贝克型肌营养不良症的认知特征和神经精神障碍：文献系统综述。
Cognitive profile and neuropsychiatric disorders in Becker muscular dystrophy: A systematic review of literature.
机构信息
Department of Brain and Behavioural Sciences, Unit of Child Neurology and Psychiatry, University of Pavia, Pavia, Italy.
Child Neurology and Psychiatry Unit, IRCCS Mondino Foundation, Pavia, Italy.
出版信息
Neurosci Biobehav Rev. 2022 Jun;137:104648. doi: 10.1016/j.neubiorev.2022.104648. Epub 2022 Mar 31.
BACKGROUND
Brain co-morbidities in DMD are well-documented, less is known about the cognitive, behavioral and psychosocial functioning of patients with BMD.
METHODS
The systematic review was carried out on two databases (Pubmed and Scopus) according to the PRISMA guidelines. We included all research articles specific to BMD written after 1995.
RESULTS
Studies examining neuropsychological and neurobehavioral functioning in BMD are few and have several methods limitations. BMD population is characterized by high rates of cognitive impairment, with specific involvement of different cognitive areas. Unlike DMD, verbal skills are better preserved. Neurodevelopmental and emotional/behavioral disorders have great importance in BMD, due to their high prevalence. Lack of Dp140 or Dp71 can cause intellectual disability, these isoforms are probably responsible for the other brain-related comorbidities as well.
DISCUSSION
The results suggest that cognitive and neuropsychiatric comorbid symptoms may affect a significant proportion of BMD patients therefore it is important to mental health and neuropsychological screening. Finding tools for an adequate assessment is a priority in order to include brain outcome measures in clinical trials.
背景
DMD 患者的脑部合并症已有大量文献记载，而关于 BMD 患者的认知、行为和心理社会功能的信息则较少。
方法
根据 PRISMA 指南，在两个数据库（PubMed 和 Scopus）上进行了系统综述。我们纳入了自 1995 年以来所有针对 BMD 的特定研究文章。
结果
研究 BMD 患者神经心理学和神经行为功能的研究较少，且存在多种方法上的局限性。BMD 人群的认知障碍发生率较高，涉及不同的认知领域。与 DMD 不同的是，言语技能较好地保留。神经发育和情绪/行为障碍在 BMD 中非常重要，因为它们的患病率很高。缺乏 Dp140 或 Dp71 可能导致智力障碍，这些同工型可能也是导致其他与大脑相关的合并症的原因。
讨论
结果表明，认知和神经精神合并症症状可能会影响相当一部分 BMD 患者，因此对精神健康和神经心理学筛查很重要。找到合适的评估工具是当务之急，以便在临床试验中纳入大脑结果测量。
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