急性瀰散型腦脊髓炎後之復健：病例報告

Rehabilitation Problems in an Adult Patient after Acute Disseminated Encephalomyelitis: A Case Report

陳筱慧(Hsiao-Huei Chen) ； 謝霖芬(Lin-Fen Hsieh) ； 曾鳳菊(Feng-Chu Tseng)

《台灣復健醫學雜誌》 34卷4期 (2006/12) Pp. 247-253

https://doi.org/10.6315/2006.34(4)07

摘要

急性瀰散型腦脊髓炎(acute disseminated encephalomyelitis)是一種中樞神經去髓鞘疾患，好發於小孩和年輕人，在中老年人十分罕見。病患在發病的1到3星期以前，常有新近感染或接種疫苗。雖然確實的致病因子仍屬未知，但一般認為可能與T 細胞中介自體免疫反應有關。病灶可遍及腦白質、腦幹、小腦和脊髓，臨床表現多樣，通常預後良好。本篇報導的病例為一位49歲女性患者，突然發生左側肢體無力和言語吶吃，兩日內病情急遽惡化，演變成去皮質強直(decorticate rigidity)、呼吸衰竭且失去意識。理學檢查發現雙側第5、6、7、9、10、11、12 對腦神經均有麻痺且有巴賓斯基徵象(Babinski sign)。腦部磁振造影(magnetic resonance image)檢查發現有多部位去髓鞘病灶散佈於兩側大腦半球、視丘和腦幹。使用類固醇脈衝治療及復健治療之後，病人的各種症狀及功能，包括神智、呼吸功能、語言障礙、運動功能及神經性膀胱功能障礙等均有顯著改善。追蹤一年後患者已可獨立行走，且呼吸、吞嚥、膀胱等功能均恢復正常，只遺留頭痛、輕度言語吶吃及肌肉張力增強等後遺症。本文探討病患之症狀、診斷、鑑別診斷及復健，並回顧相關文獻資料，以作為臨床醫師處理此類病人的參考。

關鍵字

急性瀰散型腦脊髓炎 ； 磁振造影 ； 復健

並列摘要

Acute disseminated encephalomyelitis is a monophasic disorder of unknown pathogenesis, characterized by demyelination of the central nervous system. The possible T-cell immune-mediated inflammatory process, affecting mainly children and young adults, typically follows a recent infection or immunization. Meningeal symptoms are common early in the course and often begin 1-3 weeks after infection. Abnormalities of motor, sensory, gait, visual, and cognitive function are variable and depend on the location (cerebral white matter, brainstem, cerebellum, or spinal cord) of the most severely damaged areas of the nervous system. Acute disseminated encephalomyelitis is unusual in middle-aged or elderly adults. The mortality rate is high in the patients with severe cerebral damage after measles, rubella, or rabies vaccination. Sequelae include seizures, mental syndrome, muscular weakness, and atrophy. Although near complete recovery (over 90%) in young survivors is the hallmark of acute disseminated encephalomyelitis, its long-term effect on elderly patients is still in dispute owing to little experience. We presented here the case of a 49-year-old woman with bilateral congenital hip dislocation and chronic headache, who suddenly developed hoarseness and left-sided weakness. The full-bloom encephalopathic disturbances resulted in unresponsiveness, respiratory failure, and decorticate rigidity in two days. Physical examination revealed cranial nerve palsies, and bilateral positive Babinski sign and Hoffman sign. Her corneal reflexes, cough reflex, and swallowing reflex were all diminished. Oligoclonal bands were not found in her lumbar cerebrospinal fluid, and head computerized tomography (CT) did not show any abnormality. Brain magnetic resonance image (MRI) scans showed asymmetric areas of demyelinated plaques over the bilateral cerebral hemispheres, thalamus, and brainstem without mass effect. These findings are typical of the encephalitic form of acute disseminated encephalomyelitis. However, no obvious prior infections or vaccination were found. Methylprednisolone treatment and active rehabilitation much improved our middle-aged adult patient's clinical picture and functional status. After 1-year follow up, few residual lesions appeared in the brain MRI. The patient could walk independently with a walker, and her respiration, swallowing, and bladder functions returned to normal. Delayed post-encephalitic sequelae did not occur and her basic activities of daily living were totally independent. However, headache, mild dysarthria, and increased muscle tone remained. She was only partly able to perform the instrumental activities of daily living such as shopping because of poor endurance and calculation skill. The clinical features and rehabilitation problems related to acute disseminated encephalomyelitis are presented in this paper.

並列關鍵字

acute disseminated encephalomyelitis ； magnetic resonance image ； rehabilitation