Peds Neuro Flashcards
full term newborn –> what will be myelinated at birth?
deep brain:
- internal capsule post limb
- middle cerebellar peduncle
- thalamus
- brainstem dorsal
- perirolandic region
myelination –> mature by what age?
2yo
myelination –> T1 signal? T2 signal?
T1: bright
T2: dark
myelination –> progression pattern?
central to peripheral
caudal to rostral
dorsal to ventral
sensory then motor
dev of normal cortex –> depend on what aspect of germinal matrix cell? (2)
migration –> organization
what is polymicrogyria?
neuronal migration or cortical lamellar organization –> disturbed –> cortex –> normal 6-layer organization –> deranged –> cortical dev malform –> sulci –> numerous + shallow
polymicrogyria –> etiology? (3)
- in utero infx (ie CMV)
- in utero ischemia
- genetic
polymicrogyria –> clinical manifestation? (3)
- dev delay
- quadriparesis
- intractable sz
polymicrogyria –> MC location?
bilat perisylvian
polymicrogyria –> MR appearance? (2)
- sulci –> small, thick, bumpy, irreg
- G-W diff –> irreg, less well-defined
what is lissencephaly? MOA?
arrested neurons –> not complete outward migration to superficial cortex –> cortex 4 layers (normal 6) –> absent/decreased cortical convulution –> cortical surface thick & smooth
lissencephaly –> etiology –> 1 example?
CMV (will show calcifications)
lissencephaly –> clinical presentation?
sz
lissencephaly –> 2 subtypes?
- type I (classical)
- type II (cobblestone)
type II (cobblestone) lissencephaly –> assoc synd?
Walker-Warburg synd
Walker-Warburg synd –> synd? (4)
- cobblestone lissencephaly
- cerebellar malformation
- ocular malform
- muscular dystrophy
type I (classical) lissencephaly –> key imaging findings? (2)
- smooth cortex
- cerebral hemisphere –> “hourglass” (“figure of 8”) configuration
type II (cobblestone) lissencephaly –> key imaging finding?
finely undulating cortex
what is gray matter heterotopia?
clusters of normal neurons –> abnormal locations
gray matter heterotopia –> potential complication?
sz
gray matter heterotopia –> 3 main subtypes?
- periventricular nodular heterotopia
- subcortical heterotopia
- marginal glioneural heterotopia
corpus callosum –> agenesis –> MC clinical manifestation? (2)
- refractile sz
- dev delay
hydrocephalus –> ventricular/hemispheric ratio –> how to measure? normal value?
coronal –> level of 3rd ventricle/foramen of Monro –> midline to lat ventricle/midline to hemisphere –> <0.35
hydrocephalus –> 3rd ventricle –> normal value?
transverse <5mm
what is craniosynostosis?
premature closure of skull suture
craniosynostosis –> etiology?
- idiopathic
- primary condition
- 2ary/syndromic (ie genetic, metabolic)
primary craniosynostosis –> presentation? epidemiology?
M>F –> at birth
primary craniosynostosis –> MC location?
sagittal suture
craniosynostosis –> sagittal suture –> head shape?
scaphocephaly/dolichocephaly –> long & narrow
craniosynostosis –> coronal suture –> head shape?
brachycephaly –> short AP, wide lat
craniosynostosis –> coronal suture –> brachycephaly –> orbit appearance?
“harlequin eye”
craniosynostosis –> metopic suture –> head shape?
trigonocephaly –> pty forehead
craniosynostosis –> all sutures –> what condition?
clover-leaf skull (Kleeblattschadel)
clover-leaf skull (Kleeblattschadel) –> assoc synd? (3)
- thanatophoric dysplasia
- Apert synd
- Crouzon synd
what is colpocephaly?
lat ventricle –> occipital horn –> dilated
corpus callosum agenesis –> ventricular system appearance?
lat ventricle:
- parallel, wide spaced
- colpocephaly
- medial impressions
3rd ventricle:
- enlrg
- high-riding
corpus callosum agenesis –> colpocephaly –> MOA?
post brain –> white matter decrease
corpus callosum agenesis –> lat ventricle –> medial impressions –> MOA?
corpus callosum agenesis –> axons that normally constitute corpus callosum (Probst bundles) –> aberrant course –> parallel interhemispheric fissure –> medial impressions on lat ventricles
corpus callosum agenesis –> ventricular system appearance –> signs? (2)
- racing car sign
- moose head sign
corpus callosum agenesis –> assoc conditions? (2)
other midline abnormalities:
- midline lipoma
- interhemispheric cyst
what is schizencephaly?
malformation –> cerebral hemisphere –> full thickness cleft –> lined by dysplastic gray matter (usu polymicrogyria) –> abnormal comm bw ventricles & subarachoid space
schizencephaly –> assoc condition? (2)
- 30% –> cortical malformation (gray matter heterotopia)
- septo-optic dysplasia
schizencephaly –> 2 subtypes?
- open lip
- closed lip
septo-optic dysplasia –> synd? (2)
- septum pellucidum –> agenesis
- optic N hypoplasia
schizencephaly –> MC location?
frontal lobe
cortical cleft –> ddx? (4)
- schizencephaly
- porencephaly (cortex replaced by cystic struct)
- encephalomalacia
- surg resect cavity
differentiate: schizencephaly vs other cortical clefts? (1)
schizencephaly –> lined by gray matter
other cortical clefts –> not lined by gray matter
what is holoprosencephaly?
congenital malformation –> forebrain –> not divide into 2 hemispheres
holoprosencephaly –> assoc anomalies? (2)
- midline maxillofacial anomalies –> single central incisor
- ACA –> azygous configuration (single ACA)
holoprosencephaly –> 3 subtypes?
most to least severe:
- alobar
- semilobar
- lobar
alobar holoprosencephaly –> imaging appearance? (2)
- cerebral hemispheres –> complete lack of separation
- single lrg monoventricle –> comm w lrg dorsal cyst
semilobar holoprosencephaly –> imaging appearance? (3)
- cerebral hemisphere –> ant –> unified
- cerebral hemisphere –> post –> some separation
- post midline –> lrg interhemispheric cyst
lobar holoprosencephaly –> imaging appearance? (2)
- cerebral hemisphere –> ant –> most rostral aspect –> unified
- corpus callosum –> ant –> absent
what is Chiari I?
cerebellar tonsils –> inf displace –> below foramen magnum
Chiari I –> 2 MC complications?
- # 1 cervical syringomyelia
- hydrocephalus
Chiari I –> clinical manifestation? (3)
variable:
- occasional exertional HA
- severe myelopathy, brainstem compromise
- sleep apnea, feeding problems
what is syringomeylia?
fluid w/in spinal cord –> usu dilation of central canal
differentiate: Chiari I syringomyelia vs other syringomyelia? (1)
Chiari I syringomyelia –> no comm w 4th ventricle
other syringomyelia –> may comm w 4th vent
Chiari I –> classic dx criterion?
cerebellar tonsil herniation:
- unilat –> 5mm
- bilat –> 3mm
pediatric –> CT brain –> cerebellar tonsil –> inf displaced –> no other findings –> impression?
borderline tonsillar ectopia
Chiari I –> cerebellar tonsil herniation –> other assoc findings? (4)
- post fossa –> crowded
- cerebellar tonsils –> pted
- syringomyelia
- hydrocephalus
Chiari II –> 1ary abnormality?
vermis, cerebellar tonsils, medulla –> herniate into foramen magnum –> resultant tectum beaking
Chiari II –> hindbrain herniate –> other findings? (4)
- myelomeningocele –> lumbar
- 4th ventricle –> inf –> elong
- hydrocephalus
- supratentorial anomaly (corpus callosum dysgenesis, heterotopia, sulcation abnormal)
Dandy-Walker malformation –> key findings? (4)
- enlrg post fossa
- lrg post fossa cyst –> comm w 4th vent
- vermis hypoplasia
- torcular-lambdoid inversion
differentiate: Dandy-Walker malformation vs variant (1)
Dandy-Walker malformation: enlrg post fossa
Dandy-Walker variant: normal post fossa size
neurofibromatosis type 1 (von Recklinghausen dz) –> synd? (4)
- cafe au lait spots
- peripheral N sheath tumor (plexiform neurofibroma)
- CNS malig (optic N glioma)
- bony abn (sphenoid wing dysplasia)
NF1 –> what is “bright spots” (“unidentified bright objects”)?
- seen on what MR seq?
- age range?
4-12yo
myelin vacuolization –> T2 bright spots
what is neurofibroma? MC location?
N sheath tumor –> WHO grade I
sub/cutaneous N
what is plexiform neurofibroma?
neurofibroma subtype –> more aggressive
plexiform neurofibroma –> potential complication?
malig degen
neurofibromatosis type 1 (von Recklinghausen dz) –> assoc w what brain neoplasms? (3)
- optic N glioma
- juvenile pilocytic astrocytoma
- brainstem glioma
optic N glioma –> assoc synd/condition?
50% –> NF1
tumors of Schwann cell origin? (2)
- schwannoma
- neurofibroma
neurofibroma –> imaging appearance?
network of fusiform shaped masses
- target sign
neurofibroma –> what is target sign?
fibrocollagenous core –> central T2 hypo
nerve –> target sign –> ddx? (2)
what does target sign indicate?
- schwannoma
- neurofibroma
benign
differentiate: schwannoma vs neurofibroma? (1)
schwannoma
- encapsulated
neurofibroma
- nonencapsulated
neurofibromatosis type 1 (von Recklinghausen dz) –> assoc w what bone abn? (7)
- sphenoid wing dysplasia
- post vert body scallop
- rib notch “twisted ribbon ribs”
- focal gigantism
- cervical kyphoscoliosis –> acute angle
- neuroforamin enlrg
- tibia bowing