Path VIII Flashcards
Abnormal neuronal glial proliferation can lead to what malformation
microcephaly or megalocephaly
abnormal neuronal migration can lead to what
periventricular nodular heterotopia
lissencephaly/subcortical band heterotopia
cobblestone Cx/ congenital muscular dystrophy
abnormal cortical organization leads to
polymicrogyria
focal cortical dysplasia
fetal alcohol syndrome affects what layers
ventricular zone and bipolar zone
disrupt myelin and migration of neurons
5 outcomes in malformations of cortical development
1: fewer or more than normal neurons
2: neurons fo not migrate at all from ventricles or half way
3: some neurons reach Cx but man do not
4: neurons overshoot Cx and are in subarachnoid space
5: late stage migration and cx organization disrupted
fewer or excessive normal neurons leads to
micro or megalocephaly
if neurons do not migrate from ventricles what is it called
periventricular heterotopia
nodular– nodules protrude into ventricle
if neurons migrate half way from ventricles it is called
subcortical band heterotopia
if many neurons do not reach Cx
lissencephaly
pachygyria
cobble stone Cx
lissencephaly
smooth brain no gyri
neurons overshoot Cx and go to subarachnoid space
marginal leptomeningeal glioneuronal heterotopia
cobblestone Cx
if late stage migration and cortical organization is disrupted
polymicrogyria
normal adult brain weight
1200-1400 gm
classificaiton for microcephaly
3 standard deviations below mean head circumference or less than 42 cm at full growth
genetic abnormality in lissencephaly
LIS1 mutation
sometimes assoc with microtubule motor proteins or microtubule dynamics
neurlogic disability with polymicrogyria
seizures, sever pyschomotor retardation, spasticity
what can cause polymicrogyria
ischemia, twinning, infections
how many layers of grey matter in polymicrogyria
4 or less
What is Focal Cortical Dysplasia
sporadic developmental malformation of cerebral Cx causing intractable seizures and cognitive impairment
core pathology of focal cortical dysplasia
abnormal cortical cytoarchitecture from loss of normal layering
most frequent path in brain tissure removed in epilepsy surgery in children
focal cortical dysplasia