Neurology

Question 1

Agenesis of cerebellar vermis

Cystic dilatation of the 4th ventricle

Enlargement of the posterior fossa

Answer 1

Dandy-Walker Malformation

Question 2

Short webbed neck

Decreased ROM of the cervical vertebrae

Low posterior hairline accompanied by hydrocephalus

Answer 2

Klippel-Feil Syndrome

Question 3

Obstruction within the ventricular system

Abnormality of the aqueduct or a lesion in the 4th ventricle

Answer 3

Obstructive or Noncommunicating Hydrocephalus

Question 4

Obliteration if the subarachnoid cisterns; malfunction of arachnoid villi

Follows a subarachnoid hemorrhage; leukemic infiltrates

Answer 4

Nonobstructive or communicating Hydrocephalus

Question 5

Single nocturnal seizure with clonic movement of the mouth and gurgling

Usually begins 5-10y/o; resolves by 16y/o

Answer 5

Benign Rolandic Epilepsy

Question 6

Prior history of febrile seizures or head trauma

Prodrome: lethargy

Oral or motor automatisms, altered consciousness, head and eye deviation, contralateral twitching or tonic-clonic movements, posturing

Answer 6

Temporal lobe epilepsy

Question 7

Sudden cessation of motor activity or speech with a blank facial expression and flickering of the eyelids

Uncommon before 5 y/o
NEVER associated with an aura
NOT associated with postictal state
DO NOT lose body tone

Answer 7

Absence Seizure

Question 8

Associated with an aura

Sudden loss of consciousness -> cyanotic -> apneic

Loss of sphincter control

With postictal state: vomiting confusion, somnolence, intense headache

Answer 8

Generalized tonic clonic seizure

Question 9

Repetitive seizures consisting of brief often symmetric myoclonic contractions with loss of body tone and falling or slumping forward

Answer 9

Myoclonic Seizure

Question 10

Begin at 4-8mos

Brief symmetric contractions of the neck, trunk, extremities

EEG: hypsarrhythmia

Answer 10

Infantile Spasms

Question 11

DOC for infantile spasm

Answer 11

Vigabatrin

Question 12

DOC for absence seizure

Answer 12

Ethosuximide and Valproic Acid

Question 13

Management for GTC

Answer 13

Phenobarbital
Phenytoin
Carbamazepine
Lamotrigine
Valproic acid
Gabapentin

Question 14

Most common seizure disorder in childhood

Normal EEG, normal neurologic exam
(+) family history

Answer 14

Febrile seizure

Question 15

Lasts a few seconds to 15 minutes
Initially generalized and tonic clonic followed by a brief period of post-ictal drowsiness
Occurs only once in 24 hours

Answer 15

Simple febrile seizure

Question 16

Duration is more than 15minutes
Focal seizure activity
Repeated convulsions occur within 24hours

Answer 16

Complex febrile seizure

Question 17

Recurrent headache with symptom-free intervals and atleast three of the following:

(+) family history
Associated with aura
Relief after sleep
Abdominal pain
Unilateral 
Nausea and vomiting
Throbbing in character

Answer 17

Migraine

Question 18

Headache most apparent during school day
Common after puberty
Waxes and wanes
Band-like tightness or pressure
Most are in the frontal region

Answer 18

Tension headache

Question 19

Cafe au lait macules that spare the face; axillary or inguinal freckling; Lisch nodules; optic glioma

Answer 19

Neurofibromatosis (Von-Reckling-hausen)

Question 20

Multisystemic; seizures; mental retardation

Tubers in the periventricular area (candle dripping appearance); ash leaf, shagreen patch

Answer 20

Tuberous Sclerosis

Question 21

Most common etiologic agent in bacterial Meningitis

Answer 21

1st 2 mos: Group B Streptococcus

2mos - 12 years: S. pneumoniae, H. influenza, N. Meningitidis

Question 22

CSF analysis in Bacterial Meningitis

Answer 22

Pleocytosis, high CSF protein level, low CSF sugar

Question 23

Treatment for Bacterial meningitis

Answer 23

N. meningitides: Penicillin IV

S. pneumoniae: 3rd gen Cephalosporin or Penicillin IV

L. monocytogenes or Hib: Ampicillin

Question 24

Most likely organism to cause viral meningitis

Answer 24

Echovirus

Question 25

CSF analysis in Viral Meningitis

Answer 25

Normal glucose, normal to slightly increased protein, lymphocytosis

Question 26

Most common location of brain abscess

Answer 26

Cerebrum (80%)

Question 27

Indication for surgery in brain abscess

Answer 27

``` (+) gas in the abscess Multiloculated abscesses Posterior fossa location Fungal cause Assoc. infections like mastoiditis, periorbital abscess, sinusitis ```

Question 28

Earliest and most constant signs in Myasthenia Gravis

Answer 28

Ptosis | Some degree of extraocular muscle weakness

Question 29

Due to immune mediated neuromuscular blockade Decreased availability of Ach receptors due to circulating receptor binding Abs Rapid fatigue of muscles (proximal muscle)

Answer 29

Myasthenia gravis

Question 30

Acute unilateral facial nerve palsy that is not assoc with other cranial neuropathies or brainstem dysfunction Usually develops abruptly 2 weeks after a systemic viral infection

Answer 30

Bell's palsy

Question 31

Autoimmune reaction that develops in response to a previous infection leading to aberrant demyelination of peripheral nerves and ventral motor nerve roots Campylobacter jejuni and herpesvirus infection

Answer 31

Guillain barre Syndrome

Question 32

Weakness begins in the lower extremities and progressively involves the trunk, upper limbs, bulbar muscles (Landry ascending paralysis) Tendon reflexes are the last function to recover and lower extremity weakness is last to resolve

Answer 32

Guillain barre Syndrome

Question 33

Diagnostic of Guillain barre Syndrome

Answer 33

Dissociation between high CSF protein and a lack of cellular response (albuminocytologic dissociation)

Question 34

Most common solid tumors 2nd most cost prevalent malignancy in childhood

Answer 34

Brain tumors

Question 35

2 distinct patterns of presentation

Answer 35

Increased ICP and focal neurologic signs

Question 36

Most common infratentorial tumor With best prognosis

Answer 36

Cerebellar astrocytoma

Question 37

Most often found in cerebellum; heterogenous enhancements often invading the 4th ventricle and can cause obstructive hydrocephalus M>F; 4-8y/o Homer-wright rosettes - circular patterns of tumor cells surrounding a center of neutrophils

Answer 37

Medulloblastoma

Question 38

Solid and cystic areas that tend to calcify; short stature; pressure to optic chiasm producing bitemporal visual defects

Answer 38

Craniopharyngioma