Nonarteriosclerotic Diseases of Cerebral Arteries

This group encompasses vascular diseases of varied etiologies, including inflammatory and. noninflammatory vasculopathies, hereditary arteriopathies, malformations, and mineralization related to aging.

The diagnosis of these diseases often presents difficulties; early diagnosis, however, is important because appropriate therapy may halt or even reverse the disease process. The clinical presentation varies across a broad spectrum. The disease may begin acutely as a TIA or a full blown stroke, or it may progress gradually with headaches, multifocal neurologic signs, seizures, behavioral changes, psychosis, and cognitive decline often progressing to dementia. Several diseases also affect the systemic blood vessels and produce visceral and cutaneous changes. Segmental constriction (beading) of the arterial wall, as seen on angiogram, is characteristic for some; for others, the definite diagnosis may require tissue biopsy. The cerebral pathology also ranges widely: Some angiopathies have a predilection for the large, and some for the small vessels; infarctions and hemorrhages may be solitary or multiple, small or large.

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