Neurology

Question 1

account for the largest proportion of congenital anomalies of the CNS

Answer 1

Neural tube defect
- result from failure of neural tube to close spontaneously between the 3rd and 4th wk of in utero development

Question 2

What is an atypical dimple that warrants imaging?

Answer 2

deep, > 5 mm , >25 mm from anal verge

Question 3

Which part of the brain is CSF formed?

Answer 3

ventricular system by the choroid plexus

Question 4

Hydrocephalus resulting from obliteration of the subarachnoid cisterns or malfunction of the arachnoid villi

Answer 4

non-obstructive/communicating hydrocephalus
— most commonly follows a subarachoid hemorrhage

Question 5

hydrocephalus resulting from obstruction within the ventricular system

Answer 5

obstructive or noncommunicating
— most commonly associated with an abnormality of the aqueduct of Sylvius or lesion in the 4th ventricle

Question 6

most prominent sign in infants with hydrocephalus

Answer 6

accelerated rate of enlargement of the head

Question 7

most important imaging modality in identifying the specific cause and severity of hydrocephalus

Answer 7

CT scan and/or MRI + Cranial UTZ

Question 8

Most common megalencephalic syndrome

Answer 8

Sotos Syndrome (Cerebral gigantism)

Question 9

Type of NTD: cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal or occipital cortex dispersed over the membrane

Answer 9

Hydranencephaly

Question 10

major complications of shunting

Answer 10

occlusion
bacterial infection

Question 11

represents the most severe form of dysraphism

Answer 11

Myelomeningocele

Question 12

Folic acid supplementation is proven to reduce the incidence of NTDs in pregnancy at risk by at least 50%, thereby, it should be taken when?

Answer 12

before conception and continued until at least 12th wk of gestation

Question 13

what dose of folic acid should be taken during pregnancy

Answer 13

0.4 mg OD
but if high risk pregnancy, 4 mg OD

Question 14

most frequent type of recurrent headache

Answer 14

Migraine

Question 15

definition of chronic migraine

Answer 15

if it occurs more than 15 days per month

Question 16

most common form of migraine in both children and adults

Answer 16

Migraine without aura

Question 17

Define status migrainosus

Answer 17

migraine that persists beyond 72 hours

Question 18

Major risk factor for bacterial meningitis

Answer 18

lack of preexisting immunity to specific pathogens and serotypes

Question 19

Most important step/procedure in the diagnosis of Meningitis

Answer 19

Lumbar puncture

Question 20

contraindications of lumbar puncture

Answer 20

• evidence of increased ICP
• severe cardiopulmonary compromise (eg shock)
• infection of the skin overlying the site of LP

Question 21

initial emperic treatment for children suspected with meningitis

Answer 21

3rd generation cephalosporin (usually Ceftriaxone) + Vancomycin

Question 22

enumerate the most common neurologic sequelae from meningitis

Answer 22

hearing loss (sensorineural hearing loss - most common)
cognitive impairment
recurrent seizures
delay in acquisition of language
visual impairment
behavioral problems

Question 23

Chemoprophylaxis given for close contacts with meningococcal meningitis

Answer 23

Rifampicin (10 mkdose) q12 for 2 days or
Ceftriaxone 125 mg IM x 1 for <15 yr old
Ceftriaxone 250 mg IM x 1 for >15 yr old
or
Ciprofloxacin 500 mg PO x 1

Question 24

Prophylaxis given to all household contacts of patients with invasive disease caused by H. influenza type B

Answer 24

Rifampin (20 mkday) OD for 4 days

Question 25

Predominant organisms that cause brain abscesses

Answer 25

Streptococci sp.

Question 26

risk of developing epilepsy who have first afebrile seizure

Answer 26

30% if normal neuro PE and other tests, 20%

Question 27

this test should be performed in all cases of a first unprovoked nonfebrile seizure to help predict the risk of seizure recurrence

Answer 27

routine EEG

Question 28

febrile seizures usually occur during this age group

Answer 28

6 and 60 months — peak 12-18 months

Question 29

difference between simple febrile seizure and complex febrile seizure

Answer 29

SFS: generalized, lasting for 15 min (max), not recurrent within a 24 hr period CFS: prolonged (>15 min), focal, recurs within 24 hours

Question 30

duration of Febrile status epilepticus

Answer 30

30 minutes

Question 31

percentage of neurologically healthy infants who experience at least 1 simple febrile seizure

Answer 31

between 2% and 5%

Question 32

major risk factors for recurrence of febrile seizures

Answer 32

Age < 1 yr Duration of fever <24 hr Fever 38-39 C

Question 33

recurrence risk of children with febrile sezure who have no risk factors

Answer 33

12%

Question 34

mutation in this gene is the most common cause of Dravet Syndrome

Answer 34

SCN1A gene

Question 35

this micronutrient deficiency is associated with an increased risk of febrile seizures

Answer 35

Iron deficiency

Question 36

most common hereditary neuromuscular disease affecting all races and ethnic groups

Answer 36

Duchenne muscular dystrophy

Question 37

clinical features of Duchenne Muscular Dystrophy

Answer 37

Progressive weakness Intellectual impairment Hypertrophy of the calves, with proliferation of connective tissue and progressive fibrosis in muscle

Question 38

what test is greatly elevated in DMD

Answer 38

Serum CK level — usually at 15K to 35K (normal <160)

Question 39

what is the type of muscle affected in autoimmune myasthenia gravis?

