The clinical spectrum of cerebral amyloid angiopathy

Cerebral amyloid angiopathy is a common cause of spontaneous lobar hemorrhage in elderly patients. We discuss seven patients with amyloid angiopathy presenting without major lobar hemorrhage. The patients' presentations fell into two groups: recurrent transient neurologic symptoms and rapidly progressive dementia. The cases with transient episodes had a spread of symptoms to contiguous body areas during episodes. Each had evidence of small hemorrhage or subsequent large hemorrhage in the cortical location corresponding to the symptoms, suggesting petechial hemorrhage with focal seizure as the cause of the transient spells. Three cases of dementia developed with relatively rapid time courses, progressing from intact baseline to profound dementia in spans of a few days to 2 years. Pathologic abnormalities, in addition to amyloid angiopathy, included patchy white matter demyelination and tissue loss, petechial cortical hemorrhages, cortical infarctions, and a variable degree of neuritic plaques and neurofibrillary tangles. The clinical spectrum of cerebral amyloid angiopathy includes these two neurologic syndromes.