Dysautoregulation of the cerebral circulation in primary systemic amyloidosis.
A case of primary systemic amyloidosis associated with marked dysautoregulation of the cerebral circulation in response to hypotension is reported. The major amyloid protein was immunoglobulin A lambda type. An autonomic nerve function test showed severe and extensive disturbances of the autonomic nervous system. The cerebrovascular reactivity to 5% CO2 inhalation was spared. To our knowledge, this is the first case to demonstrate that amyloid peripheral autonomic neuropathy can be accompanied by dysautoregulation of the cerebral circulation.
D000686:Amyloidosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D001341:Autonomic Nervous System / Q000503:physiopathology; D002560:Cerebrovascular Circulation; D002561:Cerebrovascular Disorders / Q000209:etiology* / Q000503:physiopathology; D006706:Homeostasis; D006801:Humans; D008297:Male; D008875:Middle Aged