Narcolepsy and low CSF orexin (hypocretin) concentration after a diencephalic stroke.
Idiopathic narcolepsy usually results from a loss of the hypothalamic neuropeptide orexin (hypocretin), but the cause of secondary narcolepsy resulting from focal brain lesions is unknown. The authors describe a young man who developed narcolepsy after a large hypothalamic stroke. His lesion included much of the hypothalamic region in which orexin is produced, and his CSF concentration of orexin was low. The authors hypothesize that a loss of orexin neurons or their relevant targets may be the specific neuropathology causing this and many other cases of secondary narcolepsy.
Scammell
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Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA 02115, USA. tscammel@caregroup.harvard.edu
Nishino
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Mignot
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Saper
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D000328:Adult; D002352:Carrier Proteins / Q000134:cerebrospinal fluid*; D004027:Diencephalon / Q000473:pathology / Q000503:physiopathology*; D004569:Electroencephalography; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008279:Magnetic Resonance Imaging; D008297:Male; D009290:Narcolepsy / Q000134:cerebrospinal fluid* / Q000473:pathology / Q000503:physiopathology*; D009479:Neuropeptides / Q000134:cerebrospinal fluid*; D000068797:Orexins; D020521:Stroke / Q000134:cerebrospinal fluid* / Q000473:pathology / Q000503:physiopathology
D002352:Carrier Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins