Neurology

Question 1

Neurofibromatosis 1

Answer 1

Café-au-lait spots (>= 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamatomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas

Question 2

Neurofibromatosis 2

Answer 2

Bilateral vestibular schwannomas

Multiple intracranial schwannomas, mengiomas and ependymomas

Question 3

Autonomic dysreflexia

Answer 3

Medical emergency in spinal cord injury

can only occur if the spinal cord injury occurs above the T6 level

severe hypertension, flushing and sweating without a congruent response in heart rate

Management of autonomic dysreflexia involves removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.

Question 4

Essential tremor

Answer 4

Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs

Features
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)

Management
propranolol is first-line
primidone is sometimes used

Question 5

Anterior spinal artery occlusion

Answer 5

bilateral spastic paresis and loss of pain and temperature sensation

Question 6

Friedrich’s ataxia

Answer 6

bilateral spastic paresis, loss of proprioception and vibration sensation and an intention tremor

Question 7

Middle cerebral artery stroke

Answer 7

contralateral hemiparesis and sensory loss with the upper extremity being more affected than the lower, contralateral homonymous hemianopia and aphasia

Question 8

Anterior cerebral artery stroke

Answer 8

contralateral hemiparesis and sensory loss with the lower extremity being more affected than the upper

Question 9

Sarcoidosis

Answer 9

bilateral parotid gland enlargement

bilateral facial nerve palsy