Idiopathic autonomic neuropathy. An important disease in differential diagnosis

Abstract

Two weeks after a mild viral infection a previously healthy 22-year-old woman developed ileus, a severe abnormality of orthostasis, sicca (Sjögren's) syndrome, bilateral miosis and generalized hyphidrosis. Pelvic endoscopy and laparotomy failed to clarify the cause of the mechanical ileus. Neurological examination revealed an isolated abnormality of the autonomic system, involving both sympathetic and parasympathetic components. Schellong's, Schirmer's and the ninhydrin tests were markedly abnormal. There was no heart rate variation on breathing and a post-Valsalva hypotensive blood pressure overshoot. Further tests failed to find a cause of the neuropathy. The diagnosis of idiopathic panautonomic neuropathy (pandysautonomia) was made. The ileus and hypotension were treated symptomatically with neostigmine, cisapride, midodrine and, initially, with enemas, nasogastric tube feeding and parenteral fluids. The patient was free of symptoms at follow-up examination a year later. Idiopathic autonomic neuropathy should be considered in the differential diagnosis of functional abnormalities of the sympathetic and/or parasympathetic nervous system, especially in previously healthy young people, in the presence of orthostatic, unexplained gastrointestinal and hidrotic symptoms.