Tajima Yasutaka, Matsumura Mariko, Yaguchi Hiroaki, Mito Yasunori
Department of Neurology, Brain Science Center, Sapporo City General Hospital, Japan.
Intern Med. 2018;57(6):867-871. doi: 10.2169/internalmedicine.7153-16. Epub 2018 Mar 15.
A 27-year-old woman with optic neuritis and cervical myelitis developed hypertrophic demyelinating polyneuropathy. It was hypothesized that the diagnosis was combined central and peripheral demyelination. A hypertrophic nerve was observed subcutaneously, and magnetic resonance imaging demonstrated marked hypertrophy of the nerve roots. The patient was negative for anti-aquaporin 4 antibodies. Her anti-neurofascin 155 antibody levels was slightly elevated, but it was not definitely positive. Pulsed steroid therapy and the administration of immunoglobulin ameliorated her symptoms. Molecules in both the peripheral and central nervous systems might be target antigens, but further investigations will be needed to clarify the precise pathogenic mechanisms.
一名患有视神经炎和颈髓炎的27岁女性患上了肥厚性脱髓鞘性多发性神经病。据推测，诊断为中枢和周围脱髓鞘合并症。皮下观察到一条肥厚的神经，磁共振成像显示神经根明显肥大。该患者抗水通道蛋白4抗体检测为阴性。她的抗神经束蛋白155抗体水平略有升高，但并非明确阳性。脉冲类固醇疗法和免疫球蛋白治疗改善了她的症状。外周和中枢神经系统中的分子可能都是靶抗原，但需要进一步研究以阐明确切的致病机制。
