Clinical Observation in Childhood Myasthenia Gravis

孩童肌無力之臨床觀察

何美美(Meei-Meei Her) ； 沈友仁(Yu-Zen Shen)

《Acta Paediatrica Sinica》 28卷6期 (1987/12) Pp. 438-446

摘要

肌無力症是神經傳導物質乙醯胺在神經肌肉接合處發生傳導障礙所致，大多數的肌無力症發生於成人，屬於自體免疫疾病，有關孩童肌無力症之研究則比較少。作者收集1976年到1985年臺大醫院診斷肌無力症孩童病患共59例，平均追綜檢查時間為4.1年。發病年齡相當早，平均為5.8歲，有10位發病於2歲以前。發生症狀時，有15位病童看小兒科，29位看神經科，15位看眼科，看兒科之病童年齡大多小於4歲。
全身型肌無力病人共16位，其中6位發作初即有全身症狀，另10位是由眼型，轉變為全身型其轉變時間大都在發病後二年內。三位病童有家族史，其中二位是姊弟關係，發病年齡分別為5個月及1歲，另一位為11歲女孩，其姊姊也在16歲發生肌無力症。7位病童有其他合併疾病，其中5位有甲狀腺機能亢進。
眼型病人對抗乙醯胺酵素藥物及類固醇治療反應良好，有51%在平均10.5個月發生緩解，但71%很快於平均15個月時再發。類固醇藥物大多會發生副作用，很多病人因此而停藥。全身型肌無力病人對藥物反應也良好，但大多需合併使用上二種藥物，全部病人中共7位接受胸腺摘除，其中眼型2位，全身型5位，摘除時間平均為發病後2.8年。手術後效果欠佳，僅有2位達到緩解，其中1位1年後再發，3位沒有改善。胸腺組織報告和疾病嚴重與否沒有關係。
由以上研究可知孩童肌無力症大多以眼型表現，發病年齡相當早，臨床對抗乙醯胺酵素之藥物反應和類固醇一樣好，但緩解時間不長，大多需長期服藥，病情穩定但不易斷根。

並列摘要

A retrospective study of 59 children (29 boys and 30 girls) with myasthenia gravis was carried out in observation periods from 1 to 14 years (4.1 years average). Onset of disease was at an average 5.8 years. Ten children had presentations before two years of age. Ninety percent had ocular myasthenia on presentation. Ophthalmoplegia followed ptosis between one month and three years in 17 cases. Over a quarter (27%) of all patients eventually had generalized myasthenia. Ptosis responded well to anticholineserase and/or prednisolone. Remission occurred in 51% of the solely ocular myasthenia but 71% of these suffered relapse, all confined to ocular muscles. Seven patients received thymectomy 2.8 years (5 month to 6.5 years) after onset of symptoms. Two cases had remission after thymectomy, but one relapsed one year later. There seemed to be no correlation between thymic histology and severity of disease. There was familial occurrence of myasthenia gravis in three cases. Hyperthyroidism was found in five (8.5%). It is concluded that there is a benign but persistent clinical course in childhood myasthenia gravis.

並列關鍵字

myasthenia gravis ； ocular form ； generalized form ； remission ； thymectomy