Undetectable levels of CSF hypocretin-1 (orexin-A) in two prepubertal boys with narcolepsy

Neuropediatrics
H TsukamotoT Kanbayashi

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.

Citations

May 17, 2005·Brain Research. Molecular Brain Research·Vânia D'AlmeidaJosé N Nobrega
Nov 1, 2003·Sleep Medicine·Seiji Nishino, Emmanuel Mignot
Oct 10, 2003·Current Opinion in Pulmonary Medicine·Ann C Halbower, Carole L Marcus
Nov 25, 2004·Annals of Neurology·Yves DauvilliersMehdi Tafti
Jan 21, 2009·Sleep Medicine Reviews·Sona Nevsimalova
May 2, 2007·Sleep Medicine·Seiji Nishino
Jul 22, 2014·Medicinal Research Reviews·Quanhui ChenDong Gao

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