CSF hypocretin-1 (orexin-A) concentrations in narcolepsy with and without cataplexy and idiopathic hypersomnia
Abstract:We measured cerebrospinal fluid (CSF) hypocretin-1 levels in 11 patients with narcolepsy–cataplexy, five with narcolepsy without cataplexy and 12 with idiopathic hypersomnia (IHS). All patients were Japanese. As reported in Caucasian patients, undetectable or very low hypocretin-1 levels were observed in most (9 out of 11) Japanese narcolepsy–cataplexy patients. Our hypocretin-deficient narcoleptics included three prepubertal cases within few months after the disease onset. All nine hypocretin-deficient patients were human leuckocyte antigen (HLA) DR2 positive, while two who had normal CSF hypocretin-1 levels were HLA DR2 negative. In contrast, none of the narcolepsy without cataplexy and IHS subjects had undetectable low levels. Low CSF hypocretin-1 is therefore very specific for HLA DR2 positive narcolepsy–cataplexy, and the deficiency is likely to be established at the early stage of the disease.
Document Type: Original Article
Affiliations: 1: Department of Neuropsychiatry, Akita University School of Medicine, Akita, Japan, 2: Department of Neuropsychiatry, Juntendo University School of Medicine, Tokyo, Japan, 3: Department of Neuropsychiatry, Asahikawa Medical College, Asahikawa, Japan, 4: Department of Pediatrics, Nagoya City University Medical School, Nagoya, Japan, 5: Department of Pediatric Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan, 6: Center for Narcolepsy, Stanford University School of Medicine, CA, USA
Publication date: 2002-03-01