beta-endorphin and Prader-Willi-Syndrome

Children with the Prader-Willi syndrome have severe and often intractable hyperphagia unresponsive to medical or surgical treatment. Although the effect of opioid antagonists on suppressing appetite in humans has been inconsistent, we evaluated the effectiveness of a new opioid antagonist, naltrexone, in suppressing appetite in four obese adolescents with the Prader-Willi syndrome. Data were collected during the double blind oral administration of the drug and placebo for two 7-day periods. No clinical or biochemical toxicity was apparent during the naltrexone period, and measures of attention span, alertness, and mood did not change. Nutrient intake remained excessive during both the drug and placebo periods. Thus, naltrexone was ineffective in suppressing appetite, at least during the short term.

Topics: Administration, Oral; Adolescent; Appetite; Behavior; beta-Endorphin; Double-Blind Method; Emotions; Endorphins; Female; Humans; Male; Naltrexone; Prader-Willi Syndrome; Random Allocation

By means of a specific two-site immunoradiometric assay, we explored the beta-endorphin levels in various brain regions of a patient affected by Prader-Labhart-Willi Syndrome. The rank of the beta-endorphin levels of five cerebral zones (hypothalamus, substantia grisea centralis, pons dorsalis, medulla oblongata dorsalis medialis, thalamus medialis) of the patient was homologous to that of subjects without the syndrome, except for the medulla oblongata dorsalis medialis. In patient with the Prader-Labhart-Willi Syndrome this region had a higher ranking level than in subjects without it. However, a functional meaning cannot be attributed to such difference because the patient of this study did not exhibit neurological disturbances relating to elevated beta-endorphin levels in the medullary region investigated.

Topics: Adult; beta-Endorphin; Brain; Brain Mapping; Death, Sudden; Female; Humans; Immunoradiometric Assay; Prader-Willi Syndrome

No significant difference was found in the range or mean values of ir-beta-endorphin in the plasma of 6 patients with the Prader-Labhart-Willi syndrome compared to 7 of their normal siblings. The hypothesis that some of the symptoms of the P-L-W syndrome are due to excessive opioid activity is not supported by measurement of peripheral levels of ir-beta-endorphin.

Topics: Adolescent; Adult; beta-Endorphin; Child; Endorphins; Female; Humans; Male; Prader-Willi Syndrome; Radioimmunoassay