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Flashcards in Path VII Deck (45):
1

myelomeningocele

herniation of CNS tissue through vertebral defect

2

common spot for myelomeningocele

lumbosacral

3

Tx myelomeningocele

surgical correction

4

meckel gruber syndrome

ciliopathy

5

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

9

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

14

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

17

what part of brain herniates with chiari type I

peg of cerebellar tonsil

18

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

21

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

23

cape like distribtuion of pain

chiari type I

24

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

26

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

bat wings appearance of corpus callosum

agenesis