Cerebral autosomal dominant arteriopathy with subcortical infarcts and lecukoencephalopathy
- VernacularTitle:伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病
- Author:
Yongzhen PEI
;
Gelin XU
;
Xinfeng LIU
- Publication Type:Journal Article
- Keywords:
Cerebrovascular disease;
Subcortical infarction;
Leukoencephalopathy;
Notch 3 gene
- From:
Journal of Medical Postgraduates
2003;0(09):-
- CountryChina
- Language:Chinese
-
Abstract:
Cerebral autosomal dominant arteriopathy with subcortical infarcts and lecukoencephalopathy (CADASIL) is a recently described neurovascular disease affecting young to middle aged individuals. The disease is caused by mutations in the Notch3 gene located in the long arm of chromosome 19. The disease clinically is characterized by migrainous headaches ,mood disturbances, focal neurologic deficits, transient ischemic attaches, strokes and dementia. Pathologically, the disease is characterized by a stereotypic degeneration of the arterial walls with deposition in the media of a nonatheromatous,(nonamyloidotic) substance that under the electron microscope (EM) appears as a granular osmiophilic material (GOM). A review of current literature is presented concerning the clinical, radiological, pathologic and genetic feature of CADASIL.
