Fronczek, R. and Lammers, G.J. and Balesar, R. and Unm, U.A.hopa and Swaab, D.F. (2005) The number of hypothalamic hypocretin (orexin) neurons is not affected in Prader-Willi syndrome. The Journal of clinical endocrinology and metabolism, 90(9), 5466-5470. ISSN 0021972X. doi: 10.1210/jc.2005-0296.
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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.
|Additional Information:||doi: 10.1210/jc.2005-0296|
|Institutes:||Netherlands Institute for Neuroscience (NIN)|
|Deposited On:||13 Feb 2009 17:14|
|Last Modified:||10 Dec 2009 13:14|
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