Abstract
We report on two prepubertal narcoleptic boys with undetectable levels of hypocretin-1 (orexin-A) in their cerebrospinal fluid (CSF). The disease onset times were 6 and 8 years, and CSF was collected 8 and 20 months after the onset, respectively. The initial symptoms were excessive daytime sleepiness, cataplexy and disrupted nocturnal sleep. Both subjects are DRB1*1501 and DQB1*0602 positive. The measurement of CSF hypocretin-1 is valuable for the decisive diagnosis of narcolepsy and for selecting the type of treatment in prepubertal children. Our results suggest that a significant degree of hypocretin deficiency is already present at the disease onset.
Key words
Narcolepsy - Cataplexy - Hypocretin (Orexin) - Human leukocyte antigen - Child - Sleep disorder
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M.D. Haruko Tsukamoto
Department of Pediatrics, Nagoya City University Medical School
Kawasumi, Mizuho-cho, Mizuho-ku
Nagoya 467 - 8601
Japan
Email: Harukot@aol.com