Neurology

Question 1

Triad of:

1. Epilepsy in infant, sudden flexion in tonic fashion
2. hypsarrhythmia on EEG
3. mental retardation

Answer 1

West Syndrome

infantile spasms also known as Salaam spasm

Question 2

Hemifacial contortions, inability speak, abnormal feelings in tongue

Answer 2

Benign rolandic epilepsy (Centrotemporal spikes)

Question 3

Episodic visual distortions/ hallucinations, migraine like headaches

Answer 3

Benign occipital epilepsy

Question 4

Drop attacks + mental retardation

Age 1-3

Answer 4

Lennox Gastaut syndrome

Question 5

Intractable seizures (severe myoclonic epilepsy in infancy)

Answer 5

Dravet Syndrome

Question 6

3Hz spike wave
Brought on y hyperventilation
Small Motor Sx (lip smacking, eyelid flickering)

Answer 6

Absence seizure

Question 7

seizures after waking

Answer 7

Juvenile myoclonic epilepsy

Question 8

Port wine stain

Answer 8

Sturge Weber Syndrome

Question 9

Cafe au last spots + Lisch nodules

Answer 9

Neurofibromatosis Type 1

Lisch nodules = gold spots in iris

Question 10

Bilateral vestibular schwannoma

Answer 10

Neurofibromatosis Type 2

Question 11

Ash leaf patch + Shagreen patches + angiofibroma + seizure

Answer 11

Tuberous sclerosis complex

Question 12

Telangiectasia + increased susceptibility to infection + ataxia

Answer 12

Ataxia telangiectasia

Question 13

Champagne bottle legs + onion bulb appearance on nerve biopsy

Answer 13

Charcot Marie syndrome

Question 14

Worsening ataxia + absent lower limb reflexes + extensor plantar response arises in 2nd decade of life

Answer 14

Friedrich’s ataxia

Question 15

Clasp knife muscle tone

Answer 15

Spastic cerebral palsy

Question 16

What is cerebral palsy?

Answer 16

Persistent unchanging disorder to movement and posture due to a defect/lesion in developing brain
Non progressive + motor / posture problems + insult to developing brain (<5)

Question 17

Types of Cerebral Palsy (3)

Answer 17

1. Spastic - 70% - damage to motor cortex or corticospinal tracts. Triad: limb tone +++, brisk reflexes + extensor plantar responses
2. Diskinetic - damage to basal ganglia resulting in involuntary movements in whole body. Triad: dystonia, athetosis, chorea
3. Ataxic - damage to cerebellum. Fine tremor worse on movement initiation