White matter stuff Flashcards
31 yoF
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)
lacunar and subcortical white-matter strokes and vascular dementia in younger patients.
It is an autosomal dominant disorder (NOTCH3 gene).
CADASIL inheritance
It is an autosomal dominant disorder (NOTCH3 gene).
mutation results in stenosis of small to medium perforating vessels and causes fibrotic thickening of the basement membranes with deposition of granular osmiophilic material around vascular smooth muscles.
CADASIL clinical picture
Symptoms first occur in the third and fourth decades and typically include recurrent transient ischemic attacks (TIA) and strokes across multiple vascular territories.
Alexander disease
- confluent bilateral cerebral white matter signal(anterior slightly greater than posterior),
- periventricular, subcortical, and deep white matter.
- T2/(FLAIR) hyperintensity within the bilateral basal ganglia and thalami.
metachromatic leukodystrophy
tigroid
adrenoleukodystrophy
macrocephaly
Canavan
adrenoleukodystrophy