Acute idiopathic dysautonomia: electrophysiology and response to intravenous immunoglobulin

Acute idiopathic dysautonomia is an uncommon syndrome consisting of varying degrees of sympathetic and parasympathetic dysfunction. The etiology and potential treatments for the disorder are uncertain.1 Treatment with IV immunoglobulin (IVIg) has been reported in only two patients.2,3 We describe the results of electrophysiologic and autonomic testing and response to IVIg of a patient with acute idiopathic dysautonomia. A 61-year-old man was evaluated for severe lightheadedness and syncope with standing. Two weeks earlier, he had developed acute, lancinating pains in his arms and legs, difficulty urinating, erectile dysfunction, constipation, fever, and orthostatic hypotension with syncope. He had no visual blurring, changes in sweating, or weakness. He received IV fluid at that time without benefit. The limb pain disappeared spontaneously over several days. On initial evaluation, he was afebrile. Supine blood pressure was 150/90 mmHg with a heart rate of 60 beats/minute. Blood pressure seated was 130/85 mmHg; blood pressure standing fell to 85/50 mmHg with an associated heart rate of 68 beats/minute. He was severely symptomatic when standing. Neurologic examination showed no evidence of …