Peds Neuro Flashcards
1
Q
full term newborn –> what will be myelinated at birth?
A
deep brain:
- internal capsule post limb
- middle cerebellar peduncle
- thalamus
- brainstem dorsal
- perirolandic region
2
Q
myelination –> mature by what age?
A
2yo
3
Q
myelination –> T1 signal? T2 signal?
A
T1: bright
T2: dark
4
Q
myelination –> progression pattern?
A
central to peripheral
caudal to rostral
dorsal to ventral
sensory then motor
5
Q
dev of normal cortex –> depend on what aspect of germinal matrix cell? (2)
A
migration –> organization
6
Q
what is polymicrogyria?
A
neuronal migration or cortical lamellar organization –> disturbed –> cortex –> normal 6-layer organization –> deranged –> cortical dev malform –> sulci –> numerous + shallow
7
Q
polymicrogyria –> etiology? (3)
A
- in utero infx (ie CMV)
- in utero ischemia
- genetic
8
Q
polymicrogyria –> clinical manifestation? (3)
A
- dev delay
- quadriparesis
- intractable sz
9
Q
polymicrogyria –> MC location?
A
bilat perisylvian
10
Q
polymicrogyria –> MR appearance? (2)
A
- sulci –> small, thick, bumpy, irreg
- G-W diff –> irreg, less well-defined
11
Q
what is lissencephaly? MOA?
A
arrested neurons –> not complete outward migration to superficial cortex –> cortex 4 layers (normal 6) –> absent/decreased cortical convulution –> cortical surface thick & smooth
12
Q
lissencephaly –> etiology –> 1 example?
A
CMV (will show calcifications)
13
Q
lissencephaly –> clinical presentation?
A
sz
14
Q
lissencephaly –> 2 subtypes?
A
- type I (classical)
- type II (cobblestone)
15
Q
type II (cobblestone) lissencephaly –> assoc synd?
A
Walker-Warburg synd
16
Q
Walker-Warburg synd –> synd? (4)
A
- cobblestone lissencephaly
- cerebellar malformation
- ocular malform
- muscular dystrophy
17
Q
type I (classical) lissencephaly –> key imaging findings? (2)
A
- smooth cortex
- cerebral hemisphere –> “hourglass” (“figure of 8”) configuration
18
Q
type II (cobblestone) lissencephaly –> key imaging finding?
A
finely undulating cortex
19
Q
what is gray matter heterotopia?
A
clusters of normal neurons –> abnormal locations
20
Q
gray matter heterotopia –> potential complication?
A
sz
21
Q
gray matter heterotopia –> 3 main subtypes?
A
- periventricular nodular heterotopia
- subcortical heterotopia
- marginal glioneural heterotopia
22
Q
corpus callosum –> agenesis –> MC clinical manifestation? (2)
A
- refractile sz
- dev delay
23
Q
hydrocephalus –> ventricular/hemispheric ratio –> how to measure? normal value?
A
coronal –> level of 3rd ventricle/foramen of Monro –> midline to lat ventricle/midline to hemisphere –> <0.35
24
Q
hydrocephalus –> 3rd ventricle –> normal value?
A
transverse <5mm
25
what is craniosynostosis?
premature closure of skull suture
26
craniosynostosis --> etiology?
- idiopathic
- primary condition
- 2ary/syndromic (ie genetic, metabolic)
27
primary craniosynostosis --> presentation? epidemiology?
M>F --> at birth
28
primary craniosynostosis --> MC location?
sagittal suture
29
craniosynostosis --> sagittal suture --> head shape?
scaphocephaly/dolichocephaly --> long & narrow
30
craniosynostosis --> coronal suture --> head shape?
brachycephaly --> short AP, wide lat
31
craniosynostosis --> coronal suture --> brachycephaly --> orbit appearance?
