Idiopathic autonomic neuropathy, an important disease in differential diagnosis

History and clinical findings: Two weeks after a mild viral infection a pre viously healthy ZZ-year-old woman developed ileus, a severe abnormality of orthostasis, sicca (Sjogren's) syndrome, bilateral miosis and generalized h yphidrosis. Pelvic endoscopy and laparotomy failed to clarify the cause of the mechanical ileus. Investigations: Neurological examination revealed an isolated abnormality o f the autonomic system, involving both sympathetic and parasympathetic comp onents. Schellong's, Schirmer's and the ninhydrin tests were markedly abnor mal. There was no heart rate variation on breathing and a post-Valsalva hyp otensive blood pressure overshoot. Further tests failed to find a cause of the neuropathy. Diagnosis, treatment and course: The diagnosis of idiopathic panautonomic n europathy (pandysautonomia) was made. The ileus and hypotension were treate d symptomatically with neostigmine, cisapride, midodrine and, initially, wi th enemas, nasogastric tube feeding and parenteral fluids. The patient was free of symptoms at follow-up examination a year later. Conclusions: Idiopathic autonomic neuropathy should be considered in the di fferential diagnosis of functional abnormalities of the sympathetic and/or parasympathetic nervous system, especially in previously healthy young peop le, in the presence of orthostatic, unexplained gastrointerstinal and hidro tic symptoms.