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Compiled PPS board reviewer > 5 CNS > Flashcards

5 CNS Flashcards

1
Q

Most severe form of dysraphism involving vertebral column and SC

A

Myelomeningocele

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2
Q

Poorest prognosis of encephalocele

A

Neural tissue in sac w/hydrocephalus

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3
Q 
Occipital encephalocoele
Cleft lip/palate
Microcephaly
Microphthalmia
Abnormal genitalia
Polycystic kidneys
Polydactyly
A

Meckel Gruber syndrome

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4
Q

Triad of Lennox Gastaut Syndrome

A

Intractable seizure
Slow spike wave EEG
MR

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5
Q

Mc symptom of simple partial seizure

A

Asynchronous chronic or tonic movt

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6
Q

MC cx of death of Von-Hippel Lindau dse

A

Renal carcinoma

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7
Q

MC of neonatal meningitis

A

GBS and E.coli

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8
Q

AOG when screening of maternal serum AFP to diagnose NTD

A

16-18wks AOG

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9
Q

prevention for NTD

A
  1. 4mg folic acid daily

0. 4mg folic acid 1 month prior up to 12wks AOG if w/ previous pregnancy of NTD

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10
Q

midline defect of the vertebral bodies w/o protrusion of spinal cord meninges

A

Spina Bifida Occulta

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11
Q

meninges herniate through a defect in the posterior vertebral arch

A

meningocoele

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12
Q

due to downward herniation of the medulla and cerebellar tonsills

A

chiari crisis

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13
Q

medications that increase the risk of myelomeningocele

A 
TMP
Carbamazepine
phenytoin
phenobarbital
primidone (antagonizes folic acid)
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14
Q

increase risk for NTD

A

Valproic acid

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15
Q

contains sac plus cerebral cortex, cerebellum or portion of the brainstem

A

enecphaloecele

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16
Q

major form of dysraphism in skull

A

cranium bifidum

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17
Q

large defect of the calvarium, meninges & scalp

A

anencephaly

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18
Q

absence of cerebral convolutions and poorly formed sylvian fissure from faulty neuroblast migration

A

Lissencephaly (agyria)

associated w/ Miller Dierker syndrome

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19
Q

unilateral o bilateral clefts w/in the cerebral hemisphere d/t abnormality of morphogenesis

A

Schizencephaly

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20
Q

cysts or cavities w/in the brain from development defects/ acquired lesion including infarction of tissue

A

porencephaly

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21
Q

defective cleavage of prosencephalon and inadequate induction of forebrain structures

A

holoprosencephaly

assoc w/ maternal diabetes, sonic hedgehog, cyclopia, cebocephaly & premaxillary agenesis

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22
Q

widely separated frontal horns w/ an abnormally high position of the 3rd ventricle

A

agenesis of the corpus callosum

aicardi syndrome

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23
Q

sucking jaw movement w/ eyelid blinking

A

Marcus Gunn phenomenon

CN 3 and 7

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24
Q

absence of the 7th CN - bilateral facial weakness, abducens nerve paralysis, feeding difficulties, immobile or dull facies

A

mobius syndrome

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25
Q

HC >3SD below mean for age/sex/race

A

microcephaly

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26
Q

non genetic causes of microcephaly

A 
radiation
cmv
rubella
toxoplasmosis
fetal alcohol syndrome
phenylalanine
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27
Q

impaired circulation and absorption of CSF

A

hydrocephalus

- inc CSF production by cricoid plexus papilloma

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28
Q

CSF Flow

A

Choroid plexus –> Lateral ventricle thru FORAMEN OF MONROE –>into 3rd ventricle -> AQUEDUCT OF SYLVIUS –> 4th ventricle –> FORAMEN OF LUSCHKA & MAGENDIE –> Subarachnoid space where it is absorbed in the Arachnoid granulations

Come Let Me Treat Sylvia For Lunch Maybe Somewhere in Ayala

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29
Q

MC malformation of posterior fossa & hindbrain

A

Chiari malformation

type I - not assoc w/ hydrocephalus
type II - progressive hydrocephalus w/ myelomeningocele

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30
Q

part of the continum of posterior fossa anomalies that include cystic dilatation of the 4th ventricle, hypoplasia of cerebellar vermis, hydrocephalus, & an enlarged posterior fossa w/ elevation of the lateral venous sinus & tentorium

