[The Shy-Drager syndrome].

For 15 years a now 70-year-old woman had been having occasional episodes of circulatory collapse which 7 years ago were diagnosed as being caused by severe idiopathic orthostatic hypotension. These episodes had recently become much more frequent and she was hardly able to be upright for more than one minute. In the Schellong test the blood pressure fell from 80/50 mm Hg when lying to 70/30 mm Hg on standing, the pulse rate remaining unchanged at 60/min. The standing test had to be abandoned after 90 seconds. Serum catecholamine concentrations (epinephrine 65 ng/l, norepinephrine 100 ng/l) did not rise on standing (epinephrine 25 ng/l, norepinephrine 105 ng/l). 24-hour urinary excretion of vanillyl mandelic acid was at the lower limit of normal (2.2 mg). The autonomic dysfunction of circulatory control suggested a Shy-Drager syndrome. Other signs of autonomic failure included gastroparesis, decreased tear and sweat secretion and transitory urinary incontinence. Symptomatic treatment with elastic stockings, fludrocortisone, etilefrine, dihydroergotamine, L-dopa, yohimbine and amezinium methylsulfate gave the patient greater mobility without achieving normal blood pressure responses.