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
NF1 –> bone abn –> sphenoid wing dysplasia –> potential complication?
pulsatile enoph/exophthalmos
NF1 –> assoc extracranial neoplasm? (4)
- Wilms tumor
- rhabdomyosarcoma
- renal AML
- leiomyosarcoma
NF1 vs NF2 –> which is MC?
NF1
NF2 –> synd?
MISME: mult inherited - schwannoma - meningioma - ependymoma
NF2 –> typical clinical presentation?
hearing loss –> d/t bilat vestibular schwannoma (acoustic neuroma)
what finding is dx for NF2?
bilat vestibular schwannoma (acoustic neuroma)
Sturge-Weber –> sydn? (3)
- capillary malformation –> facial port wine stain
- ocular abn
- cortical venous –> fail of normal dev
Sturge-Weber –> MOA?
primitive embryologic cephalic venous plexus –> fail to regress
Sturge-Weber –> port wine stain –> MC location?
trigeminal N (CN V1) –> ophthalmic br:
- forehead
- upper eyelid
Sturge-Weber –> cortical venous –> fail of normal dev –> what condition?
leptomeningeal venous angiomatosis: vascular malformation –> dilated capillaries & venules
Sturge-Weber –> leptomeningeal venous angiomatosis –> natural progression of dz?
chronic ischemia –> cortical atrophy –> cortical calcification
Sturge-Weber –> leptomeningeal venous angiomatosis –> characteristic imaging findings? (3)
- cortical atrophy –> subcortical calcification
- pial enhancement
- enlrg choroid plexus (increased flow –> compensatory hypertrophy)
what is phakomatoses?
grp of neurocutaneous disorder –> involve embryonic ectoderm struct:
- CNS
- skin
- eye
phakomatoses –> include which conditions? (5)
- NF1
- NF2
- Sturge-Weber
- tuberous sclerosis
- Von Hippel Lindau synd
tuberous sclerosis –> extracranial manifestation? (3)
- renal –> mult AML
- cardiac –> rhabdomyoma
- lung –> lymphangioleiomyomatosis
tuberous sclerosis –> brain findings? (3)
- sub/cortical tubers (hamartomas)
- subependymal nodules
- subependymal giant cell astrocytoma
tuberous sclerosis –> subependymal giant cell astrocytoma –> imaging appearance?
subependymal nodule:
- new enhance
- enlrging
midbrain –> “molar tooth” configuration –> ddx? (1)
Joubert synd & related disorders (JSRD)
Joubert synd & related disorders (JSRD) –> primary abn?
cerebellar vermis –> aplasia/hypoplasia
differentiate: Joubert synd & related disorders (JSRD) vs Dandy Walker complex? (1)
Joubert synd & related disorders (JSRD):
- no hydrocephalus
- no lrg post fossa cyst
Dandy Walker complex:
- hydrocephalus
- lrg post fossa cyst
differentiate: positional plagiocephaly vs unilat lambdoid synostosis –>
- onset
- ipsilat ear position
- side of frontal bossing
- how common?
- tx
positional plagiocephaly:
- onset: wks after birth
- ear position: ant
- frontal bossing: ipsilat
- how common: MC
- tx: conservative
unilat lambdoid synostosis:
- onset: birth
- ear position: post
- frontal bossing: contralat
- how common: least common
- tx: surg
what is ant plagiocephaly?
unilat coronal synostosis
what is post plagiocephaly?
unilat lambdoid synostosis
craniosynostosis –> lambdoid suture –> head shape?
turricephaly –> tall
scaphocephaly/dolichocephaly –> assoc condition?
Marfan’s
trigonocephaly –> eye appearance?
“quizzical eye”
trigonocephaly –> assoc clinical presentation?
growth restriction of frontal lobes –> cognitive disorder
coronal synostosis –> bilat –> association?
syndromic mental retard
craniosynostosis –> imaging modality of choice?
CT + 3D recons
absence/dysplasia of greater sphenoid wing –> dx?
NF1
tibial pseudoarthrosis –> dx?
NF1
scoliosis –> dx?
NF1
lat thoracic meningocele –> dx?
NF1
bone defect in region of lambdoid suture –> dx?
NF1
asterion defect –> dx?
NF1
Apert synd –> synd? (2)
- brachycephaly
- syndactyly
Crouzon’s synd –> mnenomic? (7)
C’s:
- coronal sutures fused
- can’t chew
- Chiari I
- coarctation
- hydroCephalus
- Central bones short (rhizomelia)
- Crazy eyes (exophthalmos)
3 types of skull markings?