A

Dandy Walker Malformation

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31
Q

MC syndrome w/ 50% of patients having prenatal macrocephaly & 100% of patient having macrocephaly by 1yo

A

Sotos Syndrome

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32
Q

premature closure of cranial suture

A

craniosynostosis

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33
Q

MC premature closure of sagittal suture

A

Scaphocephaly

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34
Q

premature closure of coronal and sphenofrontal suture

A

frontal plagiocephaly

35
Q

bilateral closure of coronal suture, underdeveloped orbits, ocular proptosis, maxillary hypoplasia and orbital hypertelorism

A

Crouzon Syndrome

36
Q

premature fusion of multiple suture

A

Alpert Syndrome

37
Q

Cloverleaf skull

A

kleeblattschadel deformity; carpenter syndrome

38
Q

asymmetric craniosynostosis & plagiocephaly

A

Chotzen syndrome

39
Q

Etiology of Communicating or Nonobstructive hydrocephalus

A 
achondroplasia
basilar impression
benign enlargement of subarachanoid space
choroid plexus papilloma
meningeal malignancy
meningitis
posthemorrhagic
40
Q

Etiology of noncommunicating or obstructive hydrocephalus

A 
aqueductal stenosis
mitochondrial
AR or dominant L1CAM mutation
chiari malformation
Dandy walker malformation
Klippel Feil syndrome
mass lesion
41
Q

triad of Dandy Walker Malformation

A

agenesis of cerebellar vermis
cystic dilatation of the 4th ventricle
enlargement of the posterior fossa

42
Q

triad of Klippel Feil syndrome

A

short webbed neck
decreased ROM of cervical vertebrae
low posterior hairline accompanied by hydrocephalus

43
Q

Contraindications in doing LP

A 
increased ICP
pending herniation
critical illness
skin infection at site of puncture
thrombocytopenia
44
Q

> 2 unprovoked seizures occurring at the same time frame of >24hrs

A

epilepsy

45
Q

single seizure lasting >15mins or a series of seizure w/o return to baseline mental status between each episode

A

status epilepticus

46
Q

single nocturnal sz w/ clonic movement of the mouth

A

Benign Rolandic Epilepsy

  • resolves by 16 yo
  • tx: Oxacarbazepine, carbamazepine, levetiracetam, valproic acid
47
Q

prodrome of Temporal lobe epilepsy

A

lethargy

48
Q

sudden cessation of motor activity or speech w/ blank facial expression and flickering of eyelids

A

Absence seizure

tx: ethosuximide/ valproic acid

49
Q

MC generalized motor seizure

A

Generalized Tonic-Clonic

50
Q

repetitive seizure consisting of brief, often symmetric muscular contraction w/ loss of body tone & falling or slumping forward

A

myoclonic epilepsy

51
Q

confined to the neck, truck & extremities
normal EEG
resolve by 2yo w/o treatment

A

Benign myoclonus of infancy

52
Q

Triad of Lennox Gastaut Syndrome

A

intractable seizure
slow spike EEG waves
mental retardation

53
Q

myoclonic jerks on awakening

12-16 y/o

A

juvenile myoclonic epilepsy/ Janz epilepsy

54
Q

GTC sz, myoclonic jerks, MR
10-18 yo
polyspike wave in EEG

A

Lafora Disease
progressive myoclonic epilepsies
tx: clonazepam, valproic acid

55
Q

begin at 4-8months
brief symmetric contraction of neck, trunk, extremities
EEG: hypsarrhthmia

A

infantile spasms (west syndrome)

56
Q

tx of focal sz

A

oxcarbazepine

carbamazepine

57
Q

tx of absence sz

A

Ethosuximide - as effective as valproic acid but less toxic

valproic acid & lamotrigine (less effective)

58
Q

tx for juvenile myoclonic epilepsy (12-18yo)