"harlequin eye"
32
craniosynostosis --> metopic suture --> head shape?
trigonocephaly --> pty forehead
33
craniosynostosis --> all sutures --> what condition?
clover-leaf skull (Kleeblattschadel)
34
clover-leaf skull (Kleeblattschadel) --> assoc synd? (3)
- thanatophoric dysplasia
- Apert synd
- Crouzon synd
35
what is colpocephaly?
lat ventricle --> occipital horn --> dilated
36
corpus callosum agenesis --> ventricular system appearance?
lat ventricle:
- parallel, wide spaced
- colpocephaly
- medial impressions
3rd ventricle:
- enlrg
- high-riding
37
corpus callosum agenesis --> colpocephaly --> MOA?
post brain --> white matter decrease
38
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
39
corpus callosum agenesis --> ventricular system appearance --> signs? (2)
- racing car sign
| - moose head sign
40
corpus callosum agenesis --> assoc conditions? (2)
other midline abnormalities:
- midline lipoma
- interhemispheric cyst
41
what is schizencephaly?
malformation --> cerebral hemisphere --> full thickness cleft --> lined by dysplastic gray matter (usu polymicrogyria) --> abnormal comm bw ventricles & subarachoid space
42
schizencephaly --> assoc condition? (2)
- 30% --> cortical malformation (gray matter heterotopia)
| - septo-optic dysplasia
43
schizencephaly --> 2 subtypes?
- open lip
| - closed lip
44
septo-optic dysplasia --> synd? (2)
- septum pellucidum --> agenesis
| - optic N hypoplasia
45
schizencephaly --> MC location?
frontal lobe
46
cortical cleft --> ddx? (4)
- schizencephaly
- porencephaly (cortex replaced by cystic struct)
- encephalomalacia
- surg resect cavity
47
differentiate: schizencephaly vs other cortical clefts? (1)
schizencephaly --> lined by gray matter
other cortical clefts --> not lined by gray matter
48
what is holoprosencephaly?
congenital malformation --> forebrain --> not divide into 2 hemispheres
49
holoprosencephaly --> assoc anomalies? (2)
- midline maxillofacial anomalies --> single central incisor
| - ACA --> azygous configuration (single ACA)
50
holoprosencephaly --> 3 subtypes?
most to least severe:
- alobar
- semilobar
- lobar
51
alobar holoprosencephaly --> imaging appearance? (2)
- cerebral hemispheres --> complete lack of separation
| - single lrg monoventricle --> comm w lrg dorsal cyst
52
semilobar holoprosencephaly --> imaging appearance? (3)
- cerebral hemisphere --> ant --> unified
- cerebral hemisphere --> post --> some separation
- post midline --> lrg interhemispheric cyst
53
lobar holoprosencephaly --> imaging appearance? (2)
- cerebral hemisphere --> ant --> most rostral aspect --> unified
- corpus callosum --> ant --> absent
54
what is Chiari I?
cerebellar tonsils --> inf displace --> below foramen magnum
55
Chiari I --> 2 MC complications?
- #1 cervical syringomyelia
| - hydrocephalus
56
Chiari I --> clinical manifestation? (3)
variable:
- occasional exertional HA
- severe myelopathy, brainstem compromise
- sleep apnea, feeding problems
57
what is syringomeylia?
fluid w/in spinal cord --> usu dilation of central canal
58
differentiate: Chiari I syringomyelia vs other syringomyelia? (1)
Chiari I syringomyelia --> no comm w 4th ventricle
other syringomyelia --> may comm w 4th vent
59
Chiari I --> classic dx criterion?
cerebellar tonsil herniation:
- unilat --> 5mm
- bilat --> 3mm
60
pediatric --> CT brain --> cerebellar tonsil --> inf displaced --> no other findings --> impression?
borderline tonsillar ectopia
61
Chiari I --> cerebellar tonsil herniation --> other assoc findings? (4)
- post fossa --> crowded
- cerebellar tonsils --> pted
- syringomyelia
- hydrocephalus
62
Chiari II --> 1ary abnormality?
vermis, cerebellar tonsils, medulla --> herniate into foramen magnum --> resultant tectum beaking
63
Chiari II --> hindbrain herniate --> other findings? (4)
- myelomeningocele --> lumbar
- 4th ventricle --> inf --> elong
- hydrocephalus
- supratentorial anomaly (corpus callosum dysgenesis, heterotopia, sulcation abnormal)
64
Dandy-Walker malformation --> key findings? (4)
- enlrg post fossa
- lrg post fossa cyst --> comm w 4th vent
- vermis hypoplasia
- torcular-lambdoid inversion
65
differentiate: Dandy-Walker malformation vs variant (1)
Dandy-Walker malformation: enlrg post fossa
Dandy-Walker variant: normal post fossa size
66
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)
67
NF1 --> what is "bright spots" ("unidentified bright objects")?
- seen on what MR seq?
- age range?
4-12yo
myelin vacuolization --> T2 bright spots
68
what is neurofibroma? MC location?
