Microvascular degeneration in hereditary cystatin C amyloid angiopathy of the brain.

[hereditary cerebral hemorrhage with amyloidosis]

Hereditary cystatin C amyloid angiopathy (HCCAA ), an autosomal dominant form of cerebral amyloid angiopathy (CAA ) occurring primarily in Iceland , is characterized by a variant cystatin C amyloid deposition in the walls of cerebral parenchymal and leptomeningeal vessels . Cystatin C is also found to colocalize with amyloid beta /A 4 protein in cerebral vessel walls of patients with Alzheimer 's disease (AD ), sporadic CAA , and hereditary cerebral hemorrhage with amyloidosis , Dutch type (HCHWA-D ). The abundance of cystatin C deposition in cerebral blood vessel walls suggests that cellular elements of the vessel wall itself may play a role in its deposition . Microvascular changes in the brains of HCCAA patients were investigated by single - and double -label immunohistochemistry . We found that cystatin C amyloid immunoreactivity was present not only in cerebral cortical and leptomeningeal vessels , but also in white matter parenchymal vessels . Cystatin C deposition was more prominent in the media of parenchymal vessels and in the adventitia of leptomeningeal vessels . Smooth muscle (sm ) cells were few or could not be identified within vessel walls showing extensive cystatin C deposition , suggesting progressive loss of these cells as cystatin C accumulates . However , in less severely affected vessels , cystatin C was present in cells that also had the phenotype of sm , suggesting that sm cells synthesize or process cystatin C . Cystatin C immunoreactivity was in addition , detected in some neuronal cell bodies throughout the cortex in patients with HCCAA and AD -related CAA . Our results indicate that cellular components of the vessel walls may play an important role in cystatin C deposition , as they do in beta /A 4 deposition in AD -related CAA . Cystatin C deposition within the vascular media and adventitia , with associated vessel wall injury as manifested by sm cell loss , represents microvascular degeneration that leads to cerebral hemorrhage .

Diseases
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Diseases presenting "cystatin c was present in cells that also had the phenotype of sm" symptom

hereditary cerebral hemorrhage with amyloidosis

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