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The number of hypothalamic hypocretin (orexin) neurons is not affected in Prader-Willi syndrome.

Onderzoeksgroep Swaab
Publicatiejaar 2005
Gepubliceerd in The Journal of clinical endocrinology and metabolism
Auteur(s) R. Fronczek, G.J. Lammers, R. Balesar, U.A.hopa Unm, D.F. Swaab

CONTEXT: Narcoleptic patients with cataplexy have a general loss of hypocretin (orexin) in the lateral hypothalamus, possibly due to an autoimmune-mediated degeneration of the hypocretin neurons. In addition to excessive daytime sleepiness, Prader-Willi syndrome (PWS) patients may show narcolepsy-like symptoms, such as sleep-onset rapid eye movement sleep and cataplexy, independent of obesity-related sleep disturbances, which suggests a disorder of the hypocretin neurons. OBJECTIVE: We hypothesized that the narcolepsy-like symptoms in PWS are caused by a
decline in the number of hypocretin neurons. DESIGN: We estimated the number of hypocretin neurons in postmortem hypothalami using immunocytochemistry and an image analysis system. SETTING: This study was conducted at the Netherlands Institute for Brain Research. PATIENTS: Eight PWS adults, three PWS infants, and 11 controls were studied. MAIN OUTCOME MEASURE: The total number of hypocretin neurons in the lateral hypothalamus was measured. RESULTS: There was no significant difference in the total number of hypocretin-containing neurons among the seven PWS patients (in whom sufficient hypothalamic material was available to quantify total cell number) and seven age-matched controls, either in adults or in infants. A significant decline with age was found in adult PWS patients (r = -0.9; P = 0.037). CONCLUSIONS: We conclude that a
decrease in the number of hypocretin neurons does not play a major role in the occurrence of narcolepsy-like symptoms in PWS.

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