Dykens E M, Cassidy S B, King B H
Neuropsychiatric Institute, Division of Child and Adolescent Psychiatry, University of California, Los Angeles 90024-1759, USA.
Am J Ment Retard. 1999 Jan;104(1):67-77. doi: 10.1352/0895-8017(1999)104<0067:MBDIPS>2.0.CO;2.
Maladaptive behavior was compared across 23 people with Prader-Willi syndrome due to paternal deletion to 23 age- and gender-matched subjects with maternal uniparental disomy. Controlling for the higher IQs of the uniparental disomy group, deleted cases showed significantly higher maladaptive ratings on the Child Behavior Checklist's Internalizing, Externalizing, and Total domains as well as more symptom-related distress on the Yale-Brown Obsessive-Compulsive Scale. Across both measures, deleted cases were more apt to skin-pick, bite their nails, hoard, overeat, sulk, and withdraw. A dampening of symptom severity is suggested in Prader-Willi syndrome cases due to maternal uniparental disomy. Findings are compared to Angelman syndrome, and possible genetic mechanisms are discussed, as are implications for Prader-Willi syndrome and obsessive-compulsive behaviors.
对23名因父系缺失导致普拉德-威利综合征的患者与23名年龄和性别匹配的母系单亲二倍体患者的适应不良行为进行了比较。在控制单亲二倍体组较高智商的情况下，缺失病例在儿童行为清单的内化、外化和总领域的适应不良评分显著更高，并且在耶鲁-布朗强迫症量表上与症状相关的痛苦更多。在这两项测量中，缺失病例更倾向于抠皮肤、咬指甲、囤积、暴饮暴食、生闷气和退缩。母系单亲二倍体导致的普拉德-威利综合征病例中症状严重程度有所减轻。研究结果与天使综合征进行了比较，并讨论了可能的遗传机制以及对普拉德-威利综合征和强迫行为的影响。
