Department of Clinical Genetics, Maastricht University Medical Centre, GROW School for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands.
Res Dev Disabil. 2011 Sep-Oct;32(5):1729-35. doi: 10.1016/j.ridd.2011.02.027. Epub 2011 Mar 31.
Previous studies have suggested an association between PWS and comorbid psychiatric illness. Data on prevalence rates of psychopathology is still scarce. This paper describes a large-scale, systematic study investigating the prevalence of psychiatric illness in a Dutch adult PWS cohort. One hundred and two individuals were screened for psychiatric illness. Case vignettes were written by the first author on 63 individuals with a positive screening on psychopathology according to the interviews, medical history, medication use and behavioural questionnaires. These case vignettes were rated by two psychiatrists specializing in intellectual disability (ID). Psychopathology was divided into four diagnostic categories: bipolar disorder with psychotic symptoms, psychotic illness, depressive illness with psychotic symptoms and depressive illness without psychotic symptoms. Nine out of 53 persons (17%) with a 15q11-13 deletion and 28 out of 44 (64%) persons with maternal uniparental disomy (mUPD) were diagnosed with a current or previous psychiatric illness. Depressive illness with psychotic symptoms was the cause of psychiatric problems in the majority of persons with PWS due to deletion (56%). In the case of mUPD, almost all individuals with histories of psychopathology suffered from psychotic symptoms (85%) with or without affective component. Psychiatric examination should be part of general management of adults with PWS, especially when caused by mUPD. More attention should be paid to the presence of precursor symptoms, indicating a developing psychiatric episode. Longitudinal studies are needed to gain more insight into the natural history of psychiatric illness in adults with PWS.
先前的研究表明，PWS 与合并的精神疾病之间存在关联。有关精神病理学患病率的数据仍然很少。本文描述了一项大规模、系统的研究，该研究调查了荷兰成年 PWS 队列中精神疾病的患病率。对 102 名个体进行了精神疾病筛查。根据访谈、病史、用药情况和行为问卷，对 63 名有阳性精神病理学筛查的个体进行了案例描述。由两位专门从事智力障碍 (ID) 的精神科医生对这些案例描述进行了评分。精神病理学分为四个诊断类别：伴有精神病性症状的双相情感障碍、精神病性疾病、伴有精神病性症状的抑郁障碍和不伴有精神病性症状的抑郁障碍。在 53 名 15q11-13 缺失的个体中，有 9 名（17%）和在 44 名母源单亲二体（mUPD）的个体中，有 28 名（64%）被诊断为当前或既往有精神疾病。由于缺失，患有 PWS 的个体中，约有 56%的个体患有伴有精神病性症状的抑郁障碍，是导致其精神问题的主要原因。在 mUPD 的情况下，几乎所有有精神病史的个体都患有精神病性症状（85%），伴有或不伴有情感成分。对患有 PWS 的成年人，精神科检查应作为一般管理的一部分，尤其是在由 mUPD 引起的情况下。应更加注意前驱症状的存在，这些症状表明可能正在发生精神发作。需要进行纵向研究，以更深入地了解患有 PWS 的成年人的精神疾病的自然史。
