Neuromyelitis optica in a patient with an early onset demyelinating episode: Clinical and autoantibody findings
Neuromyelitis optica in a patient with an early onset demyelinating episode: Clinical and autoantibody findings
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DOI：
10.1016/j.clineuro.2007.08.018
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发表时间：
2007-12-01
影响因子：
1.9
通讯作者：
Klehmet, Juliane
中科院分区：
文献类型：
--
作者：
Beyer, Anja-Maria;Wandinger, Klaus-Peter;Klehmet, Juliane
最近的临床和实验室发现大大提高了我们对视神经脊髓炎（NMO）作为一种体液介导的自身免疫性疾病的认识。我们报告了一位在白喉-百日咳-破伤风（DPT）疫苗接种后6个月发生横贯脊髓炎的患者，因此被认为是急性播散性脑脊髓炎（ADEM）的提示。15年后，进一步的病程显示典型的NMO符合所有诊断标准。这一进展表明NMO具有广泛的临床和时间异质性，ADEM可能发生在共同的自身免疫素质背景下。尽管使用利妥昔单抗去除b细胞后出现了治疗反应，但NMO-IgG自身抗体的阳性状态保持不变，而抗水通道蛋白-4 （AQP-4）抗体的直接检测结果始终为阴性。我们的研究结果挑战了NMO-IgG的致病相关性，并表明NMO-IgG和aqp -4自身抗体的检测具有不同的诊断价值。(C) 2007 Elsevier B.V.版权所有
Recent clinical and laboratory findings have substantially advanced our understanding of neuromyelitis optica (NMO) as a humorally mediated, autoimmune disorder. We report on a patient who suffered a first episode of transverse myelitis at the age of 6 months following diphtheria-pertussis-tetanus (DPT) vaccination which had therefore been considered suggestive of acute disseminated encephalomyelitis (ADEM). Fifteen years later, the further disease course revealed typical NMO meeting all diagnostic criteria. This development points to a broad clinical and temporal heterogeneity of NMO, with ADEM probably occurring in the context of a shared autoimmune diathesis. Despite therapy response following B-cell depletion by rituximab, positive NMO-IgG autoantibody status remained unchanged, whereas direct testing for anti-aquaporin-4 (AQP-4)-antibodies was negative throughout. Our findings challenge the pathogenic relevance of NMO-IgG and indicate a varying diagnostic value of testing for NMO-IgG and AQP-4-autoantibodies. (C) 2007 Elsevier B.V. All rights reserved.