兒童小腦動靜脈畸形出血後之小腦性緘默症：兩病例報告

小腦性緘默症(cerebellar mutism syndrome)為一種少見的症狀，大多發生於接受後顱窩腫瘤手術之後的兒童而少見於成人，只有極少數案例因小腦動靜脈畸形出血而引起。其症狀包括暫時性的緘默症，但聽理解能力正常，吞嚥機能障礙，可能合併出現躁動不安，認知功能變差，大小便失禁等症狀。在口語表達能力恢復後會有一段較長時間的構音困難(dysarthria)。本篇報告二位因小腦動靜脈畸形出血術後而產生緘默症之兒童（一位11歲，一位15歲），其緘默不語持續時間各為6個月及2.5個月。此二位患者皆是聽理解能力恢復早於口語表達能力，而且恢復初期皆表現出躁動不安及吞嚥障礙等症狀。而患者在各項功能逐漸恢復之後，仍持續表現出明顯的構音困難。小腦性緘默症可能是因術後組織水腫、缺血或小腦結構損傷而造成交叉性小腦－大腦神經功能聯繫斷絕(crossed cerebello-cerebral diaschisis)，因而影響語言表達功能，但實際的機制則尚未完全明瞭。此病例報告，希望能提供醫療人員在照顧此類患者時，對其可能出現的症狀及預後能作一個參考。

關鍵字

小腦性緘默症；動靜脈畸形；交叉性小腦－大腦神經功能聯繫斷絕

並列摘要

Cerebellar mutism syndrome is a rare but devastating consequence following posterior fossa surgery or lesion. It tends to occure predominantly in children, and is characterized by transient mutism, oromotor apraxia, apathy, hypokinesis, irritability and mood lability. The vermis, paravermian region and deep cerebellar nuclei are important structures which are highly correlated with the occurrence of cerebellar mutism syndrome. We report two cases, aged 11 and 15 years, who developed cerebellar mutism after rupture of posterior fossa arteriovenous malformation. Swallowing difficulty, irritability and aggressive behavior occurred concomitant with mutism. For the two cases, their symptoms of mutism lasted for 6 months and 2.5 months respectively. Unfortunately, dysarthria occurred following the resolution of mutism, even though the swallowing ability and behavioral control improved. The pathophysiologic mechanism of this syndrome may be due to crossed cerebello-cerebral diaschisis caused by tissue swelling, vasospasm or direct injury to these structures of cerebellum. The long term effect of cerebellar mutism on high cognitive function remains to be investigated.

並列關鍵字

cerebellar mutism ； arteriovenous malformation ； crossed cerebello-cerebral diaschisis

國際替代計量

兒童小腦動靜脈畸形出血後之小腦性緘默症：兩病例報告

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