Prader—Willi syndrome and the hypothalamus
Open accessAbstract
Dysfunction of various hypothalamic systems may be the basis of a number of symptoms in Prader—Willi syndrome. The often abnormal position of the baby in the uterus at the onset of labour, the high percentage of infants with asphyxia and the high proportion of children born prematurely or post‐maturely may all be related to abnormal fetal hypothalamic systems, as the fetal hypothalamus plays a crucial role in labour. Abnormal luteinizing hormone‐releasing hormone neurones are thought to be responsible for the decreased levels of sex hormones, resulting in non‐descended testes, undersized sex organs and insufficient growth during puberty. A lack of growth hormone‐releasing hormone may also contribute to the short stature of patients with Prader—Willi syndrome. In addition, the aberrant control of body temperature and daytime hypersomnolence may result from hypothalamic disturbances. The number of oxytocin neurones – the putative satiety neurones – in the hypothalamic paraventricular nucleus is markedly decreased in Prader—Willi syndrome. This is presumed to be the basis of the insatiable hunger and obesity of patients with the syndrome. □ Growth hormone, growth hormone‐releasing hormone, hypothalamus, insatiable hunger, obesity, oxytocin, perinatal problems, Prader—Willi syndrome
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Appetite disturbance in PWS has been attributed to the hypothalamic dysfunction that characterizes the disorder, which also causes growth-hormone deficiency, hypogonadism, and temperature dysregulation.
“Appetite disturbance in PWS has been attributed to the hypothalamic dysfunction that characterizes the disorder, which also causes growth-hormone deficiency, hypogonadism, and temperature dysregulation [677].”
From Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings (2012) · cites this paper as [677]
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- Authors
- Swaab, D.
- Venue
- Acta Paediatrica
- Publisher
- Wiley
- Published
- 1997-11
- Volume
- 86
- Issue
- S423
- Pages
- 50-54
- ISSN
- 0365-1436 (ISSN-L)
- 0803-5253
- 1651-2227
- OpenAlex
- W2062940073
- Cited by
- 10
- References
- 26
- Citations by year
- 2023: 1 · 2022: 3 · 2021: 1 · 2019: 3 · 2017: 1
- FWCI
- 0.00
- Type
- journal article
- Language
- en
- Open access
- green
- pure.knaw.nl
- Licence
- Locations
- Acta Paediatrica (publishedVersion)
- PubMed (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- KNAW Research Portal (The Royal Netherlands Academy of Arts and Sciences) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic Syndromes and Imprinting
- Life Sciences › Neuroscience › Endocrine and Autonomic Systems › Regulation of Appetite and Obesity
- Health Sciences › Medicine › Reproductive Medicine › Hypothalamic control of reproductive hormones
- Keywords
- Prader-Willi syndrome, hypothalamus, luteinizing hormone-releasing hormone neurons, gonadal dysgenesis, short stature, growth hormone-releasing hormone, thermoregulation, excessive daytime sleepiness, oxytocin neurons, paraventricular nucleus, satiety, obesity, fetal asphyxia, preterm birth
- MeSH terms
- Humans, Hypothalamus, Gonadotropin-Releasing Hormone, Prader-Willi Syndrome, Growth Hormone-Releasing Hormone
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