in 10-35% MND patients?

Answer 12

Frontotemporal dementia

Question 13

Drug which has a modest impact on survival in MND

Answer 13

Riluzole- antilgutamergic

Question 14

Gene implicated in 20% of familial cases?

Answer 14

Superoxide dismutase 1 (SOD1)

Question 15

Upper motor neurone signs (2)

Answer 15

Increased tone

Hyper-reflexia

Question 16

Lower motor neurone signs (4)

Answer 16

Decreased tone
Fasciculation
Wasting
Hyporeflexia

Question 17

How can spinal stenosis be differentiated from claudication?

Answer 17

Spinal stenosis tends to get better when walking uphill/cycling, since this causes back flexion

Question 18

Arreflexic flaccid paralysis in reponse to acute cord transection

Answer 18

Spinal shock

Question 19

Brown-Sequard syndrome symptoms?

Answer 19

Ipsilateral dorsal column and corticospinal tract level

Contralateral spinothalamic tract level

Question 20

Patient presentation- “cape-like” pain/temperature loss, with distal upper limb weakness

Answer 20

Central cord syndrome

Question 21

What usually causes central cord syndrome?

Answer 21

Hyper-extension or hyper-flexion to an already stenosed neck

Question 22

Cause of syringomyelia

Answer 22

Expansion of the central cord canal due to CSF blockage

Question 23

Symptoms of syringomyelia

Answer 23

Cape-like spinothalamic loss
LMN signs in upper limbs
UMN signs in lower limbs

Question 24

Malformation commonly associated with syringomyelia

Answer 24

Chiari malformation- extension of the cerebellar tonsils into the spinal cord

Question 25

Back pain red flags (5)

Answer 25

Cauda equina symptoms (urinary retention/incontinence, faecal incontinence, saddle anaesthesia)
Nocturnal pain
Fever/chills
Unexplained weight loss
Past history of cancer