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Narcolepsy associated with lesions of the diencephalon

From the Departments of Neurology (Dr. Aldrich) and Psychiatry (Dr. Naylor), University of Michigan Medical Center, Ann Arbor, MI.

Although symptomatic narcolepsy, or narcolepsy due to identifiable brain lesions, was once thought to be common, there are few well-documented reported cases since the discovery of the association of REM sleep abnormalities with narcolepsy. Even fewer such reports have been accompanied by human leukocyte antigen (HLA) testing. We report 3 patients who fulfill criteria for symptomatic narcolepsy, 1 with a craniopharyngioma, the 2nd with a hypothalamic syndrome of unknown etiology, and the 3rd with obstructive hydrocephalus and a sarcoid granuloma in the region of the 3rd ventricle. The first 2 were positive for HLA-DR2 while the 3rd was negative for the HLA-DR2 and HLA-DQwl antigens. These findings suggest that diencephalic lesions can be associated with signs and symptoms of narcolepsy that are clinically indistinguishable from those of idiopathic narcolepsy, and that the HLA-DR2 antigen is not required in all cases of symptomatic narcolepsy.

Address correspondence and reprint requests to Dr. Aldrich, Department of Neurology, Taubman Center 1920/0316, University of Michigan Medical Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109–0316.

Presented in abstract form at the Association of Professional Sleep Societies annual meeting, Washington, DC, June 1989.

Received March 23, 1989. Accepted for publication in final form May 15, 1989.

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