A

levetiracetam
topiramate
valproic acid

59
Q

tx for infantile spasm

A

ACTH
steroids
vigabatrin

60
Q

tx for epilepsy w/ GTC sz only

A

levetiracetam
lamotrigine
topiramate
valproic acid

61
Q

Age of febrile seizure

A

6-60mos

62
Q

duration of SFS

A

<15 mins

63
Q

duration of CFS

A

> 15 mins

64
Q

initially GTC then a brief period of post ictal drowsiness

A

SFS

65
Q

indication of LP

A

-<6mos w/fever & seizure
- ill appearing
- any age w/ clinical s/sx of concern
-<18 mos if w/1st episode of febrile sz
- option for 6-12 mos:
if SFS is deficient in Hib and streptococcal immunization (unknown immunization status)
if pretreated w/ antibiotics

66
Q

Major risk factor for recurrence of febrile sz

A

<1yo
<24hrs fever
38-39C

67
Q

minor RF for recurrence of febrile sz

A 
family hx
CFS
daycare
male gender
hypothermia at presentation
68
Q

gene mutation on chromosome 17q11.2

encodes for neurofibromin w/c inhibits Ras oncogene

A

Von Recklinghausen dse
NF1
- progressive dse that can affect almost every organ
- MC skin lesions are cafe-au-lait spots

69
Q

criteria for NF1

A

any 2 of the ff are present:

  • > 6 cafe au lait macules >5mm in diameter in prepubertal & >15mm in postpubertal individuals
  • axillary or inguinal freckling with multiple hyperpigmented areas 2-3mm in diameter
  • > 2 iris Lisch nodule (hamartomas located in the iris)
  • > 2 neurofibromas or one plexiform neurofibroma
  • distinctive osseous lesion
  • optic glioma
  • 1st degree relative with NF1 whose diagnosis was based on aforementioned criteria
70
Q

criteria for NF2

A

1 of the ff are present

  • bilateral 8th nerve masses (acoustic neuroma)
  • parent, sibling, child w/ NF2 & either unilateral 8th nerve masses or any of the ff: neurofibroma, meningioma, glioma, schwannoma
71
Q

4 main manifestation of tuberous sclerosis

A

MR
seizure
cutaneous lesions
tumor in various organs

72
Q

Diagnostic Criteria for TS

A 
2M or 1M2m
MAJOR:
-skin lesion
- brain lesion
- eye lesion
- tumor of other organs (cardiac rhabdomyoma, renal angiomyolipoma, pulmonary lymphangioleimyomatosis)

Minor:

  • cerebral white matter migration lines
  • gingival fibromas
  • bone cyst
  • retinal achromatic patch
  • confetti skin lesion
  • nonrenal hamartomas
  • multiple renal cysts
  • hamartomatous rectal polyps
73
Q

Ash lead on trunk & extremities

Shagreen patch on lumbosacral region

A

tuberous sclerosis

74
Q

serpentine or railroad track appearance

A 
Sturge Weber Syndrome
- port wine stain
seizure
hemiparesis
stroke like episode
intracranial calcification
MR
brain is atrophic and calcified
75
Q

MC cause of death of Von Hippel Lindau Dse

A

renal carcinoma

76
Q

PHACE syndrome

A 
Posterior fossa malformation
Hemangioma
Arterial abnormalities
Coarctation of the aorta
Eye abnormalities

tx: INFa

77
Q 
unilateral weakness with UMN signs
sensory abnormalities
visual complain
ataxia
paresthesia of LE & face
A

multiple sclerosis

tx: IV methylprednisolone

78
Q

immune mediated demyelinating disorder can follow immunization or infections (MMRV, herpes zoster, URTI)

A

Acute Disseminated Encephalomyelitis (ADEM)
- fever, lethargy, weakness, ataxia, sz –> delirium, coma, myelopathy & focal neurologic signs

tx: IV methylprednisolone, IVIG

79
Q

MC cause of neonatal meningitis

A

GBS

E.coli

80
Q

etiologic agent of bacterial meningitis in 1st 2 months of life

A

Grp B streptococcus
Gram negative enteric bacilli
L. monocytogenes

81
Q

etiologic agent of bacterial meningitis in 2-12 yo

A

S. pneumoniae
H. influenza
N. meningitidis

82
Q

MC cause of viral meningoencephalitis

A

enterovirus

83
Q

MC manifestation of viral meningoencephalitis

A

cerebellar ataxia

84
Q

MC cause of brain abscess in neonates

A

citrobacter

Compiled PPS board reviewer (60 decks)

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