Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis

被引:20
作者
Adams, H
de Blieck, EA
Mink, JW
Marshall, FJ
Kwon, J
Dure, L
Rothberg, PG
Ramirez-Montealegre, D
Pearce, DA
机构
[1] Univ Rochester, Med Ctr, Pediat Clin Res Off, Sch Med & Dent, Rochester, NY 14642 USA
[2] Univ Alabama Birmingham, Dept Pediat, Birmingham, AL USA
关键词
D O I
10.1017/S0012162206000570
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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页码:259 / 264
页数:6
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