Acute disseminating encephalomyelitis in neuromyelitis optica
Acute disseminating encephalomyelitis in neuromyelitis optica
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DOI：
10.1001/archneurol.2007.59
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发表时间：
2008-02-01
影响因子：
--
通讯作者：
Gotkine, Marc
中科院分区：
文献类型：
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作者：
Eichel, Roni;Meiner, Zeev;Gotkine, Marc
目的：报告2例合并重症急性播散性脑脊髓炎的视神经脊髓炎（NMO）患者的临床和影像学特征。第一个患者有抗水通道蛋白 4 抗体 (NMO-IgG)，但没有病灶增强，而第二个患者 NMO-IgG 血清阴性，但磁共振成像有明显的病灶增强。 设计：对 10 名表现出与 NMO 谱系疾病相一致特征的患者进行临床、实验室和放射学分析，其中 2 名患者出现急性播散性 地点：哈达萨大学神经内科住院病房。患者：一年内入院的具有 NMO 谱系疾病特征的患者。干预措施：回顾病史和影像数据，分析血清样本是否存在 NMO-IgG。主要结果指标：脑部受累的临床和临床旁证据。结果： 10 名患者接受检测，其中 5 名 NMO-IgG 呈阳性。一名血清阳性和一名血清阴性患者出现急性播散性脑脊髓炎样发作。在这两种情况下，临床、实验室和脑电图检查结果均支持急性播散性脑脊髓炎的诊断。磁共振成像显示两名患者均存在广泛的双侧白质病变。注射钆后，血清阳性患者的病灶明显缺乏强化，而血清阴性患者则观察到强烈的病灶强化。 结论：磁共振成像无病灶强化的急性播散性脑脊髓炎可能代表血清阳性 NMO 的儿童期表现。缺乏增强表明血脑屏障完整，并支持由于水通道功能障碍而诱发水肿的独特机制。
Objective: To report the clinical and radiological features of 2 patients with neuromyelitis optica (NMO) associated with severe acute disseminating encephalomyelitis. The first patient had anti-aquaporin 4 antibodies (NMO-IgG) but no lesion enhancement, in contrast to the second patient who was seronegative for NMO-IgG but had clear lesion enhancement on magnetic resonance imaging.Design: Clinical, laboratory, and radiological analysis of 10 patients presenting with features compatible with an NMO-spectrum disorder, 2 of whom developed acute disseminating encephalomyelitis.Setting: Inpatient ward at the Department of Neurology, Hadassah University.Patients: Patients admitted during a 1-year period with features compatible with an NMO-spectrum disorder.Interventions: Medical histories and imaging data were reviewed and serum samples were analyzed for the presence of NMO-IgG.Main Outcome Measures: Clinical and paraclinical evidence of brain involvement.Results: Of 10 patients tested, 5 were positive for NMO-IgG. One seropositive and 1 seronegative patient had an acute disseminating encephalomyelitis-like episode. In both cases, the clinical, laboratory, and electroencephalographic findings supported a diagnosis of acute disseminating encephalomyelitis. Magnetic resonance imaging demonstrated extensive bilateral white matter lesions in both patients. Lesions in the seropositive patient were notably lacking in enhancement following gadolinium injection, whereas robust lesion enhancement was observed in the seronegative patient.Conclusions: Acute disseminating encephalomyelitis without lesion enhancement on magnetic resonance imaging may represent a childhood manifestation of seropositive NMO. The lack of enhancement suggests an intact blood-brain barrier and supports a unique mechanism of edema induction due to dysfunction of water channels.