[Autonomic nerve disorders in generalized amyloidosis].
Various autonomic disturbances are usually seen in systemic amyloidosis with polyneuropathy, especially in familial amyloid polyneuropathy (FAP). In this paper we summarized the clinicopathological features of these autonomic symptoms. Orthostatic hypotension and bowel dysfunctions are two major autonomic manifestations of FAP, and at autopsy severe deposition of amyloid is observed in an extensive area of peripheral autonomic nervous system including sympathetic ganglia. Remarkable depletion of the extrinsic nerves with relative preservation of the intrinsic nerves is a characteristic finding in the gastrointestinal tract of the patients with FAP, and this abnormal innervation may produce the peculiar bowel disorders. Oral administration of L-threo-3,4-dihydroxyphenylserine, a precursor of noradrenaline, is effective for the treatment of these autonomic symptoms of FAP patients.
Yazawa
M
M
Department of Medicine (Neurology), Shinshu University School of Medicine.
Ikeda
S
S
D000328:Adult; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D001342:Autonomic Nervous System Diseases / Q000209:etiology*; D005767:Gastrointestinal Diseases / Q000209:etiology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000209:etiology; D008875:Middle Aged