在多模式康复和免疫疗法期间通过纵向成像监测晚发性拉斯穆森脑炎的疾病活动性

Q4 Immunology and Microbiology Clinical and Experimental Neuroimmunology Pub Date : 2024-07-17 DOI:10.1111/cen3.12805
Lucas C. Adam, Lana Gilly, Joerg Mueller, Joerg Wissel, Anatol Kivi
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引用次数: 0

摘要

拉斯穆森脑炎(Rasmussen's encephalitis,RE)是一种罕见的自身免疫性脑病,通常在儿童早期发病,引起单侧大脑皮层自身免疫性炎症,导致进行性神经功能缺损,尤其是局灶性癫痫发作。成人的晚发性变异型 RE 进展较慢,并表现出非典型特征。尽管进行了大量研究,但病因仍然难以捉摸,妨碍了准确诊断和治疗方案的选择。我们报告了一例经活检证实的晚发型RE病例,患者为一名71岁的男性,病程长达12年。在接受静脉注射免疫球蛋白治疗和免疫抑制治疗后,病情趋于稳定,临床评估和影像学检查均证实了这一点。起初,受影响的半球出现急性肿胀,随后经过多年的萎缩性脑病稳定为残留状态,对侧半球出现病灶性疾病征兆。多模式康复和免疫疗法减轻了脑萎缩,降低了信号增强。晚发性变异性RE康复治疗仍未得到充分发展,主要集中在症状管理和术后功能恢复方面。在临床实践中,纵向成像对于监测免疫疗法的反应至关重要。
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Longitudinal imaging for monitoring disease activity in late‐onset Rasmussen's encephalitis during multimodal rehabilitation and immune therapy
Rasmussen's encephalitis (RE) is a rare autoimmune encephalopathy typically manifesting in early childhood, causing unilateral autoimmune inflammation of the cerebral cortex, leading to progressive neurological deficits, notably focal epileptic seizures. The late‐onset variant of RE in adults progresses slower and presents atypical features. Despite extensive research, the etiology remains elusive, hindering accurate diagnosis and treatment options.We present a biopsy‐confirmed late‐onset variant of RE case in a 71‐year‐old man with a disease course of 12 years. After the initiation of intravenous immunoglobulin therapy and immunosuppressive treatment, disease stabilization was achieved, as evidenced by clinical assessments and imaging. Initially, the affected hemisphere swelled hyperacutely, followed by years of atrophic encephalopathy stabilizing into a residual state, with emerging focal disease signs in the contralateral hemisphere. Multimodal rehabilitation and immune therapy attenuated brain atrophy and reduced signal enhancement.Late‐onset variant of RE rehabilitation remains underdeveloped, focusing on symptom management and functional recovery post‐surgery. Longitudinal imaging is crucial for monitoring immune therapy response in clinical practice.
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来源期刊
Clinical and Experimental Neuroimmunology
Clinical and Experimental Neuroimmunology Immunology and Microbiology-Immunology and Microbiology (miscellaneous)
CiteScore
1.60
自引率
0.00%
发文量
52
期刊最新文献
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