N sheath tumor --> WHO grade I
sub/cutaneous N
69
what is plexiform neurofibroma?
neurofibroma subtype --> more aggressive
70
plexiform neurofibroma --> potential complication?
malig degen
71
neurofibromatosis type 1 (von Recklinghausen dz) --> assoc w what brain neoplasms? (3)
- optic N glioma
- juvenile pilocytic astrocytoma
- brainstem glioma
72
optic N glioma --> assoc synd/condition?
50% --> NF1
73
tumors of Schwann cell origin? (2)
- schwannoma
| - neurofibroma
74
neurofibroma --> imaging appearance?
network of fusiform shaped masses
| - target sign
75
neurofibroma --> what is target sign?
fibrocollagenous core --> central T2 hypo
76
nerve --> target sign --> ddx? (2)
what does target sign indicate?
- schwannoma
- neurofibroma
benign
77
differentiate: schwannoma vs neurofibroma? (1)
schwannoma
- encapsulated
neurofibroma
- nonencapsulated
78
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
79
NF1 --> bone abn --> sphenoid wing dysplasia --> potential complication?
pulsatile enoph/exophthalmos
80
NF1 --> assoc extracranial neoplasm? (4)
- Wilms tumor
- rhabdomyosarcoma
- renal AML
- leiomyosarcoma
81
NF1 vs NF2 --> which is MC?
NF1
82
NF2 --> synd?
```
MISME:
mult inherited
- schwannoma
- meningioma
- ependymoma
```
83
NF2 --> typical clinical presentation?
hearing loss --> d/t bilat vestibular schwannoma (acoustic neuroma)
84
what finding is dx for NF2?
bilat vestibular schwannoma (acoustic neuroma)
85
Sturge-Weber --> sydn? (3)
- capillary malformation --> facial port wine stain
- ocular abn
- cortical venous --> fail of normal dev
86
Sturge-Weber --> MOA?
primitive embryologic cephalic venous plexus --> fail to regress
87
Sturge-Weber --> port wine stain --> MC location?
trigeminal N (CN V1) --> ophthalmic br:
- forehead
- upper eyelid
88
Sturge-Weber --> cortical venous --> fail of normal dev --> what condition?
leptomeningeal venous angiomatosis: vascular malformation --> dilated capillaries & venules
89
Sturge-Weber --> leptomeningeal venous angiomatosis --> natural progression of dz?
chronic ischemia --> cortical atrophy --> cortical calcification
90
Sturge-Weber --> leptomeningeal venous angiomatosis --> characteristic imaging findings? (3)
- cortical atrophy --> subcortical calcification
- pial enhancement
- enlrg choroid plexus (increased flow --> compensatory hypertrophy)
91
what is phakomatoses?
grp of neurocutaneous disorder --> involve embryonic ectoderm struct:
- CNS
- skin
- eye
92
phakomatoses --> include which conditions? (5)
- NF1
- NF2
- Sturge-Weber
- tuberous sclerosis
- Von Hippel Lindau synd
93
tuberous sclerosis --> extracranial manifestation? (3)
- renal --> mult AML
- cardiac --> rhabdomyoma
- lung --> lymphangioleiomyomatosis
94
tuberous sclerosis --> brain findings? (3)
- sub/cortical tubers (hamartomas)
- subependymal nodules
- subependymal giant cell astrocytoma
95
tuberous sclerosis --> subependymal giant cell astrocytoma --> imaging appearance?
subependymal nodule:
- new enhance
- enlrging
96
midbrain --> "molar tooth" configuration --> ddx? (1)
Joubert synd & related disorders (JSRD)
97
Joubert synd & related disorders (JSRD) --> primary abn?
cerebellar vermis --> aplasia/hypoplasia
98
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
99
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
100
what is ant plagiocephaly?
unilat coronal synostosis
101
what is post plagiocephaly?
unilat lambdoid synostosis
102
craniosynostosis --> lambdoid suture --> head shape?
turricephaly --> tall
103
scaphocephaly/dolichocephaly --> assoc condition?
Marfan's
104
trigonocephaly --> eye appearance?
"quizzical eye"
105
trigonocephaly --> assoc clinical presentation?
growth restriction of frontal lobes --> cognitive disorder
106
coronal synostosis --> bilat --> association?
syndromic mental retard
107
craniosynostosis --> imaging modality of choice?
CT + 3D recons
108
absence/dysplasia of greater sphenoid wing --> dx?
NF1
109
tibial pseudoarthrosis --> dx?
NF1
110
scoliosis --> dx?
