5 CNS Flashcards
(84 cards)
1
Q
Most severe form of dysraphism involving vertebral column and SC
A
Myelomeningocele
2
Q
Poorest prognosis of encephalocele
A
Neural tissue in sac w/hydrocephalus
3
Q
Occipital encephalocoele Cleft lip/palate Microcephaly Microphthalmia Abnormal genitalia Polycystic kidneys Polydactyly
A
Meckel Gruber syndrome
4
Q
Triad of Lennox Gastaut Syndrome
A
Intractable seizure
Slow spike wave EEG
MR
5
Q
Mc symptom of simple partial seizure
A
Asynchronous chronic or tonic movt
6
Q
MC cx of death of Von-Hippel Lindau dse
A
Renal carcinoma
7
Q
MC of neonatal meningitis
A
GBS and E.coli
8
Q
AOG when screening of maternal serum AFP to diagnose NTD
A
16-18wks AOG
9
Q
prevention for NTD
A
- 4mg folic acid daily
0. 4mg folic acid 1 month prior up to 12wks AOG if w/ previous pregnancy of NTD
10
Q
midline defect of the vertebral bodies w/o protrusion of spinal cord meninges
A
Spina Bifida Occulta
11
Q
meninges herniate through a defect in the posterior vertebral arch
A
meningocoele
12
Q
due to downward herniation of the medulla and cerebellar tonsills
A
chiari crisis
13
Q
medications that increase the risk of myelomeningocele
A
TMP Carbamazepine phenytoin phenobarbital primidone (antagonizes folic acid)
14
Q
increase risk for NTD
A
Valproic acid
15
Q
contains sac plus cerebral cortex, cerebellum or portion of the brainstem
A
enecphaloecele
16
Q
major form of dysraphism in skull
A
cranium bifidum
17
Q
large defect of the calvarium, meninges & scalp
A
anencephaly
18
Q
absence of cerebral convolutions and poorly formed sylvian fissure from faulty neuroblast migration
A
Lissencephaly (agyria)
associated w/ Miller Dierker syndrome
19
Q
unilateral o bilateral clefts w/in the cerebral hemisphere d/t abnormality of morphogenesis
A
Schizencephaly
20
Q
cysts or cavities w/in the brain from development defects/ acquired lesion including infarction of tissue
A
porencephaly
21
Q
defective cleavage of prosencephalon and inadequate induction of forebrain structures
A
holoprosencephaly
assoc w/ maternal diabetes, sonic hedgehog, cyclopia, cebocephaly & premaxillary agenesis
22
Q
widely separated frontal horns w/ an abnormally high position of the 3rd ventricle
A
agenesis of the corpus callosum
aicardi syndrome
23
Q
sucking jaw movement w/ eyelid blinking
A
Marcus Gunn phenomenon
CN 3 and 7
24
Q
absence of the 7th CN - bilateral facial weakness, abducens nerve paralysis, feeding difficulties, immobile or dull facies
A
mobius syndrome
25
HC >3SD below mean for age/sex/race
microcephaly
26
non genetic causes of microcephaly
```
radiation
cmv
rubella
toxoplasmosis
fetal alcohol syndrome
phenylalanine
```
27
impaired circulation and absorption of CSF
hydrocephalus
| - inc CSF production by cricoid plexus papilloma
28
CSF Flow
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
29
MC malformation of posterior fossa & hindbrain
Chiari malformation
type I - not assoc w/ hydrocephalus
type II - progressive hydrocephalus w/ myelomeningocele
30
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
Dandy Walker Malformation
31
MC syndrome w/ 50% of patients having prenatal macrocephaly & 100% of patient having macrocephaly by 1yo
Sotos Syndrome
32
premature closure of cranial suture
craniosynostosis
33
MC premature closure of sagittal suture
Scaphocephaly
34
premature closure of coronal and sphenofrontal suture
frontal plagiocephaly
35
bilateral closure of coronal suture, underdeveloped orbits, ocular proptosis, maxillary hypoplasia and orbital hypertelorism
Crouzon Syndrome
36
premature fusion of multiple suture
Alpert Syndrome
37
Cloverleaf skull
kleeblattschadel deformity; carpenter syndrome
38
asymmetric craniosynostosis & plagiocephaly
Chotzen syndrome
39
Etiology of Communicating or Nonobstructive hydrocephalus
```
achondroplasia
basilar impression
benign enlargement of subarachanoid space
choroid plexus papilloma
meningeal malignancy
meningitis
posthemorrhagic
```
40
Etiology of noncommunicating or obstructive hydrocephalus
```
aqueductal stenosis
mitochondrial
AR or dominant L1CAM mutation
chiari malformation
Dandy walker malformation
Klippel Feil syndrome
mass lesion
```
41
triad of Dandy Walker Malformation
agenesis of cerebellar vermis
cystic dilatation of the 4th ventricle
enlargement of the posterior fossa
42
triad of Klippel Feil syndrome
