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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Spinal cord biopsy findings of anti-aquaporin-4 antibody-negative recurrent longitudinal myelitis in a patient with sicca symptoms and hepatitis C viral infection.
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Spinal cord biopsy findings of anti-aquaporin-4 antibody-negative recurrent longitudinal myelitis in a patient with sicca symptoms and hepatitis C viral infection.

机译：患有干燥症状和丙型肝炎病毒感染的患者抗aquaporin-4抗体阴性的复发性纵向脊髓炎的脊髓活检结果。

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摘要

We describe the pathological features of a spinal cord biopsy from a 69-year-old woman with anti-aquaporin-4 antibody-negative recurrent longitudinal myelitis. Spinal cord MRI showed T2 high-intensity lesions with strong gadolinium enhancement, when episodes of sensory-motor impairment were repeated. The radiological abnormality was corrected by corticosteroid administration, but improvement of the symptoms was minimal. Although the patient had sicca symptoms and fulfilled four of the diagnostic criteria for Sjogren syndrome, the diagnosis was excluded, because of infection with hepatitis C virus, an exclusion criterion of Sjogren syndrome. In the spinal cord lesions, necrotic changes affected both myelin and axons. Infiltrating lymphocytes were predominantly T-cells. The proliferation of small vessels with hyalinization and concomitant occlusive change was remarkable. These pathological findings resembled those previously reported in Sjogren syndrome. Ultrastructurally, the endothelial cells of the small vessels showed features of activated cells and contained vesiculo-tubular structures in the cytoplasm, indicating that increased blood-brain barrier (BBB) permeability might contribute to pathogenesis. We speculated that increased BBB permeability and T-cell entry in the spinal parenchyma triggered pathological reactions resulting in necrotic changes in the spinal cord. Obstruction of small vessels might add ischemic damage to the lesions. The clinical course and pathological findings indicated that damage progressed rapidly in the spinal cord and was irreversible. The lesions apparently differed from typical demyelination plaques. Faced with such spinal cord lesions, a preventive therapeutic approach is necessary to avoid attack-associated disability.
机译：我们描述了一个69岁的妇女与抗水通道蛋白4抗体阴性的复发性纵向脊髓炎的脊髓活检的病理特征。脊髓MRI显示，当感觉运动障碍反复发作时，T2高强度病变伴有strong增强。皮质类固醇激素可以纠正放射学异常，但症状的改善很小。尽管患者出现干燥症状，并且符合干燥综合征的四项诊断标准，但由于感染了丙型肝炎病毒而排除了诊断，这是干燥综合征的一项排除标准。在脊髓病变中，坏死变化影响髓磷脂和轴突。浸润淋巴细胞主要是T细胞。透明化和伴随闭塞变化的小血管增生是显着的。这些病理发现类似于先前在干燥综合征中报道的那些。在超微结构中，小血管的内皮细胞表现出活化的细胞特征，并在细胞质中包含水泡管结构，表明血脑屏障（BBB）通透性增加可能有助于发病。我们推测，BBB通透性增加和T细胞进入脊髓实质触发了病理反应，导致脊髓坏死性改变。小血管阻塞可能会增加病变的缺血性损害。临床过程和病理结果表明，损伤在脊髓中进展迅速，并且是不可逆的。病变明显不同于典型的脱髓鞘斑。面对这样的脊髓损伤，必须采取预防性治疗方法来避免与发作有关的残疾。

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