急性瀰漫性腦脊髓炎：一病例報告

Acute Disseminated Encephalomyelitis: Report of a Case

急性瀰漫性腦脊髓炎(acute disseminated encephalomyelitis, ADEM)是一種去髓鞘(demyelination)疾病，主要侵犯中樞神經系統並產生急性、散播性的脫髓鞘炎性病變，患者以男性居多，好發於年輕人，甚少見於幼兒，尤其是小女孩。患者通常在感染或疫苗接種後發生，可能是免疫系統對髓鞘產生自體免疫反應所致，臨床症狀包括發燒、頭痛、瞻妄、意識改變、癲癇發作、頸部僵硬、視神經炎、急性橫截性脊髓炎、肢體無力等，大多數病人為單次發作。此案例是一名三歲五個月的女孩，主述發燒12天，合併咳嗽、流鼻水、以及雙側耳朵疼痛，初期診斷為支氣管肺炎及中耳炎，並給予紅黴素及amoxillin/clavulanate治療，使用抗生素數日後病人逐漸退燒，但神經學症狀卻出現異常，臨床上表現出雙側腳無力、右手無力且疼痛、以及頸部僵硬。黴漿菌抗體(IgM)檢驗呈陽性，腦脊髓液檢查正常，磁振造影檢查發現腦部有多處病灶，散布在大腦、小腦、腦幹、脊髓，最後診斷是黴漿菌感染關聯性急性瀰漫性腦脊髓炎(ADEM)，並使用靜脈注射免疫球蛋白(IVIG)治療。數日後她的神經學症狀逐步改善並出院，之後在門診追蹤，六個月後磁振造影檢查顯示腦部病灶已恢復正常，由於本病例是一名罕見之小女孩且與黴漿菌感染有關，特提出報告。

Acute disseminated encephalomyelitis is a type of post-infectious encephalomyelitis that occurs mostly in children and occasionally in adults. We report the case of a 3-year 5-month-old girl with bronchopneumonia and acute otitis media. She had neurological symptoms including bilateral leg weakness, right arm weakness, pain, and neck stiffness after defervescence of fever. Brain and spinal T2-weighted flair magnetic resonance images revealed multifocal demyelinating lesions with variable enhancement within the white and gray matter of cerebral hemispheres, cerebellum, brainstem, and spinal cord. The diagnosis of acute disseminated encephalomyelitis was made. Her neurological symptoms gradually improved two weeks after administration of intravenous immunoglobulin 0.5 g/kg/day for two days. Six months later, follow-up T2-weighted flair magnetic resonance images showed resolution of the demyelinating lesions in the brain parenchyma. We report a rare case of a little girl with acute disseminated encephalomyelitis.