{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:26:27Z","timestamp":1786976787359,"version":"build-2736575974"},"reference-count":51,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,12,2]],"date-time":"2004-12-02T00:00:00Z","timestamp":1101945600000},"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":[[2005,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Angelman syndrome (AS) is a rare congenital disorder characterized by impairments in intellectual, neurological and motor functioning and a postulated behavioral profile. This study compared behavioral characteristics of 62 individuals with genetically confirmed AS and 29 individuals with presumed AS from clinical features, with a control group of young persons with intellectual disability (ID) derived from an Australian epidemiological register. Twelve behavioral items from the developmental behavior checklist (DBC) were used for this comparison. The groups were matched for chronological age, gender, and level of ID. In the AS group, significant differences were found for 10 behaviors, with poor attention span and impulsivity being less common, and overactivity\/restlessness, chewing or mouthing objects, eating non\u2010food items, gorging food, food fads, fascination for water, hand flapping and sleep disturbance being more common. Interestingly, there was no difference in prevalence of unprovoked laughter. Comparison of the results of the genetically confirmed with the genetically unconfirmed AS cases showed no significant differences between individual behavior prevalence. These findings show that a \u201cbehavioral phenotype\u201d of AS can be distinguished from others of similar level of ID, but it is different from that hitherto published. Abnormal food related behaviors, hyperactivity, fascination for water, hand flapping, and sleep disturbance should be included in a \u201cbehavioral phenotype\u201d for AS. Apart from hyperactivity, \u201cADHD\u2010type\u201d behaviors are not more characteristic of AS than in ID generally. Therefore, the Consensus Criteria for the diagnosis of AS need to be reviewed. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.30154","type":"journal-article","created":{"date-parts":[[2004,12,2]],"date-time":"2004-12-02T18:48:36Z","timestamp":1102013316000},"page":"8-12","source":"Crossref","is-referenced-by-count":75,"title":["Behavioral aspects of Angelman syndrome: A case control study"],"prefix":"10.1002","volume":"132A","author":[{"given":"Raymond J.","family":"Berry","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Leitner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam R.","family":"Clarke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart L.","family":"Einfeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,12,2]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"485","article-title":"The aberrant behavior checklist: A behavior rating scale for the assessment of treatment effects","volume":"89","author":"Aman MG","year":"1985","journal-title":"Am J Ment Defic"},{"key":"e_1_2_6_3_1","first-page":"492","article-title":"Psychometric characteristics of the aberrant behavior checklist","volume":"89","author":"Aman M","year":"1985","journal-title":"Am J Ment Defic"},{"key":"e_1_2_6_4_1","first-page":"283","article-title":"The aberrant behavior checklist\u2010community: Factor validity and effect of subject variables for adults in group homes","volume":"100","author":"Aman MJ","year":"1995","journal-title":"Am J Ment Retard"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1965.tb07844.x"},{"key":"e_1_2_6_6_1","first-page":"209","article-title":"Cross validation of the factor structure of the aberrant behavior checklist for persons with mental retardation","volume":"96","author":"Bihm EM","year":"1991","journal-title":"Am J Ment Retard"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320510109"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19981204)80:4<385::AID-AJMG15>3.0.CO;2-9"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320560213"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19961211)66:2<221::AID-AJMG19>3.0.CO;2-V"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1352\/0895-8017(2000)105<0025:PBAWQA>2.0.CO;2"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.67.7.889"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320460105"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.29.6.412"},{"key":"e_1_2_6_15_1","first-page":"522","article-title":"Measuring behavioral phenotypes: Provocations from the \u2018New Genetics\u2019","volume":"99","author":"Dykens EM","year":"1995","journal-title":"Am J Ment Retard"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1994.tb11874.x"},{"key":"e_1_2_6_17_1","unstructured":"EinfeldSL TongeBJ.1992.Manual for the developmental behavior checklist. 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