Tsai Sen-Wei, Wu Shyi-Kuen, Liou Ying-Ming, Shu San-Ging
Department of Physical Medicine and Rehabilitation, Taichung Veteran's General Hospital, Taiwan.
Pediatr Int. 2008 Apr;50(2):221-4. doi: 10.1111/j.1442-200X.2008.02563.x.
The aim of the present study was to gain a better understanding of the motor performance in Williams syndrome for early intervention of rehabilitation programs.
Eleven Williams syndrome patients were evaluated from the pediatric clinics. Seven patients younger than 42 months were evaluated with the Bayley II Test for mental development index (MDI) and psychomotor development index (PDI). Four patients older than 42 months were evaluated with the Bruininks-Oseretsky Test (short form) of motor profile. The raw scores were measured and converted to the standard scores for comparison.
A significantly mental and psychomotor development delay of 6.1 and 5.7 months individually was found compared to that of the mean age (P > 0.0047 and 0.0053). Juvenile Williams syndrome patients were apparently retarded (<10 per thousand rank) in motor development in comparison with persons of the same age. The results showed that motor performance was severely delayed not only in Williams syndrome children but also in Williams syndrome juveniles.
It is important for clinicians to work out a comprehensive plan to help the motor development of patients with Williams syndrome in addition to treating their medical problems, and upper limb dexterity may be the goal for training.
本研究旨在更好地了解威廉姆斯综合征的运动表现，以便为康复计划的早期干预提供依据。
从儿科诊所选取11名威廉姆斯综合征患者进行评估。对7名年龄小于42个月的患者进行贝利婴幼儿发展量表第二版测试，以评估智力发展指数（MDI）和心理运动发展指数（PDI）。对4名年龄大于42个月的患者进行布吕宁克斯-奥塞瑞斯基运动技能测试（简式），测量原始分数并转换为标准分数进行比较。
与平均年龄相比，发现智力和心理运动发展明显延迟，分别延迟6.1个月和5.7个月（P > 0.0047和0.0053）。与同龄人相比，青少年威廉姆斯综合征患者的运动发育明显滞后（千分位排名<10）。结果表明，威廉姆斯综合征儿童和青少年的运动表现均严重延迟。
临床医生除了治疗威廉姆斯综合征患者的医疗问题外，制定全面计划以帮助其运动发育非常重要，上肢灵活性可能是训练目标。
