[A case of "pure" progressive autonomic failure in an elderly male]

A 68-year-old man was admitted to our hospital because of postural hypotension in July 1991. He was also suffering from anhydrosis, urinary disturbances, constipation and impotence. He had not developed signs of Parkinsonism, cerebellar or peripheral neuropathy four years from the onset. Various autonomic function tests showed sympathetic and parasympathetic dysfunction of mainly postganglionic origins. Thus we diagnosed this patient as "pure" progressive autonomic failure ("pure" PAF). "Pure" PAF is a new entity described by Bannister and Oppenheimer in 1982. It shows symptoms of autonomic failure without other neurological disturbances which manifest as Shy-Drager syndrome. Treatment with L-DOPS increased his blood pressure level and attenuated his symptoms due to orthostatic hypotension.