- convolutional markings
- copper beaten
- Luckenschadel (“lacunar”)
what are convolutional markings of the skull?
skull –> inner table –> normal gyral impressions
convolutional markings of the skull –> age range? why?
3-7yo –> rapid brain growth
convolutional markings of the skull –> MC location?
post skull
copper beaten skull markings –> location?
ant & post skull
copper beaten skull markings –> cause?
increased intracranial pressure:
- craniosynostosis
- obstructive hydrocephalus
Luckenschadel (“lacunar”) skull markings –> cause?
defective bone matrix
Luckenschadel (“lacunar”) skull markings –> etiology? (2)
- Chiari II
- neural tube defect
what is parietal foramina?
benign congenital defect –> delayed/incomplete ossification –> symm holes in parietal bones
Wormian bones –> MC location?
lambdoid suture
Wormian bones –> dx?
- <10
- > 10
- > 10 & absent clavicle
- <10: idiopathic
- > 10: osteogenesis imperfecta
- > 10 & absent clavicle: cleidocranial dysostosis
Wormian bones –> >10 –> ddx mnemonic?
PORK-CHOP:
- pyknodysotosis
- osteogenesis imperfecta
- rickets
- kinky hair synd (Menke’s/fucked copper metab)
- cleidocranial dysostosis
- hypothyroid/hypophosphate
- one too many 21 chrom (Down)
- primary acro-osteolysis (Hajdu-Cheney)
skull –> dermoid/epidermoid cyst –> imaging appearance?
bone defect –> benign sclerotic borders
differentiate: epidermoid vs dermoid cyst –>
- histology
- onset
- MC location
- CT appearance
- MR appearance
epidermoid
- histology: skin only
- onset: 20-40yo
- MC location: parietal –> behind ears
- CT: CSF density
- MR: T1 variable, T2 bright, no enhance
dermoid:
- histology: skin + other (hair follicle, sweat gland, etc)
- onset: earlier
- MC location: midline
- CT appearance: more heterogeneous, calcification
- MR appearance: T1/T2 bright, +/- wall enhance
skull –> dermoid –> assoc condition?
encephalocele
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- location
- subgaleal hemorrhage: bw aponeurosis & periosteum
- cephalohematoma: subperiosteal
- caput succedaneum: subcutaneous
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- cross suture?
- subgaleal hemorrhage: yes
- cephalohematoma: no
- caput succedaneum: yes
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- cause?
- subgaleal hemorrhage: vacuum extraction
- cephalohematoma: instrumt/vacuum extraction
- caput succedaneum: prolonged delivery
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- potential complication?
- subgaleal hemorrhage: rapid blood loss –> life-threatening
- cephalohematoma: superinfx –> abscess, skull osteomyelitis
- caput succedaneum: N/A
subgaleal hemorrhage –> when present?
12-72hr post delivery
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- tx?
- subgaleal hemorrhage: rapid blood loss –> potentially life-threatening –> poss surg
- cephalohematoma: none
- caput succedaneum: none
head US –> caudothalamic groove –> echogenic focus –> dx?
- preterm
- full term
preterm: germinal matrix hemorrhage
full term: choroid plexus hemorrhage
germinal matrix hemorrhage –> onset?
90% –> 1st wk of life
germinal matrix hemorrhage –> when screen US head?
1st wk of life
germinal matrix hemorrhage –> screen US head –> who? (2)
- premature <32wk
- premature –> lethargy, sz, decreased Hb, look sick…
germinal matrix hemorrhage –> grading system?
1: caudothalamic groove
2: in ventricle –> no dil
3: in ventricles –> dil
4: in parenchyma
GMH grade 4 –> MOA?
venous infarct
GMH grade 1 –> natural course? (2)
- resolve
- subependymal cyst
GMH grade 4 –> natural course?
porencephalic cyst (infarct –> hole)
pyriform aperture stenosis –> CT appearance? measuremt?
maxillary spines –> inward bow –> aperture <8mm
pyriform aperture stenosis –> assoc? (3)
- holoprosencephaly
- pituitary dysfx
- central mega incisor
CT show pyriform aperture stenosis –> next step?
CT brain
cyclical cyanosis –> improve w crying –> dx?
bilat choanal atresia
3 craniofacial syndromes?
- Crouzons
- Aperts
- Treacher-Collins (mandibulo-facial dysostosis)
Treacher-Collins synd (mandibulo-facial dysostosis) –> synd? (3)
- maxilla –> narrow –> protrude
- mandible hypoplasia –> retruded
- zygomatic arch hypoplasia
ectopic thyroid –> imaging modality of choice?
