Acute Disseminated Encephalomyelitis in Children: One Medical Center Experience

兒童急性瀰漫性腦脊髓炎：一醫學中心之經驗

翁文瑾(Wen-Chin Weng) ； 彭信逢(Steven Shinn-Forng Feng) ； 李旺祚(Wang-Tso Lee) ； 范碧娟(Pi-Chuan Fan) ； 簡穎瑄(Yin-Hsuan Chien) ； 杜戎玨(Jung-Chieh Du) ； 沈友仁(Yu-Zen Shen)

《Acta Paediatrica Taiwanica》 47卷2期 (2006/04) Pp. 67-71

https://doi.org/10.7097/APT.200604.0067

摘要

急性瀰漫性腦脊髓炎是罕見的發炎性脫髓鞘性中樞神經系統病變。我們報告本院20位罹患急性瀰漫性腦脊髓炎的病人。他們分別為四個月至15歲大。其中17位(85%)病人在發病之前有感染症狀。病童最主要的臨床症狀為意識改變(70%)及肢體無力(55%)。有10位(50%)病童在發病初期有痙攣發作，但只有一位日後轉變為癲癇。所有病人的腦部核磁共振檢查皆呈現多發性病灶，主要位於皮質下白質(80%)、腦幹(65%)、基底核(55%)、小腦(45%)、視丘(40%)、及腦室周邊白質(35%)。9位病人接受核磁共振証實有脊髓侵犯。11個病人接受高劑量類固醇靜脈注射治療，其中只有一位留下輕微的神經學症狀。二十位病人中有五位留下神經學症狀，一位死亡。有三位病人在追蹤過程復發，其中兩位日後追蹤轉變為多發性硬化症。另有一位急性瀰漫性腦脊髓炎病人在兩年後轉變為多發性硬化症。我們認為痙攣發作在急性瀰漫性腦脊髓炎的病童並不少見，但只有極少數將來會轉變成癲癇。病人具有良好的長期預後，但復發的比率並不低，因此我們建議急性瀰漫性腦脊髓炎的病童需長期追蹤，以進一步釐清急性瀰漫性腦脊髓炎和多發性硬化症的關係。

關鍵字

瀰漫性腦脊髓炎 ； 急性 ； 多面向性 ； 痙攣 ； 孩童

並列摘要

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system. The experience in children is limited. We retrospectively reviewed our experience with 20 ADEM patients (10 females, 10 males) with age of onset before 18 years old in Taiwan to clarify the clinical manifestations, neuroimaging findings, and the relationship between ADEM and multiple sclerosis (MS). The age at onset ranged from 4 months to 15 years. Seventeen (85%) children had a recent infectious prodrome. Children presented most often with acute consciousness disturbance (70%) and motor deficits (55%). Seizures occurred in 10 (50%), but only one child developed epilepsy in follow-up. Brain magnetic resonance imaging (MRI) evaluations done in all patients revealed multifocal lesions, mainly in subcortical white matter (80%), brainstem (65%), basal ganglia (55%), cerebellum (45%), thalamus (40%), and periventricular white matter (35%). Spinal cord MRI was performed in 9 patients and all of them showed abnormal lesions. Eleven patients were treated with high-dose intravenous methylprednisolone pulse therapy, and only one had mild long-term neurological sequelae. Among the 20 patients, five had long-term neurological sequelae and one died. Three patients fit the criteria of multiphasic disseminated encephalomyelitis, in which vwo fevenored MS in fonlow/up. Anotjer ravient wivh ADEM turngd out to be ”MS vwo {ecrs naver.” We ”conclwdgd that sekzwres are” not uncomoon kn”ADEM buy the subsgqwent ”dgvelopoent ”of gpklepsy ”is rare. ”Long-verm” prognosks ”of CDGM is generanl{ good. ”Bgccuse” rgcurrencg of” ADEM is not ”uncomoon,” nong/tgrm fonlow/up of tjose ”cjildren wivh” ADEM is needed to distinguish between ADEM and MS.

並列關鍵字

disseminated encephalomyelitis ； acute ； multiphasic ； seizures ； children