NF1
111
lat thoracic meningocele --> dx?
NF1
112
bone defect in region of lambdoid suture --> dx?
NF1
113
asterion defect --> dx?
NF1
114
Apert synd --> synd? (2)
- brachycephaly
| - syndactyly
115
Crouzon's synd --> mnenomic? (7)
C's:
- coronal sutures fused
- can't chew
- Chiari I
- coarctation
- hydroCephalus
- Central bones short (rhizomelia)
- Crazy eyes (exophthalmos)
116
3 types of skull markings?
- convolutional markings
- copper beaten
- Luckenschadel ("lacunar")
117
what are convolutional markings of the skull?
skull --> inner table --> normal gyral impressions
118
convolutional markings of the skull --> age range? why?
3-7yo --> rapid brain growth
119
convolutional markings of the skull --> MC location?
post skull
120
copper beaten skull markings --> location?
ant & post skull
121
copper beaten skull markings --> cause?
increased intracranial pressure:
- craniosynostosis
- obstructive hydrocephalus
122
Luckenschadel ("lacunar") skull markings --> cause?
defective bone matrix
123
Luckenschadel ("lacunar") skull markings --> etiology? (2)
- Chiari II
| - neural tube defect
124
what is parietal foramina?
benign congenital defect --> delayed/incomplete ossification --> symm holes in parietal bones
125
Wormian bones --> MC location?
lambdoid suture
126
Wormian bones --> dx?
- <10
- >10
- >10 & absent clavicle
- <10: idiopathic
- >10: osteogenesis imperfecta
- >10 & absent clavicle: cleidocranial dysostosis
127
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)
128
skull --> dermoid/epidermoid cyst --> imaging appearance?
bone defect --> benign sclerotic borders
129
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
130
skull --> dermoid --> assoc condition?
encephalocele
131
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- location
- subgaleal hemorrhage: bw aponeurosis & periosteum
- cephalohematoma: subperiosteal
- caput succedaneum: subcutaneous
132
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- cross suture?
- subgaleal hemorrhage: yes
- cephalohematoma: no
- caput succedaneum: yes
133
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- cause?
- subgaleal hemorrhage: vacuum extraction
- cephalohematoma: instrumt/vacuum extraction
- caput succedaneum: prolonged delivery
134
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
135
subgaleal hemorrhage --> when present?
12-72hr post delivery
136
differentiate: subgaleal hemorrhage vs cephalohematoma vs caput succedaneum
- tx?
- subgaleal hemorrhage: rapid blood loss --> potentially life-threatening --> poss surg
- cephalohematoma: none
- caput succedaneum: none
137
head US --> caudothalamic groove --> echogenic focus --> dx?
- preterm
- full term
preterm: germinal matrix hemorrhage
full term: choroid plexus hemorrhage
138
germinal matrix hemorrhage --> onset?
90% --> 1st wk of life
139
germinal matrix hemorrhage --> when screen US head?
1st wk of life
140
germinal matrix hemorrhage --> screen US head --> who? (2)
- premature <32wk
| - premature --> lethargy, sz, decreased Hb, look sick...
141
germinal matrix hemorrhage --> grading system?
1: caudothalamic groove
2: in ventricle --> no dil
3: in ventricles --> dil
4: in parenchyma
142
GMH grade 4 --> MOA?
venous infarct
143
GMH grade 1 --> natural course? (2)
- resolve
| - subependymal cyst
144
GMH grade 4 --> natural course?
porencephalic cyst (infarct --> hole)
145
pyriform aperture stenosis --> CT appearance? measuremt?
maxillary spines --> inward bow --> aperture <8mm
146
pyriform aperture stenosis --> assoc? (3)
- holoprosencephaly
- pituitary dysfx
- central mega incisor
147
CT show pyriform aperture stenosis --> next step?
CT brain
148
cyclical cyanosis --> improve w crying --> dx?
bilat choanal atresia
149
3 craniofacial syndromes?
- Crouzons
- Aperts
- Treacher-Collins (mandibulo-facial dysostosis)
150
Treacher-Collins synd (mandibulo-facial dysostosis) --> synd? (3)
- maxilla --> narrow --> protrude
- mandible hypoplasia --> retruded
- zygomatic arch hypoplasia
151
ectopic thyroid --> imaging modality of choice?
