Flashcards in Path VII Deck (45):
1
myelomeningocele
herniation of CNS tissue through vertebral defect
2
common spot for myelomeningocele
lumbosacral
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Tx myelomeningocele
surgical correction
4
meckel gruber syndrome
ciliopathy
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what type of defect is encephalocele
defect in cranial mesodermal development
6
where do enecephalocele's herniate
through axial mesodermal defect of the skull
7
most common spot for herniation of encephalocele
occipital lobe
8
cardinal feature of Meckel Gruber syndrome
encephalocele
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clinical signs meckel gruber syndrome
cysts in kidneys
polydactlyly
encephalocele
10
difference in kinesin and dynein
kinesin is anterograde
dynein is retrograde
11
what signaling system is affected with mutate polycystin 1 or 2
Wnt Hedgehog
12
what is hydromyelia
over distension of central canal
13
signs hydromyelia
pain in neck, shoulders usually numb
HA
leg or hand weakness
numbness or loss of sensation in hands and feet
problems with walking
loss of bowel and bladder control
spasticity and paralysis of legs
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What anomalies occur in the posterior fossa
chiari malformation I and II
15
what location of brain is posterior fossa
brainstem and cerebellum
16
what anomalie occurs in the large posterior fossa
dandy walker malformation
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what part of brain herniates with chiari type I
peg of cerebellar tonsil
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signs chiari type I herniation
asymptomatic of neck pain
lower CN palsies
sleep apnea
sudden death
cerebellar ataxia, late onset hydrocephalus, long tract signs
signs syringomyelia
19
syringomyelia assoc with with anomalie
chiari type I
20
major pathologic factor in chiari type I
occipital dysplasia
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is there a NT defect in chiari type I
no
22
what causes the syringomyelia in chiari type I
when the herniation occurs there is obstruction to CSF flow causing dilation of central canal of spinal cord
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cape like distribtuion of pain
chiari type I
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expected MRI findings for chiari type I
herniation of 1 or both cerebellar tonsils below foramen magnum
cervico medullary kinking may be present
absence supratentorial anomalies
4th ventricle in usual loaction
syringomyelia in majority
25
chiari type II characteristics
lumbosacral myelomeningocele
shallow posterior fossa with enlarged foramen magnum and low tentorial insertion, herniation of vermis and tonsils
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common finding in chiari type II
lumbosacral myelomeningocele
hydrocephalus
27
dandy walker malformation
large posterior fossa
no vermis
hydrocephalus
28
how does 4th ventricle appear in dandy walker malformation
semi transparent membrain along margins because the vermis is missing
29
communicating hydrocephalus
hypersecretion CSF
no obstruction
choroid plexus papilloma
30
causes of non communicating or obstructive hydrocephalus
obstruction foramina monro or third ventricle or aqueduct of foramina luschka or defective filtration CSF or fibrosis of subarachnoid space
hydrocephalus ex vacuo
idiopathic external hydrocephalus
31
where is foramina of luschka
out of 4th ventricle
32
what can cause obstruction 3rd ventricle
craniopharyngioma, germ cell tumors
33
hydrocephalus ex vacuo
dilatation of ventricles from loss of brain tissue
alzhemiemrs, huntingtons, MS
34
idiopathic external hydrocephalus
usually resolves without Tx
infants
probably from immaturity of arachnoid villi
35
what can cause obstruction foramina of monro
colloid cysts, tuberous sclerosis
36
what can cause fibrosis subarachnoid space
meningitis, subarachnoid hemorrhage, meningeal dissemination of tumors
37
What is transependymal edema
pressure in ventricles push fluid through ependymal lining into periventricular white matter
38
what area is hit first with hydrocephalus
periventricular white matter, loses myelin and axons
39
Holoprocencephlay
absence of cleavage of forebrain between 4-6 weeks gestation
40
conditions assoc with holoprocencephaly
maternal DM, toxoplasmosis, syphilis, rubella, fetal alcohol syndrome, genetic
41
common chromosomal anomaly with holoprocencephaly
trisomy 13 Patau
42
what anomalies are assoc with agenesis of corpus callosum
arnold chiari (II)
dandy walker syndrome
andermann syndrome
schizencephaly
43
what is andermann syndrome
motor and sensory neuropathy
K Cl cotransporter
44
signs agenesis corpus callosum
asymptomatic
subtle perceptual deficitis
seizures
psychomotor retardation!
45