Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis
Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis
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DOI：
10.1017/s0012162206000570
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发表时间：
2006-04-01
影响因子：
3.8
通讯作者：
Pearce, DA
中科院分区：
文献类型：
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作者：
Adams, H;de Blieck, EA;Pearce, DA
我们获得了26例幼年神经元性蜡样脂褐素沉积症(JNCL)儿童(男13例，女13例)的行为、精神和功能状态的信息，平均年龄12y3mo[SD 3y4mo]；25名儿童有视力障碍，18名儿童在登记前已知有阳性癫痫史。家长们完成了儿童行为检查表，修订了独立行为量表，并进行了结构化的访谈，以评估强迫症状。参与者表现出广泛的行为和精神问题，超过一半的样本被评为频繁和/或严重。男性和女性在行为和精神问题的数量上没有区别。儿童进行日常生活活动的能力也受到限制，包括自我照顾、卫生、社交和其他适合年龄的任务。结果为JNCL的行为和精神问题以及功能水平提供了一个量化的基线，可以根据这些基线来衡量进一步的下降。对行为和精神症状以及功能能力的纵向评估仍在继续，并将提供有关JNCL自然历史的急需数据。
We obtained information about the behavioral, psychiatric, and functional status of 26 children (13 males, 13 females) with juvenile neuronal ceroid lipofuscinosis (JNCL; mean age 12y 3mo [SD 3y 4mo]; range 6y 9mo to 18y 8mo). Twenty-five children had visual impairment and 18 were known to have a positive seizure history before enrollment. Parents completed the Child Behavior Checklist, Scales of Independent Behavior Revised, and a structured interview to assess obsessive-compulsive symptoms. Participants exhibited a broad range of behavioral and psychiatric problems, rated as occurring frequently and/or as severe in more than half of the sample. Males and females did not differ with regard to the number of behavioral and psychiatric problems. Children were also limited in their ability to perform activities of daily living, including self-care, hygiene, socialization, and other age-appropriate tasks. Results provide a quantitative baseline for behavioral and psychiatric problems and functional level in JNCL, against which further decline can be measured. Longitudinal assessment of behavioral and psychiatric symptoms and functional abilities is continuing and will provide much-needed data on the natural history of JNCL.