short webbed neck
decreased ROM of cervical vertebrae
low posterior hairline accompanied by hydrocephalus
43
Contraindications in doing LP
```
increased ICP
pending herniation
critical illness
skin infection at site of puncture
thrombocytopenia
```
44
>2 unprovoked seizures occurring at the same time frame of >24hrs
epilepsy
45
single seizure lasting >15mins or a series of seizure w/o return to baseline mental status between each episode
status epilepticus
46
single nocturnal sz w/ clonic movement of the mouth
Benign Rolandic Epilepsy
- resolves by 16 yo
- tx: Oxacarbazepine, carbamazepine, levetiracetam, valproic acid
47
prodrome of Temporal lobe epilepsy
lethargy
48
sudden cessation of motor activity or speech w/ blank facial expression and flickering of eyelids
Absence seizure
tx: ethosuximide/ valproic acid
49
MC generalized motor seizure
Generalized Tonic-Clonic
50
repetitive seizure consisting of brief, often symmetric muscular contraction w/ loss of body tone & falling or slumping forward
myoclonic epilepsy
51
confined to the neck, truck & extremities
normal EEG
resolve by 2yo w/o treatment
Benign myoclonus of infancy
52
Triad of Lennox Gastaut Syndrome
intractable seizure
slow spike EEG waves
mental retardation
53
myoclonic jerks on awakening
| 12-16 y/o
juvenile myoclonic epilepsy/ Janz epilepsy
54
GTC sz, myoclonic jerks, MR
10-18 yo
polyspike wave in EEG
Lafora Disease
progressive myoclonic epilepsies
tx: clonazepam, valproic acid
55
begin at 4-8months
brief symmetric contraction of neck, trunk, extremities
EEG: hypsarrhthmia
infantile spasms (west syndrome)
56
tx of focal sz
oxcarbazepine
| carbamazepine
57
tx of absence sz
Ethosuximide - as effective as valproic acid but less toxic
| valproic acid & lamotrigine (less effective)
58
tx for juvenile myoclonic epilepsy (12-18yo)
levetiracetam
topiramate
valproic acid
59
tx for infantile spasm
ACTH
steroids
vigabatrin
60
tx for epilepsy w/ GTC sz only
levetiracetam
lamotrigine
topiramate
valproic acid
61
Age of febrile seizure
6-60mos
62
duration of SFS
<15 mins
63
duration of CFS
>15 mins
64
initially GTC then a brief period of post ictal drowsiness
SFS
65
indication of LP
-<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
Major risk factor for recurrence of febrile sz
<1yo
<24hrs fever
38-39C
67
minor RF for recurrence of febrile sz
```
family hx
CFS
daycare
male gender
hypothermia at presentation
```
68
gene mutation on chromosome 17q11.2
| encodes for neurofibromin w/c inhibits Ras oncogene
Von Recklinghausen dse
NF1
- progressive dse that can affect almost every organ
- MC skin lesions are cafe-au-lait spots
69
criteria for NF1
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
criteria for NF2
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
4 main manifestation of tuberous sclerosis
MR
seizure
cutaneous lesions
tumor in various organs
72
Diagnostic Criteria for TS
```
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
Ash lead on trunk & extremities
| Shagreen patch on lumbosacral region
tuberous sclerosis
74
serpentine or railroad track appearance
```
Sturge Weber Syndrome
- port wine stain
seizure
hemiparesis
stroke like episode
intracranial calcification
MR
brain is atrophic and calcified
```
75
MC cause of death of Von Hippel Lindau Dse
renal carcinoma
76
PHACE syndrome
```
Posterior fossa malformation
Hemangioma
Arterial abnormalities
Coarctation of the aorta
Eye abnormalities
```
tx: INFa
77
```
unilateral weakness with UMN signs
sensory abnormalities
visual complain
ataxia
paresthesia of LE & face
```
multiple sclerosis
tx: IV methylprednisolone
78
immune mediated demyelinating disorder can follow immunization or infections (MMRV, herpes zoster, URTI)
Acute Disseminated Encephalomyelitis (ADEM)
- fever, lethargy, weakness, ataxia, sz --> delirium, coma, myelopathy & focal neurologic signs
tx: IV methylprednisolone, IVIG
79
MC cause of neonatal meningitis
GBS
| E.coli
80
etiologic agent of bacterial meningitis in 1st 2 months of life
Grp B streptococcus
Gram negative enteric bacilli
L. monocytogenes
81
etiologic agent of bacterial meningitis in 2-12 yo
S. pneumoniae
H. influenza
N. meningitidis
82
MC cause of viral meningoencephalitis
enterovirus
83
MC manifestation of viral meningoencephalitis
cerebellar ataxia
84
MC cause of brain abscess in neonates
citrobacter
