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Encephalomyelitis associated with rheumatoid arthritis: a case report
Folia Neuropathologica ( IF 2 ) Pub Date : 2021-03-05 , DOI: 10.5114/fn.2021.103805
Albert Acewicz 1 , Teresa Wierzba-Bobrowicz 1 , Łukasz Michałowski 2, 3 , Mariola Pęcak 4 , Sylwia Tarka 1, 5 , Dominik Chutorański 1 , Tomasz Stępień 1 , Paulina Felczak 1 , Katarzyna Sklinda 6 , Anna Nasierowska-Guttmejer 2, 7 , Małgorzata Dorobek 4
Affiliation  

Encephalitis/encephalomyelitis in the course of rheumatoid arthritis (RA) remains a matter of debate. We present a case of a patient with encephalomyelitis associated with RA confirmed with post-mortem neuropathological examination. A 68-year-old woman with a long-standing, seropositive history of RA presented progressive disturbances of consciousness. Magnetic resonance imaging (MRI) of the brain and cervical spine revealed an increase of signal intensity on T2-weighted and fluid attenuated inversion recovery (FLAIR) images with corresponding restricted diffusion involving cerebral peduncles, pons, medulla oblongata, and cervical spinal cord and mild contrast-enhancement of the right cerebral peduncle. Extensive radiological and laboratory testing, including autoantibodies to paraneoplastic anti-neuronal and neuronal cell surface antigens, were all negative except for elevated rheumatoid factor. Cerebrospinal fluid (CSF) analysis revealed moderate pleocytosis with mononuclear cell predominance, mildly increased protein level, and negative viral PCRs, bacterial cultures, flow cytometry, and neuronal surface antibodies. Despite intensive treatment with corticosteroids, antibiotics, antiviral drugs, and intravenous immunoglobulin the patient died after 3 months of hospitalization. Post-mortem neuropathological examination revealed numerous, disseminated, heterochronous ischaemic lesions, rarely with haemorrhagic transformation, predominantly in the brainstem, and widespread, diffuse microglia and T-cell infiltrations with neuronal loss and astrogliosis, most severe in the frontal and temporal lobes. Mild, perivascular lymphocyte T infiltrations involved particularly small and medium-sized vessels and were associated with brainstem ischaemic lesions. The neuropathological picture confirmed diagnosis of encephalomyelitis, which together with the clinical course suggested association with RA. Concluding, encepha­lomyelitis due to RA remains a challenging, controversial entity that needs further research and the establishment of effective diagnostic and treatment guidelines.

中文翻译:

类风湿性关节炎相关脑脊髓炎1例报告

类风湿性关节炎 (RA) 病程中的脑炎/脑脊髓炎仍然是一个有争议的问题。我们介绍了一例与 RA 相关的脑脊髓炎患者,经验尸神经病理学检查证实。一名 68 岁女性,有长期的 RA 血清阳性病史,出现进行性意识障碍。脑和颈椎的磁共振成像 (MRI) 显示 T2 加权和液体衰减反转恢复 (FLAIR) 图像上的信号强度增加,相应的弥散受限,涉及脑脚、脑桥、延髓和颈脊髓以及轻度右脑脚对比增强。广泛的放射学和实验室测试,包括针对副肿瘤性抗神经元和神经元细胞表面抗原的自身抗体,除类风湿因子升高外均为阴性。脑脊液 (CSF) 分析显示中度细胞增多症,单核细胞占优势,蛋白质水平轻度升高,病毒 PCR、细菌培养、流式细胞术和神经元表面抗体呈阴性。尽管接受了皮质类固醇、抗生素、抗病毒药物和静脉注射免疫球蛋白的强化治疗,但患者在住院 3 个月后死亡。死后神经病理学检查显示大量播散性异时性缺血性病变,很少伴有出血性转化,主要发生在脑干,广泛的弥漫性小胶质细胞和 T 细胞浸润伴神经元丢失和星形胶质细胞增生,额叶和颞叶最为严重。温和的,血管周围淋巴细胞 T 浸润特别涉及中小血管,并与脑干缺血性病变有关。神经病理学图片证实了脑脊髓炎的诊断,这与临床病程一起表明与 RA 相关。总之,RA 引起的脑脊髓炎仍然是一个具有挑战性的、有争议的实体,需要进一步研究并建立有效的诊断和治疗指南。
更新日期:2021-04-13
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