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运动障碍性脑瘫的临床表现和治疗。

Clinical presentation and management of dyskinetic cerebral palsy.

机构信息

Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium; Dominiek Savio Instituut, Gits, Belgium.

Department of Pediatrics, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

出版信息

Lancet Neurol. 2017 Sep;16(9):741-749. doi: 10.1016/S1474-4422(17)30252-1.

DOI:10.1016/S1474-4422(17)30252-1
PMID:28816119
Abstract

Cerebral palsy is the most frequent cause of severe physical disability in childhood. Dyskinetic cerebral palsy (DCP) is the second most common type of cerebral palsy after spastic forms. DCP is typically caused by non-progressive lesions to the basal ganglia or thalamus, or both, and is characterised by abnormal postures or movements associated with impaired tone regulation or movement coordination. In DCP, two major movement disorders, dystonia and choreoathetosis, are present together most of the time. Dystonia is often more pronounced and severe than choreoathetosis, with a major effect on daily activity, quality of life, and societal participation. The pathophysiology of both movement disorders is largely unknown. Some emerging hypotheses are an imbalance between indirect and direct basal ganglia pathways, disturbed sensory processing, and impaired plasticity in the basal ganglia. Rehabilitation strategies are typically multidisciplinary. Use of oral drugs to provide symptomatic relief of the movement disorders is limited by adverse effects and the scarcity of evidence that the drugs are effective. Neuromodulation interventions, such as intrathecal baclofen and deep brain stimulation, are promising options.

摘要

脑性瘫痪是儿童期最常见的严重身体残疾原因。不随意运动型脑性瘫痪(DCP)是继痉挛型脑性瘫痪之后的第二大常见类型。DCP 通常由基底节或丘脑的进行性或非进行性病变引起,其特征是存在姿势或运动异常,同时伴有肌张力调节或运动协调障碍。在 DCP 中,两种主要的运动障碍,即肌张力障碍和舞蹈手足徐动症,大多数时候同时存在。肌张力障碍通常比舞蹈手足徐动症更明显和严重,对日常活动、生活质量和社会参与有重大影响。这两种运动障碍的病理生理学在很大程度上是未知的。一些新兴的假说包括间接和直接基底节通路之间的不平衡、感觉处理障碍和基底节可塑性受损。康复策略通常是多学科的。口服药物用于缓解运动障碍的症状,但由于不良反应和缺乏药物有效性的证据,其应用受到限制。神经调节干预,如鞘内巴氯芬和深部脑刺激,是有前途的选择。

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