Dysautoregulation of the Cerebral Circulation in Primary Systemic Amyloidosis

Summary: 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.