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Malformation of Nervous Systems
Neuro Module
24
Medical
Graduate
11/15/2009

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Cards

Term
What is dysraphism with regards to the neural tube?
Definition

incomplete closure

 

Term
What is the most common neural tube abnormality?
Definition
Spina Bifida
Term
What type of spina bifida is an outpouching of just subarachnoid and arachnoid?
Definition
meningocele
Term
What is myeningomyelocele?
Definition
both arachnoid and spinal cord outside of spinal canal
Term
What should one be concered about with a congential dural sinus?
Definition
infection
Term
What is encephalocele?
Definition
the spina bifida equivalent of the brain in which the brain is enclosed in a sac outside the skull
Term
What is it called when the brain does not completely form and is completely exposed?
Definition
anencephaly
Term
What is the name for the pathologic changes seen in anencephaly in which there is a red area with disorganized tissue?
Definition
area cerebrovasculosa
Term
What is the name given to the deformitiy in which the prosencephalon does not divide into two cerebral cortex?
Definition
holoprosepncephaly
Term
What trisomies is holoprosencephalon associated with?
Definition
trisomy 13 and 18
Term
What other malformations are common in holoprosencephaly?
Definition
facial defects
Term
What is proencephaly?
Definition
cyst formation due to lack of brain tissue
Term
What is an abnormal forebrain formation defect that resembles hydrocephalus ex vacuo with failure of formation of brain tissue resulting in hydrocephalus?
Definition
hydraencephaly
Term
What is lissencephaly?
Definition
A lack of gyri and sulci creating a smooth like surface of the brain
Term
What protein is lissencephaly associated with?
Definition
Microtubule assocated protein LIS-1
Term
What malformation of the nervous system is assocated with microtubule assocated protein LIS1?
Definition
lissencephaly
Term
What is the malformation described by small folds in surface of brain that resemble little gyri?
Definition
polymicrogyria
Term
What is heterotropic cortex?
Definition
cortex formed in the wrong place due to improper migration
Term
What four defects are assocated with Arnold-Chiari SYndrome?
Definition

elongation of 4th ventricle

elongation of medulla

flattening and beaked colliculi (quadtrigeminal plate)

herniation of the vermis of the cerebellum

Term
What is Arnold-Chiari often assocated with? (2)
Definition
spina bifida and hydrocephalus
Term
What two characteritics make up the Dandy-Walker malformation?
Definition

no vermis

expanded cystic 4th ventricle

Term
What is syringomyelia?
Definition
a cavity in the spinal cord
Term
What is the name given to a cavity in the spinal cord?
Definition
syringomyelia
Term
What is cerebral palsy?
Definition
a termed used to describe neurologic deficiet soon after birth that does NOT get worse
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