BACKGROUND Adaptive skills represent the ways that children and adolescents meet their basic needs for self-care, decision making, communication, and learning in their daily life. Having a neuromuscular disease (NMD) not only presents mental health issues, but also impacts these skills. AIMS Our study aimed to compare the adaptive skills and mental health of paediatric patients with the most common NMDs with their healthy peers and assess how NMDs shape the way patients form relationships with others, engage in leisure activities and take care of their daily living needs. METHODS We used the Adaptive Behaviour Assessment System (ABAS-3) and Achenbach Child Behaviour Checklist (CBCL) to compare the adaptive skills and mental health symptoms of 50 NMD patients to a demographically-matched control group of 298 peers. We examined specific outcomes of having myotonic dystrophy (DM), Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), spinal muscular atrophy (SMA) or a mixed group of other NMDs. RESULTS All NMD patients displayed poor practical adaptive skills. When the disease was more likely to involve the central nervous system (DM, DMD) they also showed additional deficits in their conceptual and social skills. Contrary to previous research no increased rate of psychopathological symptoms was found in NMD patients, with the exception of difficulties in the social domain among patients with DM. CONCLUSIONS Although most children with NMDs displayed more limited practical skills, the specific profile of adaptive skills for each patient group needs to be taken into consideration when planning school support and other psychosocial interventions.

中文翻译：

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患有神经肌肉疾病的儿童和青少年的适应技能和心理健康

背景适应性技能代表了儿童和青少年在日常生活中满足他们对自我照顾、决策、沟通和学习的基本需求的方式。患有神经肌肉疾病 (NMD) 不仅会出现心理健康问题，还会影响这些技能。目的 我们的研究旨在比较患有最常见 NMD 的儿科患者与其健康同龄人的适应技能和心理健康，并评估 NMD 如何影响患者与他人建立关系、参与休闲活动和照顾他们的日常生活需求的方式。方法 我们使用适应性行为评估系统 (ABAS-3) 和 Achenbach 儿童行为检查表 (CBCL) 将 50 名 NMD 患者的适应性技能和心理健康症状与人口统计学匹配的 298 名同龄人的对照组进行比较。我们检查了强直性肌营养不良症 (DM)、杜氏肌营养不良症 (DMD)、贝克尔肌营养不良症 (BMD)、脊髓性肌萎缩症 (SMA) 或其他 NMD 的混合组的具体结果。结果 所有 NMD 患者都表现出较差的实际适应能力。当疾病更可能涉及中枢神经系统（DM，DMD）时，他们在概念和社交技能方面也表现出额外的缺陷。与之前的研究相反，除了 DM 患者在社交领域存在困难之外，没有发现 NMD 患者的精神病理学症状发生率增加。结论 尽管大多数患有 NMD 的儿童表现出的实践技能较为有限，但在计划学校支持和其他社会心理干预时，需要考虑每个患者群体的适应技能的具体情况。