- I-123
| - Tc-MIBI
152
birth --> hypothyroid screen --> if abnormal --> next step?
workup for:
- ectopic thyroid
- enzyme def
153
ectopic thyroid --> MC location?
back of tongue --> lingual thyroid
154
child --> midline cyst in neck --> dx?
thyroglossal duct cyst
155
thyroglossal duct cyst --> MC location? (2)
- base of tongue
| - ant hyoid
156
thyroglossal duct cyst --> tx
Sistrunk procedure --> resect:
- cyst
- tract
- hyoid
157
thyroglossal duct cyst --> enhancing nodule --> dx?
cancer (usu papillary)
158
imaging demonstrate thyroglossal duct cyst --> next step?
- US --> confirm normal thyroid location
| - Tc-MIBI/I-123 --> look for ectopic thyroid
159
neck --> dermoid cyst --> location?
midline:
- sublingual
- submandibular
160
neck --> dermoid cyst --> imaging appearance?
"sac of marbles" --> fat lobules in fluid
161
MC branchial cleft cyst?
2nd branchial cleft cyst
162
2nd branchial cleft cyst --> MC location
angle of mandible
163
2nd branchial cleft cyst --> pathognomonic finding?
notch sign: just above carotid bifurcation --> cyst bw ICA & ECA
164
18yo --> angle of mandible --> new cyst --> dx? next step?
necrotic LN:
- find cancer
- bx
165
cyst in lateral neck --> dx?
2nd branchial cleft cyst
166
hemangioma of infancy --> key MRI imaging afindings? US?
MRI:
- super T2 bright
- flow voids
US:
- diffusely vascular
167
hemangioma of infancy --> natural progression?
present at 6mo --> grow --> plateau --> involute at 6-10yo
168
hemangioma of infancy --> tx --> indication?
lrg size/rapid growth --> mass effect:
- airway
- vasc
- eye
169
hemangioma of infancy --> mass effect on airway --> tx?
BB (propranolol)
170
hemangioma of infancy --> assoc synd?
PHACES synd
171
differentiate: infantile hemangioma vs congenital hemangioma -->
- onset?
- involute?
infantile hemangioma:
- 6mo
- spont regress
congenital hemangioma:
- birth
- spont regress or not
172
OB US --> back of neck --> cystic mass --> dx?
cystic hygroma (lymphangioma)
173
cystic hygroma (lymphangioma) --> what location?
neck --> post triangle
174
cystic hygroma (lymphangioma) --> assoc? (4)
- Turners
- Downs
- aortic coarct
- fetal hydrops
175
differeniate: cystic hygroma vs hemangioma -->
- T2
- enhance?
cystic hygroma:
- T2 bright
- no enhance
hemangioma:
- T2 bright
- enhance
176
cystic hygroma (lymphangioma) --> finding that indicates worse outcome?
septations
177
medulloblastoma --> MC location
cerebellar vermis --> midline
178
medulloblastoma --> potential complication? imaging appearance?
leptomeningeal mets --> "sugar coating" --> icing-like enhance over brain surface
179
YA --> medulloblastoma --> MC location
eccentric --> cerebellar hemisphere
180
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
181
child --> MC suprasellar lesion?
craniopharyngioma
182
craniopharyngioma --> embryologic origin?
Rathke's pouch --> squamous epithelial remnants --> produce keratin
183
craniopharyngioma --> epidemiology?
bimodal:
- child
- late middle age
184
craniopharyngioma --> pituitary or extra-pituitary?
extra-pit
185
craniopharyngioma --> MRI appearance?
complex cystic mass:
- calc
- can contain protein/blood (T1 hyper)
- enhance
186
craniopharyngioma --> characteristic gross finding?
machine oil
187
retinoblastoma --> age?
<5yo
188
retinoblastoma --> CT appearance?
retinal mass:
- hyperdense
- calc
- enhance
189
what is trilat retinoblastoma?
- bilat retinoblastoma
| - pineal gland --> pineoblastoma
190
what is quadrilat retinoblastoma?
- bilat retinoblastoma
- pineoblastoma
- suprasellar retinoblastoma
191
Chiari II --> MOA?
myelomeningocele --> CSF loss --> post fossa --> sump type effect
192
child --> MC primary brain tumor
juvenile pilocytic astrocytoma
193
juvenile pilocytic astrocytoma --> 2 MC location
```
#1 cerebellum
#2 optic chiasm
```
194
juvenile pilocytic astrocytoma --> assoc synd?
NF1
195
juvenile pilocytic astrocytoma --> classic imaging appearance?
cerebellum --> cystic lesion --> enhancing mural nodule
