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NEUROLOGY 1983;33:1122
© 1983 American Academy of Neurology

Episodic hyperhidrosis, hypothermia, and agenesis of corpus callosum

Peter A. LeWitt, MD, Richard P. Newman, MD, Harry S. Greenberg, MD, Leslie L. Rocher, MD, Donald B. Calne, DM, FRCP and Joel R.L. Ehrenkranz, MD

Experimental Therapeutics Branch (Drs. Le Witt and Newman), NINCDS, NIH, Rethesda, MD, the Department of Neurology (Dr. Greenberg) and the Division of Nephrology (Dr. Rocher), University of Michigan, Ann Arbor, MI, the Department of Medicine, (Dr. Ehrenkranz), Columbia University College of Physicians and Surgeons, New York, NY, and the Department of Medicine (Dr. Calne), University of British Columbia, Vancouver, Canada.

Episodic hyperhidrosis and hypothermia are the primary symptoms of a rare central nervous system disorder of thermoregulation which is often associated with agenesis of the corpus callosum and can present in childhood or adult years. During attacks, patients may exhibit confused, withdrawn, and lethargic behavior and ataxia or other neurologic symptoms. A 21-year-old man with temperature chronically between 30 and 32 °C transiently responded to phenobarbital and to cyproheptadine therapy. A 34-year-old woman with frequent, brief episodes of hypothermia and hyperhidrosis improved with chlorpromazine treatment. Episodic thermoregulatory disturbance has been atttributed to "vagal attacks" or "diencephalic epilepsy," but the pathophysiology remains undefined.

Address correspondence and reprint requests to Dr. LeWitt, Building 10, Room 5C106, Experimental Therapeutics Branch, National Institute of Neurological & Communicative Disorders & Stroke, National Institutes of Health, Bethesda, MD 20205.

Accepted for publication November 1, 1982.




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