小脑mutism综合征(CMS)是影响许多接受后颅窝病变手术的儿童的主要并发症。病因和病理生理学仍未完全了解。CMS严重影响这些患者的生活质量和康复。尚未确定有效的治疗方法。这篇基于案例的综述旨在分析现有的证据和知识,以更好地描述这一现象,并确定CMS是否可以通过药物治疗成功治疗。
进行了系统的研究和数据库检索,分析了报告CMS医学治疗的所有论文。关于定义的最新理解和报告摘要,临床表现,病理生理学,管理,CMS的结果已经进行。
对这种综合征的定义缺乏共识。CMS是2016年后果体协会接受的术语。病理生理学仍然知之甚少,但最可能的机制是小脑传出通路近端部分的损伤。已经确定了九篇描述CMS药物治疗的积极作用的论文。氟西汀,唑吡坦,溴隐亭,和咪达唑仑是似乎可以缓解CMS症状并改善恢复的药物。迄今为止,认知康复和物理治疗是唯一可用的治疗选择。
CMS对受影响的儿童及其家庭产生了深远的影响。尽管试图确定预防措施和治疗方法,病例仍然定期发生。药物治疗已被提出,以帮助减少CMS的症状与一些有希望的结果,但是报告有限;因此,需要进一步的研究。
Cerebellar mutism syndrome (CMS) represents a major complication affecting many children that undergo surgery for posterior fossa lesions. Etiology and pathophysiology are still not fully understood. CMS deeply influences quality of life and recovery of these patients. An effective treatment has not been defined yet. This
case-based review aims at analyzing the available evidence and knowledge to better delineate this phenomenon and to determine whether CMS can be successfully treated with pharmacological therapy.
Systematic research and retrieval of databases were conducted analyzing all papers where medical treatment of CMS was reported. A summary of the latest understanding and
reports regarding definition, clinical manifestations, pathophysiology, management, and outcome of CMS has been conducted.
Consensus on definition of this syndrome is lacking. CMS is the term accepted by the Posterior Fossa Society in 2016. Pathophysiology is still poorly understood but the most likely mechanism is injury along proximal components of the efferent cerebellar pathway. Nine papers describing positive effects of pharmacological therapy for CMS have been identified. Fluoxetine, zolpidem, bromocriptine, and midazolam are the drugs that seem to alleviate symptoms of CMS and improve recovery. To date, cognitive rehabilitation and physiotherapy are the only treatment options available.
CMS has deep impact on affected children and their families. Despite attempts to identify preventive measures and treatment, cases still occur on a regular basis. Pharmacological treatments have been proposed to help reduce the symptoms of CMS with some promising results, but
reports are limited; therefore, further studies are needed.