小脑性缄默综合征言语语言障碍研究进展

杨伟; 刘恒鑫; 陈邦绮; 尹光恒; 巴甫仁; 葛明

doi:10.3969/j.issn.1672-4933.2024.01.012

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小脑性缄默综合征言语语言障碍研究进展

儿童言语语言障碍治疗 | 更新时间：2024-03-22

- 小脑性缄默综合征言语语言障碍研究进展
- Advances in Research on Speech and Language Disturbances in Cerebellar Mutism Syndrome
- 中国听力语言康复科学杂志 2024年22卷第1期页码：42-45
- 作者机构：
  
  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北京100045
  2.国家儿童医学中心/首都医科大学附属北京儿童医院耳鼻咽喉头颈外科/儿童耳鼻咽喉头颈外科疾病北京市重点实验室北京100045
  3.国家儿童医学中心/首都医科大学附属北京儿童医院影像科北京100045
  4.台北护理健康大学中国台湾 11219
- 作者简介：
  
  杨伟博士住院医师；研究方向：儿童脑肿瘤、小脑性缄默综合征
  刘恒鑫博士博士后副研究员；研究方向：言语语言病理学、嗓音发声学
  巴甫仁， E-mail： brajotslp@gmail.com
  葛明， E-mail： geming88@126.com
- 基金信息：
  
  北京市医院管理中心临床医学发展专项(XMLX202144)
- DOI：10.3969/j.issn.1672-4933.2024.01.012
  中图分类号： R767.92
- 纸质出版日期：2024-01-15，
  
  收稿日期：2023-11-15，
- 稿件说明：
移动端阅览
杨伟,刘恒鑫,陈邦绮尹光恒等.小脑性缄默综合征言语语言障碍研究进展[J].中国听力语言康复科学杂志,2024,22(01):42-45.

YANG Wei,LIU Heng-xin,CHEN Bang-qi,et al.Advances in Research on Speech and Language Disturbances in Cerebellar Mutism Syndrome[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2024,22(01):42-45.
杨伟,刘恒鑫,陈邦绮尹光恒等.小脑性缄默综合征言语语言障碍研究进展[J].中国听力语言康复科学杂志,2024,22(01):42-45. DOI： 10.3969/j.issn.1672-4933.2024.01.012.

YANG Wei,LIU Heng-xin,CHEN Bang-qi,et al.Advances in Research on Speech and Language Disturbances in Cerebellar Mutism Syndrome[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2024,22(01):42-45. DOI： 10.3969/j.issn.1672-4933.2024.01.012.

摘要

小脑性缄默综合征（cerebellar mutism syndrome，CMS）是儿童后颅窝肿瘤切除手术后常见的并发症，发病率约8%～40%。主要特征包括一过性言语语言障碍、运动功能障碍、情感障碍和认知障碍。言语障碍通常在术后当天或数天内出现，大多在术后3个月内逐渐恢复。一些患儿可能会经历较长时间的言语问题。CMS的言语语言障碍临床表现多样，包括完全缄默、语言退化、声音响度降低和构音障碍等。CMS的病程复杂，患者可能表现出不同的类型语言障碍。发病机制假说包括小脑-大脑环路损害假说，吉兰-莫拉雷三角环路损伤、小脑蚓部损伤、术后水肿假说等。目前对CMS的言语语言障碍类型机制的研究不足，值得更多的关注。未来的研究方向包括制定专业的言语语言评估工具，建立CMS的预后模型，以便更好地了解CMS及其言语语言障碍，提高诊断和治疗的精准性。

Abstract

Cerebellar mutism syndrome (CMS) is the most common complication following surgical resection of pediatric posterior fossa tumors

with an incidence ranging approximately from 8% to 40%. CMS is characterized by transient speech and language disturbances

motor dysfunction

emotional issues

and cognitive impairments. Speech disturbances typically manifest on the day of surgery or within a few days

with most recovering gradually within three months postoperatively. However

some patients may experience prolonged speech issues. Clinical manifestations of speech and language disturbances in CMS are diverse and include complete mutism

speech regression

reduced vocal intensity

and articulation disorders

among others. The course of CMS is complex

and different patients may exhibit different types of language disorders.Hypotheses regarding the pathogenesis of CMS include the cerebellar-cerebral circuit damage hypothesis

particularly the cerebellar-thalamo-cortical circuit (DTC)

the Guillain-Mollaret triangle circuit

damage to the vermis of the cerebellum

and the postoperative edema hypothesis

among others. The research regarding the different types of language impairment among CMS is lack and need more attention. Future research directions include the development of specialized speech and language assessment tools

the establishment of prognostic models for CMS

aiming to better understand CMS and its speech and language disturbances

and enhancing the precision of diagnosis and treatment.

关键词

小脑性缄默综合征言语语言障碍发病机制研究进展

Keywords

Cerebellar mutism syndromeSpeech and language disturbancesPathogenesisResearch progress

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