- I-123
- Tc-MIBI
birth –> hypothyroid screen –> if abnormal –> next step?
workup for:
- ectopic thyroid
- enzyme def
ectopic thyroid –> MC location?
back of tongue –> lingual thyroid
child –> midline cyst in neck –> dx?
thyroglossal duct cyst
thyroglossal duct cyst –> MC location? (2)
- base of tongue
- ant hyoid
thyroglossal duct cyst –> tx
Sistrunk procedure –> resect:
- cyst
- tract
- hyoid
thyroglossal duct cyst –> enhancing nodule –> dx?
cancer (usu papillary)
imaging demonstrate thyroglossal duct cyst –> next step?
- US –> confirm normal thyroid location
- Tc-MIBI/I-123 –> look for ectopic thyroid
neck –> dermoid cyst –> location?
midline:
- sublingual
- submandibular
neck –> dermoid cyst –> imaging appearance?
“sac of marbles” –> fat lobules in fluid
MC branchial cleft cyst?
2nd branchial cleft cyst
2nd branchial cleft cyst –> MC location
angle of mandible
2nd branchial cleft cyst –> pathognomonic finding?
notch sign: just above carotid bifurcation –> cyst bw ICA & ECA
18yo –> angle of mandible –> new cyst –> dx? next step?
necrotic LN:
- find cancer
- bx
cyst in lateral neck –> dx?
2nd branchial cleft cyst
hemangioma of infancy –> key MRI imaging afindings? US?
MRI:
- super T2 bright
- flow voids
US:
- diffusely vascular
hemangioma of infancy –> natural progression?
present at 6mo –> grow –> plateau –> involute at 6-10yo
hemangioma of infancy –> tx –> indication?
lrg size/rapid growth –> mass effect:
- airway
- vasc
- eye
hemangioma of infancy –> mass effect on airway –> tx?
BB (propranolol)
hemangioma of infancy –> assoc synd?
PHACES synd
differentiate: infantile hemangioma vs congenital hemangioma –>
- onset?
- involute?
infantile hemangioma:
- 6mo
- spont regress
congenital hemangioma:
- birth
- spont regress or not
OB US –> back of neck –> cystic mass –> dx?
cystic hygroma (lymphangioma)
cystic hygroma (lymphangioma) –> what location?
neck –> post triangle
cystic hygroma (lymphangioma) –> assoc? (4)
- Turners
- Downs
- aortic coarct
- fetal hydrops
differeniate: cystic hygroma vs hemangioma –>
- T2
- enhance?
cystic hygroma:
- T2 bright
- no enhance
hemangioma:
- T2 bright
- enhance
cystic hygroma (lymphangioma) –> finding that indicates worse outcome?
septations
medulloblastoma –> MC location
cerebellar vermis –> midline
medulloblastoma –> potential complication? imaging appearance?
leptomeningeal mets –> “sugar coating” –> icing-like enhance over brain surface
YA –> medulloblastoma –> MC location
eccentric –> cerebellar hemisphere
medulloblastoma –> imaging appearance:
- CT –> noncontrast
- MR –> T2, ADC
- enhance
CT: slight hyperdense (d/t densely packed cells)
MR:
- T2 hypo
- ADC low
avid enhance –> heterogenous d/t internal calc/hemorrhage
child –> MC suprasellar lesion?
craniopharyngioma
craniopharyngioma –> embryologic origin?
Rathke’s pouch –> squamous epithelial remnants –> produce keratin
craniopharyngioma –> epidemiology?
bimodal:
- child
- late middle age
craniopharyngioma –> pituitary or extra-pituitary?
extra-pit
craniopharyngioma –> MRI appearance?
complex cystic mass:
- calc
- can contain protein/blood (T1 hyper)
- enhance
craniopharyngioma –> characteristic gross finding?
machine oil
retinoblastoma –> age?
<5yo
retinoblastoma –> CT appearance?
retinal mass:
- hyperdense
- calc
- enhance
what is trilat retinoblastoma?
- bilat retinoblastoma
- pineal gland –> pineoblastoma
what is quadrilat retinoblastoma?
- bilat retinoblastoma
- pineoblastoma
- suprasellar retinoblastoma
Chiari II –> MOA?
myelomeningocele –> CSF loss –> post fossa –> sump type effect
child –> MC primary brain tumor
juvenile pilocytic astrocytoma
juvenile pilocytic astrocytoma –> 2 MC location
#1 cerebellum #2 optic chiasm
juvenile pilocytic astrocytoma –> assoc synd?
NF1
juvenile pilocytic astrocytoma –> classic imaging appearance?
cerebellum –> cystic lesion –> enhancing mural nodule