Ishii Atsushi, Ihara Hiroshi, Ogata Hiroyuki, Sayama Masayuki, Gito Masao, Murakami Nobuyuki, Ayabe Tadayuki, Oto Yuji, Takahashi Asami, Nagai Toshiro
Department of Psychiatry, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.
National Hospital Organization Hanamaki Hospital, Hanamaki, Iwate, Japan.
Behav Neurol. 2017;2017:4615451. doi: 10.1155/2017/4615451. Epub 2017 Dec 26.
The effects of age and genotype were examined, with regard to the severity of aberrant, autistic, and food-related behaviors in Prader-Willi syndrome (PWS), with an emphasis on the contrast between adolescents and young adults. The Aberrant Behavior Checklist Japanese version (ABC-J), the Food Related Problem Questionnaire (FRPQ), and the Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) were administered to 65 PWS patients, including 20 adolescents (ages 12 to 17) and 45 young adults (ages 18 to 29). Significant differences (Mann-Whitney tests) were found in ABC-J ( = 0.004) and PARS ( = 0.021), with lower scores in adolescents than in young adults. While DEL subgroups showed no significant differences between the two age groups in ABC-J ( = 0.063) and PARS ( = 0.134), mUPD subgroups showed a statistically significant difference in terms of ABC-J ( = 0.007). No significant differences were found between adolescents and young adults, in terms of FRPQ ( = 0.163). These results suggest that aberrant and autistic behaviors follow a marked worsening trend from around the age of 18. On the other hand, food-related behaviors give no sign of change at this transitory stage. Young adults with mUPD were found to be significantly more severe than adolescents with mUPD, in terms of aberrant behaviors.
研究了年龄和基因型对普拉德-威利综合征（PWS）中异常、自闭症及食物相关行为严重程度的影响，重点关注青少年与青年成人之间的差异。对65例PWS患者进行了日本版异常行为检查表（ABC-J）、食物相关问题问卷（FRPQ）以及日本自闭症协会广泛性发育障碍评定量表（PARS）评估，其中包括20名青少年（12至17岁）和45名青年成人（18至29岁）。在ABC-J（P = 0.004）和PARS（P = 0.021）方面发现了显著差异（曼-惠特尼检验），青少年得分低于青年成人。虽然DEL亚组在ABC-J（P = 0.063）和PARS（P = 0.134）方面两个年龄组之间无显著差异，但mUPD亚组在ABC-J方面存在统计学显著差异（P = 0.007）。在FRPQ方面（P = 0.163），青少年和青年成人之间未发现显著差异。这些结果表明，异常和自闭症行为在18岁左右呈现明显恶化趋势。另一方面，在这个过渡阶段，食物相关行为没有变化迹象。发现mUPD的青年成人在异常行为方面比mUPD的青少年严重得多。
