一名对皮质类固醇反应良好的年轻女性患上急性出血性白质脑炎

IF 0.3 Q3 MEDICINE, GENERAL & INTERNAL
F. Nasim, M. Fasrina, Sunil Bowattage, Wickramasooriyage K. Kularatne, V. J. Kobbegala
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引用次数: 0

摘要

背景:急性出血性脑白质炎(AHLE)是急性播散性脑白质炎(ADEM)的一种罕见暴发型疾病,它具有与脑部受影响区域相对应的快速进展性多症状表现。其特点是认知能力迅速下降、昏迷甚至迅速死亡。据推测,这是一种感染后免疫介导的单相脱髓鞘疾病。临床诊断需要脑脊液和核磁共振成像结果的支持。在最初发病时,很难将该病与多发性脱髓鞘脑脊髓炎(MDEM)和多发性硬化症区分开来:在此,我们报告了一例 36 岁的斯里兰卡僧伽罗妇女的病例,她在发热后出现癫痫发作,随后出现严重的右侧偏瘫,后来发展为缄默症和认知能力下降。她的脑脊液显示多形性红细胞增多 12 个/立方厘米,多形性红细胞占 5%,蛋白质轻度偏高,葡萄糖正常,寡克隆抗体阳性。核磁共振成像显示多发局灶性脑室上、下信号异常,脑回增厚伴出血,最大的病灶位于左额叶区,有肿块效应,但无疝。她被诊断为AHLE,接受了大剂量静脉甲基泼尼松龙治疗,随后转为口服泼尼松龙减量疗法。根据脑膜脑炎的情况,她继续静脉注射头孢他啶和阿昔洛韦。使用类固醇药物后,病情明显好转,神经功能完全恢复,后遗症极少,随访 6 个月无复发:该病例是一种非常罕见的罕见疾病,临床上需要高度怀疑,并进行早期影像学检查,以采取积极的早期免疫抑制治疗,这样才有可能阻止疾病发展为常见的后遗症,包括昏迷、死亡或残留神经病。首次发病时对未来 MDEM 或 MS 的预测和重叠是需要进一步探讨的领域。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acute hemorrhagic leukoencephalitis in a young female with excellent response to corticosteroids
Background: Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant entity of acute disseminated leukoencephalitis (ADEM) that has a rapidly progressive polysymptomatology corresponding to areas of brain affected. It’s characterized by rapid cognitive decline, coma or even rapid death. It is postulated to be a post infective immune mediated monophasic demyelinating disease. Diagnosis is clinical supported by CSF and MRI findings. It is challenging to differentiate the disease from MDEM (multiphasic demyelinating encephalomyelitis) and multiple sclerosis during the initial presentation.Case presentation: Here we report a case of a 36-yearold Sri Lankan Sinhalese lady who presented with seizure following febrile illness followed by gross right sided hemiparesis later progressing to mutism and cognitive decline. Her CSF showed pleocytosis 12/cumm with 5% polymorphs, red cells, mildly high proteins with normal glucose and positive oligoclonal bands. MRI showed evidence of multiple focal supra and infratentorial signal abnormalities and gyral thickening with hemorrhages, largest lesions being in left frontal region with mass effect but no herniation. She was diagnosed with AHLE and treated with high dose IV methyl prednisolone later converting to oral prednisolone tapering regime. She was continued on intravenous cefotaxime and acyclovir as per meningoencephalitis. Improvement with steroids was dramatic with complete neurological recovery, minimal sequel and no recurrences at 6 months follow up.Conclusion: The case is a representation of a very rare entity of an infrequently reported disease which needs high degree of clinical suspicion with early imaging for aggressive and early immunosuppressive treatment that may hinder progression to commonly described sequel including coma, death or residual neurology. The predictions on future MDEM or MS on first presentation and overlap are areas that need further exploration.
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来源期刊
Ceylon Medical Journal
Ceylon Medical Journal MEDICINE, GENERAL & INTERNAL-
自引率
0.00%
发文量
16
期刊介绍: The Ceylon Medical Journal, is the oldest surviving medical journal in Australasia. It is the only medical journal in Sri Lanka that is listed in the Index Medicus. The CMJ started life way back in 1887 as the organ of the Ceylon Branch of the British Medical Association. Except for a brief period between 1893 and 1904 when it ceased publication, the CMJ or its forbear, the Journal of the Ceylon Branch of the British Medical Association, has been published without interruption up to now. The journal"s name changed to the CMJ in 1954.
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