Psychiatric symptoms of children and adolescents with juvenile neuronal ceroid lipofuscinosis
Psychiatric symptoms of children and adolescents with juvenile neuronal ceroid lipofuscinosis
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DOI：
10.1111/j.1365-2788.2005.00659.x
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发表时间：
2005-01-01
影响因子：
3.6
通讯作者：
Aronen, ET
中科院分区：
文献类型：
--
作者：
Bäckman, ML;Santavuori, PR;Aronen, ET
青少年神经性神经样脂褐质病（JNCL）是儿童和青少年最常见的神经退行性疾病之一。临床表现包括多种复杂的精神症状，难以治疗。只能对症治疗。为了改善对症治疗，认识症状是很重要的。本研究的目的是确定JNCL患者的主要精神症状。方法对27例JNCL患者进行精神药物治疗和非精神药物治疗。平均年龄15.2岁（范围9-21岁）。采用家长、教师和患者自行填写的标准化问卷：儿童行为检查表（CBCL）、教师报告表（TRF）和儿童抑郁量表（CDI），明确本组患者的特征性精神症状。对整个研究组的症状进行记录，并在接受和未接受精神药物治疗的患者之间以及性别之间进行比较。结果根据CBCL和TRF，患者有大量的精神症状。最常见的症状是社交、思维、注意力问题、身体不适和攻击行为。根据CBCL和TRF，接受精神药物治疗的患者有更多的精神症状。此外，根据CBCL，女性患者比男性患者有更多的问题。74%的患者精神障碍的总症状评分处于临床或边缘范围。患者自己报告的抑郁症状数量很少。结论JNCL患者存在多种精神症状。为了改善药物选择和剂量，需要在开始治疗前用标准化方法对这些症状进行全面评估。应定期监测治疗的进展和可能的不良反应。
Background Juvenile neuronal ceroid lipofuscinosis (JNCL) is one of the most common neurodegenerative disorders in childhood and adolescence. The clinical picture includes diverse and complex psychiatric symptoms that are difficult to treat. Only symptomatic treatment is available. To improve symptomatic therapy, it is important to recognize the symptoms. The purpose of this study was to identify predominant psychiatric symptoms in patients with JNCL.Methods The study included 27 patients with JNCL with and without psychotropic treatment. The mean age was 15.2 (range 9-21) years. Characteristic psychiatric symptoms in this patient group were clarified by using the following standardized questionnaires filled in by parents, teachers and the patients themselves: Child Behavior Checklist (CBCL), Teacher Report Form (TRF) and Children's Depression Inventory (CDI). The symptoms were recorded for the entire study group and compared between patients with and without psychotropic treatment and between genders.Results The patients had a large number of psychiatric symptoms according to the CBCL and TRF. The most commonly reported symptoms were social, thought, attention problems, somatic complaints and aggressive behaviour. Patients receiving psychotropic medication had more psychiatric symptoms according to the CBCL and TRF. Moreover, female patients had more problems than male patients according to the CBCL. The total psychiatric symptom score was at clinical or borderline range for psychiatric disturbance in 74% of patients. The number of depressive symptoms reported by the patients themselves was low.Conclusions JNCL patients suffer from a multitude of psychiatric symptoms. To improve drug choice and dosage, a thorough evaluation of these symptoms by standardized methods is needed before initiating treatment. Progress and possible adverse effects of treatment should be monitored on a regular basis.