{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T17:45:13Z","timestamp":1790790313405,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"23","license":[{"start":{"date-parts":[[2008,11,12]],"date-time":"2008-11-12T00:00:00Z","timestamp":1226448000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2008,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Prader\u2013Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, and sleep\u2010related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral and cognitive abnormalities and SRBD have been found. We investigated cognition, behavior, and SRBD in children with PWS. Thirty\u2010one pre\u2010pubertal PWS children were evaluated (5 with paternal deletion, 14 with maternal disomy, 4 with imprinting\u2010center mutation, and in 8 the defect was not specified). Cognition was assessed by Wechsler scale subtests, and behavior by parent\u2013questionnaires. Polysomnography was performed. Cognition, behavior, and associations with SRBD were evaluated. All cognitive subtests were significantly below O SDS, with the lowest median (interquartile range) scores for the Block design subtest (\u22122.7 SDS (\u22123.0 to \u22120.3)). In 60%, verbal subtests were less affected than performance subtests. Parents reported problem behavior related to \u201cemotions\/behavior not adapted to the social situation\u201d and \u201cinsensitivity to social information.\u201d All children had SRBD, with an Apnea Hypopnea Index of 4.1\/hr (2.6\u20137.9). One performance subtest score was significantly higher in children with better sleep efficiency, and daytime sleepiness was associated with more autistic\u2010like social impairment. In contrast to our expectations, behavior was worse in children with better sleep\u2010related breathing. In pre\u2010pubertal PWS children, cognition is impaired. Neurobehavioral abnormalities are common, particularly autistic\u2010like social impairment. Sleep efficiency was associated with better performance on one of the performance subtests, and neurobehavioral abnormalities were associated with daytime sleepiness. In contrast, we could not confirm a positive association of neurobehavioral abnormalities with SRBD in PWS. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.32241","type":"journal-article","created":{"date-parts":[[2008,11,12]],"date-time":"2008-11-12T21:35:50Z","timestamp":1226525750000},"page":"3018-3025","source":"Crossref","is-referenced-by-count":25,"title":["Cognition and behavior in pre\u2010pubertal children with Prader\u2013Willi syndrome and associations with sleep\u2010related breathing disorders"],"prefix":"10.1002","volume":"146A","author":[{"given":"Dederieke A.M.","family":"Festen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maaike","family":"Wevers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Al W.","family":"de Weerd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renilde A.S.","family":"van den Bossche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo J.","family":"Duivenvoorden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita C.S.","family":"Hokken\u2010Koelega","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,11,12]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0997-75"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2869.2002.00289.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)91748-9"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0495-395"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2005-1837"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/014107688908200108"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1021263216093"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2006-0765"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e31809871dd"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-200003000-00006"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.82.2.107"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.05.039"},{"key":"e_1_2_6_14_1","volume-title":"Essentials of WISC\u2010III and WPPSI\u2010R assessment","author":"Kaufman A","year":"2000"},{"key":"e_1_2_6_15_1","volume-title":"Vragenlijst voor Inventarisatie van Sociaal Gedrag van Kinderen, Manual","author":"Luteijn E","year":"2002"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00011204"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2005-1279"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7610.2005.01520.x"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320460106"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10152"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.07.021"},{"key":"e_1_2_6_22_1","volume-title":"A Manual of Standardized Terminology, Techniques, and Scoring System for Sleep Stages of Human Subjects, Publication 204","author":"Rechtschaffen A","year":"1968"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.23-05-01569.2003"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2788.2000.00250.x"},{"key":"e_1_2_6_25_1","first-page":"787","volume-title":"Problems in human assessment","author":"Seashore H","year":"1967"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.2004.00582.x"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.125.3.872"},{"key":"e_1_2_6_28_1","volume-title":"Wechsler Intelligence Scale for Children\u2010Revised (Dutch Version), Manual","author":"van Haassen P","year":"1986"},{"key":"e_1_2_6_29_1","volume-title":"Wechsler Preschool and Primary Scale of Intelligence (Dutch Version), Manual","author":"Vander Steene G","year":"1997"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00787-004-0354-6"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201716"}],"container-title":["American Journal of Medical Genetics Part A"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.a.32241","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.32241","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T04:45:42Z","timestamp":1697604342000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.a.32241"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,11,12]]},"references-count":30,"journal-issue":{"issue":"23","published-print":{"date-parts":[[2008,12]]}},"alternative-id":["10.1002\/ajmg.a.32241"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.a.32241","archive":["Portico"],"relation":{},"ISSN":["1552-4825","1552-4833"],"issn-type":[{"value":"1552-4825","type":"print"},{"value":"1552-4833","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,11,12]]}}}