Microvasculopathy is associated with the number of cerebrovascular lesions in hereditary cerebral hemorrhage with amyloidosis, Dutch type

• Published in: Stroke (1970) 1998-08, Vol.29 (8), p.1588-1594
• Main Authors: NATTE, R, VINTERS, H. V, MAAT-SCHIEMAN, M. L. C, BORNEBROEK, M, HAAN, J, ROOS, R. A. C, VAN DUINEN, S. G
• Format: Article
• Language: English
• Subjects: Age Factors; Aged; Aged, 80 and over; Arteriosclerosis - complications; Arteriosclerosis - pathology; Biological and medical sciences; Brain - blood supply; Capillaries - pathology; Cerebral Amyloid Angiopathy - genetics; Cerebral Amyloid Angiopathy - mortality; Cerebral Amyloid Angiopathy - pathology; Cerebral Hemorrhage - genetics; Cerebral Hemorrhage - mortality; Cerebral Hemorrhage - pathology; Cerebrovascular Disorders - etiology; Cerebrovascular Disorders - genetics; Cerebrovascular Disorders - pathology; Female; Humans; Hypertension - complications; Hypertension - pathology; Intracranial Aneurysm - complications; Intracranial Aneurysm - pathology; Male; Medical sciences; Microcirculation; Middle Aged; Neurology; Vascular diseases and vascular malformations of the nervous system
• ISSN: 0039-2499; 1524-4628
• DOI: 10.1161/01.STR.29.8.1588

Microvascular changes such as microaneurysms and fibrinoid necrosis have been found in the presence of cerebral amyloid angiopathy (CAA). These CAA-associated microvasculopathies (CAA-AM) may contribute to the development of CAA-associated hemorrhages and/or infarcts, hereafter referred to as "cerebrovascular lesions." Hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D) is an autosomal dominant form of CAA, in which the amyloid angiopathy is pathologically and biochemically similar to sporadic CAA associated with aging and Alzheimer disease. To determine the significance of CAA-AM for CAA-associated cerebrovascular complications, we investigated the association between CAA-AM and cerebrovascular lesions in HCHWA-D patients. In a previous autopsy study we semiquantitatively scored CAA-AM in 29 HCHWA-D patients. In the present study we reviewed clinical charts and autopsy protocols of these same patients. We investigated whether CAA-AM was associated with age at death, number of cerebrovascular lesions, duration of clinical illness, hypertension, and atherosclerosis. An association was found between CAA-AM and the number of cerebrovascular lesions (P = 0.009). The presence of microaneurysmal degeneration was most strongly associated with the number of cerebrovascular lesions (P < 0.001). In addition, we found an association between atherosclerosis and the CAA-AM score (P = 0.047). Hypertension was not associated with CAA-AM. Our findings support previous reports suggesting an important role of secondary microvascular degenerative changes in CAA-associated cerebrovascular lesions and suggest an aggravating effect of systemic atherosclerosis, but not hypertension, on the evolution of CAA-AM. These findings may be of relevance to understanding cerebrovascular complications of sporadic or Alzheimer disease-associated CAA.