IDIOPATHIC DYSAUTONOMIA TREATED WITH INTRAVENOUS GAMMA-GLOBULIN

Background A previously healthy 23-year-old man presented with a short history of abdominal pain and diarrhoea followed by blurred vision, s evere postural hypotension, reduced sweating and unremitting fever. Me thods Examination revealed fixed dilated pupils, impaired sweating and postural hypotension. Clinical and neurophysiological examination sho wed no motor or sensory deficit. A diagnosis of idiopathic autonomic n europathy was made. He became gravely ill with profound life-threateni ng hypotension and a prolonged ileus. Findings Within 36 h of receivin g intravenous gammaglobulin (IVGG) his pupillary areflexia and severe hypotension resolved. 2 weeks later the autonomic failure recurred but again responded to treatment with IVGG. IVGG is a recognised treatmen t for Guillain-Barre syndrome. Interpretation This case report demonst rates that IVGG is also effective in the rare pure dysautonomic varian t.