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作 者:杨伟 葛明[1] YANG Wei;GE Ming(Department of Neurosurgery,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)
机构地区:[1]国家儿童医学中心、首都医科大学附属北京儿童医院神经外科,100045
出 处:《中国现代神经疾病杂志》2024年第9期712-716,共5页Chinese Journal of Contemporary Neurology and Neurosurgery
基 金:北京市医院管理中心临床医学发展专项(项目编号:XMLX202144)。
摘 要:小脑性缄默综合征是儿童后颅窝肿瘤手术后的常见并发症,在不同病理类型肿瘤中的发生率存在较大差异(髓母细胞瘤最高,占24%~30%)。发病机制尚未阐明,认为与小脑⁃大脑回路损害、小脑顶核⁃中脑导水管周围灰质损害和脑网络改变相关。基于小脑性缄默综合征危险因素构建的预测模型尚未展现出预期的稳定性,仍未推广至临床。药物治疗主要基于临床经验,但疗效有待验证,故目前缺乏明确的有效治疗方法;非药物治疗如物理治疗、作业疗法和言语治疗,对改善远期生活质量有一定作用。本文综述小脑性缄默综合征的发病机制及治疗进展,未来研究应致力于探究小脑性缄默综合征的病理生理学机制、构建更准确的预测模型、制定个性化治疗方案,改善患儿远期预后。Cerebellar mutism syndrome(CMS)is a prevalent postoperative complication in children following posterior fossa tumor surgery,with a significantly variable incidence rate across different pathological types of tumors,being highest in medulloblastoma(24%-30%).The pathogenesis of CMS remains to be elucidated,but it is believed to be associated with damage to the cerebellocerebral circuits,harm to the fastigial nuclei of cerebellum and periaqueductal gray matter of the midbrain,and alterations in brain networks.Predictive models constructed based on the risk factors of CMS have not yet demonstrated the anticipated stability and have not been widely adopted in clinical settings.Pharmacological treatments are primarily based on clinical experience,yet their efficacy requires further validation,hence there is currently a lack of a clearly effective treatment method;non⁃pharmacological treatments,such as physical therapy,occupational therapy,and speech therapy,have shown some effect on improving the long⁃term quality of life.This review summarizes the pathogenesis and therapeutic advances of CMS,and future research should be dedicated to exploring the pathophysiological mechanisms of CMS,constructing more accurate predictive models,devising personalized treatment plans,and enhancing the long⁃term prognosis for children.
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