Neurological impairments in Duchenne muscular dystrophy: A comprehensive review.

IF 2.1 4区 医学 Q3 CLINICAL NEUROLOGY
Xiao-Fang Zhang, Wenguang Hu, Jie Hu
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引用次数: 0

Abstract

Duchenne muscular dystrophy, the most prevalent form of muscular dystrophy, is characterized by neurological complications including cognitive impairment, neuropsychiatric disorders, and epilepsy. Neuroimaging investigations have demonstrated structural brain alterations, hemodynamic disturbances, and metabolic dysregulation in individuals with Duchenne muscular dystrophy. These neurological impairments are primarily attributed to cerebral dystrophin deficiency and subsequent downstream molecular/cellular abnormalities, including altered excitation-inhibition balance, blood-brain barrier disruption, calcium dysregulation, and neuroinflammation. Current therapeutic strategies focus on two main methods: (1) enhancing brain expression of truncated dystrophin; (2) addressing downstream consequences of dystrophin loss. This review synthesizes recent literature on the neurological manifestations of Duchenne muscular dystrophy, providing a theoretical framework to inform clinical management strategies.

杜氏肌营养不良症的神经损伤:综合综述。
杜氏肌营养不良症是最常见的一种肌营养不良症,其特征是神经系统并发症,包括认知障碍、神经精神障碍和癫痫。神经影像学研究表明,杜氏肌营养不良患者的脑结构改变、血流动力学紊乱和代谢失调。这些神经损伤主要归因于脑营养不良蛋白缺乏和随后的下游分子/细胞异常,包括兴奋-抑制平衡改变、血脑屏障破坏、钙调节失调和神经炎症。目前的治疗策略主要集中在两种方法上:(1)增强截断的肌营养不良蛋白的脑表达;(2)解决肌营养不良蛋白损失的下游后果。本文综述了最近关于杜氏肌营养不良的神经学表现的文献,为临床管理策略提供了理论框架。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Acta neurologica Belgica
Acta neurologica Belgica 医学-临床神经学
CiteScore
4.20
自引率
3.70%
发文量
300
审稿时长
6-12 weeks
期刊介绍: Peer-reviewed and published quarterly, Acta Neurologica Belgicapresents original articles in the clinical and basic neurosciences, and also reports the proceedings and the abstracts of the scientific meetings of the different partner societies. The contents include commentaries, editorials, review articles, case reports, neuro-images of interest, book reviews and letters to the editor. Acta Neurologica Belgica is the official journal of the following national societies: Belgian Neurological Society Belgian Society for Neuroscience Belgian Society of Clinical Neurophysiology Belgian Pediatric Neurology Society Belgian Study Group of Multiple Sclerosis Belgian Stroke Council Belgian Headache Society Belgian Study Group of Neuropathology
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