Neurology 2

Question 0

Seizures – when not to do a further workup?

Answer 0

First time afebrile seizure in otherwise healthy child with normal neuro exam

Question 1

Partial seizures?

Simple partial versus complex partial seizures?

Answer 1

one hemisphere affected

Conscious is not impaired versus decreased

Question 2

Differential diagnosis for unsteady gait?

Answer 2

1. Cerebellar dysfunction
2. Weakness – muscular, spinal cord lesions, Guillain-Barre
3. Encephalopathy
4. Seizures/Postictal.
5. Vertigo

Question 3

Most common cause of ataxia in children? Age of onset? Causes? pathophysiology? Clinical features? Diagnosis? Treatment?

Answer 3

Acute cerebellar ataxia of childhood; under 10 years old

Varicella, influenza, EBV, Mycoplasma

Immune complex deposition in the cerebellum

1. Truncal ataxia with deterioration of gait
2. Slurred speech/nystagmus
3. No fever
4. post viral illness

Exclusion. Head CT to r/o acute life-threatening causes (tumors/hemorrhage)

Supportive

Question 4

Guillain-Barre syndrome – associated infectious agent? Pathophysiology? Clinical features? Absent feature? Diagnosis? Treatment?

Answer 4

Campylobacter

Cell-mediated immune response cross reacts with Schwann cell membrane leading to demyelination of ventral spinal roots and peripheral nerves

Ascending symmetric paralysis with facial weakness

No sensory loss

1. LP - albuminocytologic dissociation (Increased protein in absence of elevated cell count)
2. EMG – decreased nerve conduction velocity
3. Spinal MRI to rule out compressive lesions

IVIg or plasmapheresis

Question 5

Variant of Guillain-Barré? Characterized by?

Answer 5

Miller-Fisher syndrome

Opthalmoplegia, Ataxia areflexia

Question 6

Drug for generalized epilepsy? Absence seizures? Partial seizures?

Answer 6

Valproic acids; ethosuximide; carbamazepine/phenytoin

Question 7

Alternative treatments for epilepsy?

Answer 7

Surgery, Vegal nerve stimulator, ketogenic diet

Question 8

Sydenham Chorea – Seen with what disease? Pathophysiology? Presents when? Begins as? Clinical findings? Findings not present? Diagnosis? Treatment?

Answer 8

Rheumatic fever; antibodies cross react with basal ganglial cells; 2-7 months after Pharyngitis; clumsiness hands

1. Restlessness/abnormal movements
2. Indistinct speech and chameleon tongue (Unable to sustain protrusion)
3. Milkmaids grip - unable to maintain grip

No changes in gait or cognition

1. ASO/ABD titer
2. MRI – increased signal intensity in caudate/putamen
3. SPECT – increased perfusion to thalamus/striatum

Haloperidol, valproic acid, pentobarbital

Question 9

Most common form of migraine in children? Possible cause? Associated focal neurologic signs? Treatment?

Answer 9

Migraine without aura; changes in cerebral blood flow due to serotonin/substance P/VIP release

1. Ophthalmoplegic migraine – unilateral ptosis ptosis or CN3 palsy
2. Basilar artery migraine – vertigo, tinnitus, ataxia, dysarthria

Sumatriptan (5HT agonist) or propranolol (prophylactic)

Question 10

Myasthenia gratis – antibodies against? Classification in children? Features? Diagnosis?

Answer 10

ACh receptor

1. Neonatal – transient weakness due to transplacental transfer
2. Juvenile – antibody formation
3. Neonatal – hypotonia, weakness, eating problems
4. Juvenile – bilateral ptosis, increasing weakness, diplopia,
5. Acetylcholine receptor antibodies
6. Decremental response to repetitive nerve stimulation
7. Tensilon test – Improvement with edrophonium

Question 11

Treatment – myasthenia gravis?

Answer 11

1. Cholinesterase inhibitors – Pyridostigmine
2. Corticosteroids/IVIg if failure of pyridostigmine
3. Plasmapheresis
4. Thymectomy

Question 12

Most common partial epilepsy childhood? Definition? inheritance? Clinical features? EEG findings? Management? Prognosis?

Answer 12

Benign rolandic epilepsy; nocturnal partial seizures with secondary generalization

Autosomal dominant

1. Oral-buccal manifestations (moaning, pooling of saliva)
2. Sprint to face/arm and generalize

Spike and wave in temporal/central regions

Valproic acid or carbamazepine

Cease during adolescence

Question 13

Absence epilepsy of childhood – age of onset? Inheritance? Clinical features? Features that are NOT seen? EEG finding? Treatment? Prognosis?

Answer 13

5-9 years; autosomal dominant

Frequent, short (5-10 seconds) episodes accompanied by automatisms

No loss of posture, incontinence, postictal state

3 Hz spike and wave

Ethosuximide

Resolve by adolescence

Question 14

Infantile spasms – age of onset? Most common cause? Clinical features? EEG findings? Management? Prognosis?

Answer 14

3 to 8 months; tuberous sclerosis

Sudden arm extension and head/trunk flexion

Hypsarrhythmia - High amplitude spike and wave

ACTH, valproic acid, vigabatrin (if tuberous sclerosis)

Poor outlook – moderate to severe mental retardation

Question 15

Febrile seizures – simple versus complex? age range? Diagnosis? Unnecessary tests? Management if frequent?

Answer 15

<15 minutes and generalized versus over 15 minutes with focal features (or recurs)

Six months to six years

History, normal neurologic exam, exclusion of CNS infection

Imaging/EEG unless neurological exam is abnormal

Daily anticonvulsant prophylaxis (valproic acid)

Question 16

Treatment for Tourette’s syndrome?

Answer 16

1. Pimozide – drugs of choice
2. Clonidine
3. Hypnotherapy

Question 17

Secondary causes of intracranial headaches (and examples)?

Answer 17

1. Irritative – meningitis, subarachnoid hemorrhage

2. Increased ICP (tumor, hydrocephalus, subdural hematoma)

Question 18

Causes of Local extracranial headaches?

Answer 18

Eyes (refractive error)
Ears (otitis media)
Nose (sinusitis),
Mouth (toothache, abscess)

Question 20

Muscular dystrophy – protein affected? Biopsy would show? Clinical signs? Management?

Answer 20

Dystrophin

1. Infiltration of lymphocytes
2. Replacement of muscle fibers with fibroblasts and lipids
3. Pseudo-hypertrophy of calves
4. Gowers – to arise from floor
5. Cardiac – Cardiomegaly, heart failure

Steroids