Shapira Nathan A, Lessig Mary C, Lewis Mark H, Goodman Wayne K, Driscoll Daniel J
College of Medicine, University of Florida, Gainesville 32610, USA.
Am J Ment Retard. 2004 Jul;109(4):301-9. doi: 10.1352/0895-8017(2004)109<301:EOTIAW>2.0.CO;2.
Prader-Willi syndrome is a multisystem neurogenetic obesity disorder with behavioral manifestations, including hyperphagia, compulsive behavior, self-injury, and mild to moderate mental retardation. In an 8-week open-label study, we evaluated adjunctive therapy with the anticonvulsant topiramate in 8 adults with Prader-Willi syndrome. Appetite was measured by a 1-hour access to food four times throughout the study and quantified with a visual analogue scale. Topiramate did not significantly change calories consumed, Body Mass Index, or increase self-reported appetite. In addition, there were no significant changes in compulsions. Surprisingly, topiramate treatment resulted in a clinically significant improvement in the self-injury (i.e., skin-picking) that is characteristic of this syndrome. Potential benefits of topiramate for self-injury should be evaluated further in controlled trials.
普拉德-威利综合征是一种多系统神经遗传性肥胖症,伴有行为表现,包括食欲亢进、强迫行为、自我伤害以及轻度至中度智力障碍。在一项为期8周的开放标签研究中,我们评估了抗惊厥药物托吡酯对8名患有普拉德-威利综合征的成年人的辅助治疗效果。在整个研究过程中,通过每周4次、每次1小时的食物摄入量来测量食欲,并使用视觉模拟量表进行量化。托吡酯并未显著改变热量摄入、体重指数或增加自我报告的食欲。此外,强迫行为也没有显著变化。令人惊讶的是,托吡酯治疗使该综合征特有的自我伤害行为(即抠皮肤)在临床上得到了显著改善。托吡酯对自我伤害的潜在益处应在对照试验中进一步评估。