Answer 39

striated muscles — particularly extraocular, palpebral muscles and swallowing

Question 40

characteristic feature of MG that distinguishes it from most other neuromuscular diseases

Answer 40

Rapid fatigue of muscles

Question 41

Primary therapeutic agent for MG

Answer 41

Cholinesterase-inhibiting drugs such as Pyridostigmine bromide (0.5-1 mg/kg/dose every 4-6, max dose 7 mkday)

Question 42

Medications that can potentiate myasthenia

Answer 42

Aminoglycosides Beta blocking agents Procainamide Chloroquine Fluoroquinolones

Question 43

autoimmune disorder thought to be a postinfectious polyneuropathy, involving mainly motor nerves

Answer 43

Guillan-Barré Syndrome

Question 44

initial symptoms of Guillan-Barre Syndrome

Answer 44

weakness usually begins in the LE, progressively involves the trunk, upper limbs and finally bulbar muscles

Question 45

diagnostic of GBS based on CSF studies

Answer 45

High CSF protein and a lack of cellular response (cytoalbuminologic dissociation)

Question 46

Treatment for severe or rapidly progressive muscle weakness in GBS

Answer 46

IVIG (0.4 g/kg/day) for 5 consecutive doses or (1g/kg/day) for 2 days

Question 47

last function in GBS to recover

Answer 47

Tendon reflexes

Question 48

function to recover first in GBS

Answer 48

bulbar function

Question 49

most common long-term residuum of GBS

Answer 49

Fatigue

Question 50

features needed for diagnosis of GBS

Answer 50

-progressive weakness in legs and arms - areflexia in weak limbs

Question 51

acute-onset peripheral facial nerve palsy not associated with any other cranial nerve neuropathies or brainstem dysfunction

Answer 51

Bell palsy

Question 52

most common causes of Bell palsy

Answer 52

Active/reactivation of herpes simplex or Varicella-zoster virus

Question 53

primitive reflex which remains throughout life

Answer 53

Parachute reflex

Question 54

Drugs that antagonize Folic acid hence these should not be given ideally to pregnant women

Answer 54

Trimethoprim Anticonvulsants such as Carbamazepine, Phenytoin, Phenobarbital and Primidone

Question 55

uncommon GBS variant associated with acute external ophthalmoplegia, ataxia and areflexia

Answer 55

Miller-Fisher Syndrome (MFS)

Question 56

Most often involved nerve in MFS

Answer 56

6th cranial nerve

Question 57

How to diagnose migraine without aura

Answer 57

A. At least 5 attacks fulfilling criteria B to D B. HA attacks lasting 4-72 hr (untreated or unsuccessfully treated) C. HA has at least 2 of the ff 4 characteristics: 1. Unilateral location 2. Pulsating quality 3. Moderate to severe pain intensity 4. Aggravation by or causing avoidance of routine physical activity (walking/climbing stairs) D. During headache at least 1 of the ff: > Nausea and/or vomiting > Photophobia and phonophobia E. Not better accounted for by another ICHD-3 diagnosis

Question 58

Triggers for migraine

Answer 58

Skipping meals Inadequate or irregular sleep Dehydration Weather changes

Question 59

Potential cause of headache with papilledema in children with normal standard findings in brain MRI

Answer 59

Idiopathic intracranial hypertension// Pseudotumor cerebri

Question 60

percentage of children who experience at least 1 seizure (febrile/afebrile) in the first 16 years of life

Answer 60

4-10%

Question 61

4 obligatory criteria in distinguishing Muscular dystrophies from all other neuromuscular disease

Answer 61

1. It is a *primary myopathy* 2. It has a *genetic basis* 3. Course is *progressive* 4. *Degeneration* and *death of muscle fibers* occur at some stage in the disease

Question 62

GBS may follow administration of vaccine against?

Answer 62

Rabies Influenza Conjugated Meningococcal

Question 63

features needed for diagnosis of GBS

Answer 63

Progressive weakness in legs and arms Areflexia in weak limbs

Question 64

most common long-term residuum of GBS

Answer 64

Fatigue