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

R. Fronczek; G.J. Lammers; R. Balesar; U.A. Unmehopa; D.F. Swaab

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

R. Fronczek, G.J. Lammers, R. Balesar, U.A. Unmehopa, D.F. Swaab

Netherlands Institute for Neuroscience (NIN)

Research output: Contribution to journal/periodical › Article › Scientific › peer-review

Abstract

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.

Original language	English
Pages (from-to)	5466-5470
Journal	The Journal of clinical endocrinology and metabolism
Volume	90
Publication status	Published - 2005

Cite this

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

abstract = "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.",

author = "R. Fronczek and G.J. Lammers and R. Balesar and U.A. Unmehopa and D.F. Swaab",

note = "doi: 10.1210/jc.2005-0296",

year = "2005",

language = "English",

volume = "90",

pages = "5466--5470",

journal = "Journal of Clinical Endocrinology and Metabolism",

issn = "0021-972X",

publisher = "The Endocrine Society",

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TY - JOUR

T1 - The number of hypothalamic hypocretin (orexin) neurons is not affected in Prader-Willi syndrome.

AU - Fronczek, R.

AU - Lammers, G.J.

AU - Balesar, R.

AU - Unmehopa, U.A.

AU - Swaab, D.F.

N1 - doi: 10.1210/jc.2005-0296

PY - 2005

Y1 - 2005

N2 - 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.

AB - 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.

M3 - Article

SN - 0021-972X

VL - 90

SP - 5466

EP - 5470

JO - Journal of Clinical Endocrinology and Metabolism

JF - Journal of Clinical Endocrinology and Metabolism

ER -

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

Abstract

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