Umansky Richard, Watson John S, Colvin Lyn, Fyfe Susan, Leonard Seonaid, de Klerk Nicholas, Leonard Helen
Children's Hospital and Research Center at Oakland, CA, USA.
J Child Neurol. 2003 Jul;18(7):481-7. doi: 10.1177/08830738030180070201.
Residual hand use in functional tasks, extent of laterality, and right or left preference were studied in 145 2- to 24-year-old, postregression Australian subjects with Rett syndrome via parent questionnaire. Hand use was markedly restricted, more for complex than simple and for external (touching food and objects) than internal tasks (scratching, rubbing eyes), suggesting a deficit in cerebral control of external, goal-oriented hand use, which is perhaps genetically determined because there is significantly greater restriction of external tasks in subjects with demonstrated MECP2 mutations. Overall, 33.6% of patients were reported with a left-hand preference, 40.7% with a right-hand preference, and 25.7% with an equal hand preference. Extent of laterality was greater for external than internal and for complex than simple external tasks. Older subjects showed less functional hand use and possibly more overall laterality. However, their hand preference was similar to younger subjects. The anomalous pattern of hand preference in Rett syndrome may be linked to the primary apraxic deficit in this disorder rather than to late manifestation of laterality.
通过家长问卷对145名年龄在2至24岁、回归后患有雷特综合征的澳大利亚受试者进行了研究，以了解其在功能性任务中的残留手部使用情况、偏侧性程度以及右利手或左利手偏好。手部使用明显受限，复杂任务比简单任务受限更明显，外部任务（触摸食物和物体）比内部任务（抓挠、揉眼睛）受限更明显，这表明大脑对外部、有目标导向的手部使用的控制存在缺陷，这可能是由基因决定的，因为在已证实存在MECP2突变的受试者中，外部任务的受限程度明显更大。总体而言，报告显示33.6%的患者为左利手，40.7%为右利手，25.7%双手偏好程度相同。外部任务的偏侧性程度大于内部任务，复杂外部任务的偏侧性程度大于简单外部任务。年龄较大的受试者手部功能使用较少，总体偏侧性可能更强。然而，他们的利手偏好与较年轻的受试者相似。雷特综合征中异常的利手偏好模式可能与该疾病的原发性失用症缺陷有关，而不是与偏侧性的晚期表现有关。
