Papero P H, Pranzatelli M R, Margolis L J, Tate E, Wilson L A, Glass P
Department of Psychiatry and Behavioral Sciences, Children's National Medical Center, George Washington University, Washington, DC 20010-2970, USA.
Dev Med Child Neurol. 1995 Oct;37(10):915-32. doi: 10.1111/j.1469-8749.1995.tb11944.x.
Childhood opsoclonus-myoclonus syndrome (OMS) is a movement disorder which typically strikes children in the early preschool years, seriously affecting intellectual, social-emotional and general adaptive development. This series of 13 cases with well-documented neurological histories, aged 1.7 to 16.3 years, provides an initial systematic evaluation of these children's neuropsychological, psychosocial and adaptive status. As expected, children with OMS had significantly reduced intelligence and severe speech and motor output problems; however, most of them also demonstrated a range of preserved neurocognitive abilities and impressive goal-directedness and communicative effort. Psychosocial problems included mild behavioral impairment on the Achenbach Child Behavior Checklist, and severe adaptive limitations on the Vineland Adaptive Behavior Scales despite relatively strong social skills. Developmental factors and likelihood of subcortical localization are discussed, and practical guidelines are provided for behavioral and educational management of these children.
儿童眼阵挛-肌阵挛综合征（OMS）是一种运动障碍，通常在幼儿早期侵袭儿童，严重影响智力、社会情感和一般适应性发展。这组13例有详细神经病史的病例，年龄在1.7至16.3岁之间，对这些儿童的神经心理、社会心理和适应状况进行了初步系统评估。不出所料，患有OMS的儿童智力显著下降，存在严重的言语和运动输出问题；然而，他们中的大多数也表现出一系列保留的神经认知能力以及令人印象深刻的目标导向性和沟通努力。社会心理问题包括在阿肯巴克儿童行为量表上有轻度行为损害，以及在文兰适应行为量表上有严重的适应局限性，尽管社交技能相对较强。讨论了发育因素和皮质下定位的可能性，并为这些儿童的行为和教育管理提供了实用指南。
