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幼年特发性关节炎与小儿原发性脱髓鞘疾病的并发。

Concurrence of juvenile idiopathic arthritis and primary demyelinating disease in a young child.

机构信息

Department of Pediatrics, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

出版信息

Mult Scler Relat Disord. 2019 Jan;27:20-22. doi: 10.1016/j.msard.2018.10.002. Epub 2018 Oct 3.

Abstract

CASE REPORT

The association of juvenile idiopathic arthritis (JIA) and primary demyelinating disease of central nervous system (CNS) in the same patient is rare. Here we present a 10-year-old girl formerly diagnosed with JIA who presented with acute total vision loss. Magnetic resonance imaging of the brain and spinal cord showed bilateral optic neuritis and T2 hyperintense lesions in the brain, cerebellum and cervical spinal cord, some of them gadolinium-enhancing. Oligoclonal bands were present in the cerebrospinal fluid. Visual evoked potentials were prolonged. Aquaporin-4 antibodies were negative. The patient was treated with methylprednisolone 30 mg/kg daily for five days, resulting in improvement in vision and gait. This first demyelinating event in this patient with JIA with clinical and paraclinical features meeting the 2017 MS diagnostic criteria supports a possible predisposition to autoimmune disorders.

CONCLUSION

The concurrence of JIA and multiple sclerosis (MS) has been reported in only two adult cases and not in the pediatric population. While JIA and MS are two distinct chronic inflammatory diseases, immunogenetic predisposition and common environmental triggers might be involved.

摘要

病例报告

幼年特发性关节炎(JIA)和中枢神经系统(CNS)原发性脱髓鞘疾病同时发生在同一位患者身上的情况较为罕见。我们在此报告了一位 10 岁的女孩,她曾被诊断为 JIA,目前出现了急性视力完全丧失。大脑和脊髓的磁共振成像显示双侧视神经炎和大脑、小脑及颈髓的 T2 高信号病变,其中一些病变增强了钆造影。脑脊液中存在寡克隆带。视觉诱发电位延长。水通道蛋白-4 抗体为阴性。患者接受了甲基泼尼松龙 30mg/kg/日的治疗,持续 5 天,视力和步态都有所改善。这位 JIA 患者的首次脱髓鞘事件具有符合 2017 年 MS 诊断标准的临床和辅助检查特征,支持其可能存在自身免疫疾病的易感性。

结论

只有两例成年病例报告了 JIA 和多发性硬化症(MS)同时发生,而在儿科人群中尚未有报道。虽然 JIA 和 MS 是两种不同的慢性炎症性疾病,但免疫遗传易感性和共同的环境触发因素可能与之相关。

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