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