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Case Reports
. 2013 Jan 1;36(1):147-8.
doi: 10.5665/sleep.2320.

Hypocretin deficiency develops during onset of human narcolepsy with cataplexy

Andri Savvidou  1 , Stine Knudsen, Mia Olsson-Engman, Steen Gammeltoft, Poul Jennum, Lars Palm
Affiliations
Case Reports

Hypocretin deficiency develops during onset of human narcolepsy with cataplexy

Andri Savvidou et al. Sleep. .

Abstract

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-vaccination, a 10 year old HLA-DQB1*0602-positive boy developed NC. The CSF hypocretin-1 level was 10 pg/ml. However, CSF saved from a pre-narcolepsy episode of Lyme disease revealed a normal hypocretin-1 level (318 pg/ml).

Conclusions: We confirm that hypocretin deficiency develops in parallel to the onset of human narcolepsy with cataplexy.

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