Pure progressive autonomic failure: a clinicopathological study.
Two cases of pure progressive autonomic failure (PAF) are presented. A postmortem study of one case (case 2) showed a pathology resembling that of Parkinson's disease. Marked cell loss was noted in the substantia nigra, nucleus ceruleus, and intermediolateral column of the spinal cord, while cell loss in the sympathetic ganglion was not remarkable. This case may be an exceptionally rare case of late-onset PAF in which autonomic failure was mainly ascribed to preganglionic (and central) pathology, although autonomic function tests suggested postganglionic sympathetic disorder in both cases.
Terao
Y
Y
Department of Neurology, Japanese Red Cross Medical Center, Tokyo.
Takeda
K
K
Sakuta
M
M
Nemoto
T
T
Takemura
T
T
Kawai
M
M
D000368:Aged; D000369:Aged, 80 and over; D001338:Autonomic Fibers, Postganglionic / Q000473:pathology / Q000503:physiopathology; D001339:Autonomic Fibers, Preganglionic / Q000473:pathology / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D001706:Biopsy; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D005728:Ganglia, Sympathetic / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D009410:Nerve Degeneration / Q000502:physiology; D009460:Neurologic Examination; D010300:Parkinson Disease / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology