Predictors of Hypocretin Deficiency in Narcolepsy without Cataplexy
نویسندگان
چکیده
1247 Hypocretin Deficiency in Narcolepsy Without Cataplexy—Andlauer et al INTRODUCTION Narcolepsy without cataplexy is a complex, heterogeneous disorder.1,2 Until recently, most narcolepsy studies have focused on narcolepsy with cataplexy, an etiologically homogenous disorder tightly associated with hypocretin deficiency and HLADQB1*06:02 positivity.3,4 Hypocretin deficiency can be tested by measuring cerebrospinal fluid (CSF) concentrations of hypocretin-1, one third of normal values or 110 pg/ml being the most optimal cutoff based on receiver operating characteristics curve PREDICTORS OF HYPOCRETIN DEFICIENCY IN NARCOLEPSY WITHOUT CATAPLEXY
منابع مشابه
Monozygotic twins concordant for narcolepsy-cataplexy without any detectable abnormality in the hypocretin (orexin) pathway.
Narcolepsy with cataplexy is thought to be a hypocretin ligand or hypocretin receptor deficiency syndrome caused by genetic and environmental factors. We looked for an abnormality of the hypocretin pathway in HLA-DQB1*0602-positive monozygotic twins who were concordant for narcolepsy-cataplexy. They had normal cerebrospinal fluid concentrations of hypocretin-1, and we found no mutation in the p...
متن کاملNarcolepsy without cataplexy: 2 subtypes based on CSF hypocretin-1/orexin-A findings.
STUDY OBJECTIVES Cerebrospinal fluid (CSF) hypocretin-1 levels and their relationship with the clinical characteristics of narcolepsy without cataplexy have not been well elucidated. Our aim was to examine whether clinical characteristics vary with CSF hypocretin-1 levels among narcoleptic patients without cataplexy. DESIGN Clinical features, variables on the multiple sleep latency test, and ...
متن کاملIncreased Immune Complexes of Hypocretin Autoantibodies in Narcolepsy
BACKGROUND Hypocretin peptides participate in the regulation of sleep-wake cycle while deficiency in hypocretin signaling and loss of hypocretin neurons are causative for narcolepsy-cataplexy. However, the mechanism responsible for alteration of the hypocretin system in narcolepsy-cataplexy and its relevance to other central hypersomnias remain unknown. Here we studied whether central hypersomn...
متن کاملCSF hypocretin-1 (orexin-A) concentrations in narcolepsy with and without cataplexy and idiopathic hypersomnia.
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 narcol...
متن کاملHypocretin deficiency develops during onset of human narcolepsy with cataplexy.
STUDY OBJECTIVES Although hypothesized through animal studies, a temporal and causal association between hypocretin deficiency and the onset of narcolepsy with cataplexy (NC) has never been proven in humans. SETTING Paediatric Department, Blekinge Hospital, Sweden, and Danish Center for Sleep Medicine, Glostrup Hospital, Denmark. PATIENT AND RESULTS Two weeks after his second Pandemrix-vacc...
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