Narcolepsy associated with lesions of the diencephalon.
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.
Aldrich
M S
MS
Department of Neurology, University of Michigan Medical Center, Ann Arbor 48109-0316.
Naylor
M W
MW
D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001927:Brain Diseases / Q000150:complications; D002648:Child; D004027:Diencephalon; D005260:Female; D006683:HLA-DQ Antigens; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D008279:Magnetic Resonance Imaging; D009290:Narcolepsy / Q000209:etiology* / Q000276:immunology; D012894:Sleep Stages; D012893:Sleep Wake Disorders / Q000209:etiology; D012895:Sleep, REM; D014057:Tomography, X-Ray Computed
D006683:HLA-DQ Antigens; C051568:HLA-DQ1 antigen; D006684:HLA-DR Antigens