- 期刊
Moyamoya Disease Initially Mimicking MELAS Syndrome in a 14-Month-Old Child
一個十四個月大毛樣毛樣病兒童最初表現擬似爲MELAS症候群
摘要
毛樣毛樣病在兒科病人是很罕見的神經學疾病。臨床上，非外傷性缺血性中風兒科病人，在早期階段要去鑑別診斷是毛樣毛樣病或是其他粒腺體疾病，是很困難的。作者報告一位14個月大台灣女童，在住院前突然有非外傷性抽慉發作，及進行式左肢體無力。最初實驗室及腦部檢查懷疑是粒腺體疾病，尤其是MELAS症候群。病人接受口服葡萄糖乳酸激發試驗，結果並不攷慮是粒腺體疾病。病人接受核磁區振血管EDAMS手術治療。目前長期神經發展及智慧預後，尚需密切追蹤評估。
並列摘要
Moyamoya disease is a neurological disease rarely seen in children outside Japan. It is diffficult to differentiate moyamoya disease in its early stage from mitochondrial disorders in children when nontraumatic ischemic stroke is considered. We present a 14-month-old Taiwanese female child who had suffered from sudden onset of nontraumatic seizure attack, after which progressive left limb weakness was noted. Initial brain imaging and a series of laboratory studies were performed which suggested mitochondrial disorders, especially mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome. The patient underwent an oral glucose lactate stimulation test (OGLST), and mitochondrial disorders were not favored. MR angiography of the circle of Willis showed moyamoya disease. The patient then underwent an encephalo-duro-arterio-myo-synangiosis (EDAMS) operation. Evaluation of the neurodevelopmental and intelligence outcome required close long-term follow-up.