Path VII Flashcards

1
Q

myelomeningocele

A

herniation of CNS tissue through vertebral defect

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2
Q

common spot for myelomeningocele

A

lumbosacral

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3
Q

Tx myelomeningocele

A

surgical correction

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4
Q

meckel gruber syndrome

A

ciliopathy

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5
Q

what type of defect is encephalocele

A

defect in cranial mesodermal development

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6
Q

where do enecephalocele’s herniate

A

through axial mesodermal defect of the skull

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7
Q

most common spot for herniation of encephalocele

A

occipital lobe

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8
Q

cardinal feature of Meckel Gruber syndrome

A

encephalocele

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9
Q

clinical signs meckel gruber syndrome

A

cysts in kidneys
polydactlyly
encephalocele

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10
Q

difference in kinesin and dynein

A

kinesin is anterograde

dynein is retrograde

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11
Q

what signaling system is affected with mutate polycystin 1 or 2

A

Wnt Hedgehog

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12
Q

what is hydromyelia

A

over distension of central canal

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13
Q

signs hydromyelia

A
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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14
Q

What anomalies occur in the posterior fossa

A

chiari malformation I and II

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15
Q

what location of brain is posterior fossa

A

brainstem and cerebellum

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16
Q

what anomalie occurs in the large posterior fossa

A

dandy walker malformation

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17
Q

what part of brain herniates with chiari type I

A

peg of cerebellar tonsil

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18
Q

signs chiari type I herniation

A
asymptomatic of neck pain
lower CN palsies
sleep apnea
sudden death
cerebellar ataxia, late onset hydrocephalus, long tract signs
signs syringomyelia
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19
Q

syringomyelia assoc with with anomalie

A

chiari type I

20
Q

major pathologic factor in chiari type I

A

occipital dysplasia

21
Q

is there a NT defect in chiari type I

A

no

22
Q

what causes the syringomyelia in chiari type I

A

when the herniation occurs there is obstruction to CSF flow causing dilation of central canal of spinal cord

23
Q

cape like distribtuion of pain

A

chiari type I

24
Q

expected MRI findings for chiari type I

A
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
Q

chiari type II characteristics

A

lumbosacral myelomeningocele

shallow posterior fossa with enlarged foramen magnum and low tentorial insertion, herniation of vermis and tonsils

26
Q

common finding in chiari type II

A

lumbosacral myelomeningocele

hydrocephalus

27
Q

dandy walker malformation

A

large posterior fossa
no vermis
hydrocephalus

28
Q

how does 4th ventricle appear in dandy walker malformation

A

semi transparent membrain along margins because the vermis is missing

29
Q

communicating hydrocephalus

A

hypersecretion CSF
no obstruction
choroid plexus papilloma

30
Q

causes of non communicating or obstructive hydrocephalus

A

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
Q

where is foramina of luschka

A

out of 4th ventricle

32
Q

what can cause obstruction 3rd ventricle

A

craniopharyngioma, germ cell tumors

33
Q

hydrocephalus ex vacuo

A

dilatation of ventricles from loss of brain tissue

alzhemiemrs, huntingtons, MS

34
Q

idiopathic external hydrocephalus

A

usually resolves without Tx
infants
probably from immaturity of arachnoid villi

35
Q

what can cause obstruction foramina of monro

A

colloid cysts, tuberous sclerosis

36
Q

what can cause fibrosis subarachnoid space

A

meningitis, subarachnoid hemorrhage, meningeal dissemination of tumors

37
Q

What is transependymal edema

A

pressure in ventricles push fluid through ependymal lining into periventricular white matter

38
Q

what area is hit first with hydrocephalus

A

periventricular white matter, loses myelin and axons

39
Q

Holoprocencephlay

A

absence of cleavage of forebrain between 4-6 weeks gestation

40
Q

conditions assoc with holoprocencephaly

A

maternal DM, toxoplasmosis, syphilis, rubella, fetal alcohol syndrome, genetic

41
Q

common chromosomal anomaly with holoprocencephaly

A

trisomy 13 Patau

42
Q

what anomalies are assoc with agenesis of corpus callosum

A

arnold chiari (II)
dandy walker syndrome
andermann syndrome
schizencephaly

43
Q

what is andermann syndrome

A

motor and sensory neuropathy

K Cl cotransporter

44
Q

signs agenesis corpus callosum

A

asymptomatic
subtle perceptual deficitis
seizures
psychomotor retardation!

45
Q

bat wings appearance of corpus callosum

A

agenesis