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小儿头部外伤伴小脑损伤后的小脑缄默综合征

Cerebellar mutism syndrome in pediatric head trauma with cerebellar injury.

作者信息

Chivet Anaïs, Delestret Isabelle, Brodar Céline, Vinchon Matthieu

机构信息

Department of Pediatric Neurosurgery, Lille University Hospital, Lille, France.

出版信息

Childs Nerv Syst. 2022 Apr;38(4):759-766. doi: 10.1007/s00381-021-05422-2. Epub 2022 Jan 7.

DOI:10.1007/s00381-021-05422-2
PMID:34993606
Abstract

PURPOSE

Cerebellar mutism syndrome (CMS) after cerebellar injury in pediatric head trauma is a poorly recognized condition that is not properly diagnosed or treated in our daily practice. We aimed to clinically identify this syndrome after isolated posttraumatic cerebellar injury and to propose pathophysiological explanation.

METHODS

We retrospectively analyzed 8 consecutive children presenting with isolated cerebellar injury over 16 years. Clinical presentation, radiological type and localization of injury, clinical initial CMS symptoms, and long-term neurocognitive outcome were reviewed.

RESULTS

Out of 8 patients presenting with isolated traumatic cerebellar injury, we diagnosed 2 cases with initial clinical symptoms of CMS. Both patients had an injury damaging median structures of the posterior fossa, especially the fourth ventricle and dentate nuclei. Initial symptoms lasted more than 1 month for one patient, who still presented concentration difficulties almost 1 year after the head injury.

CONCLUSION

CMS after traumatic cerebellar injury does exist, even if it seems to be a very rare entity. It has to be better detected and studied in order to enrich pathophysiological knowledge about CMS of all etiologies and to bring our concerned patients the suitable follow-up and rehabilitative care that they could benefit from.

摘要

目的

小儿头部外伤后小脑损伤所致的小脑缄默综合征(CMS)是一种在日常临床实践中认识不足、未得到正确诊断和治疗的疾病。我们旨在临床识别孤立性创伤后小脑损伤后的这种综合征,并提出其病理生理学解释。

方法

我们回顾性分析了16年间连续收治的8例孤立性小脑损伤患儿。对临床表现、损伤的影像学类型和定位、临床初始CMS症状以及长期神经认知结局进行了回顾。

结果

在8例孤立性创伤性小脑损伤患者中,我们诊断出2例初始临床表现为CMS。两名患者均有损伤累及后颅窝的正中结构,尤其是第四脑室和齿状核。一名患者的初始症状持续超过1个月,在头部受伤近1年后仍存在注意力不集中的问题。

结论

创伤后小脑损伤后的CMS确实存在,尽管它似乎是一种非常罕见的疾病。必须更好地检测和研究它,以丰富关于所有病因的CMS的病理生理学知识,并为我们所关注的患者提供他们可能受益的适当随访和康复护理。

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Childs Nerv Syst. 2021 Sep;37(9):2743-2751. doi: 10.1007/s00381-021-05225-5. Epub 2021 Jun 21.
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The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper.小脑认知情感/施马曼综合征:一个工作组文件。
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