Imbe Hisashi, Nakajima Hideto, Ito Takumi, Kitaoka Haruko
Department of Internal Medicine, Seikeikai Hospital.
Rinsho Shinkeigaku. 2010 Mar;50(3):168-71. doi: 10.5692/clinicalneurol.50.168.
We report the case of a 31-year-old woman who presented with neuromyelitis optica (NMO) associated with Sjögren syndrome and distal renal tubular acidosis. She was hospitalized because of cervical transverse myelopathy and right optic neuritis. She had been clinically diagnosed with Sj6gren syndrome, with a high titer of anti-SS-A antibody (1:500) and anti-SS-B antibody (1:498). She also showed hypokalemia, metabolic acidosis, and nephrocalcinosis caused by distal renal tubular acidosis associated with Sjögren syndrome. T2-weighted magnetic resonance imaging (MRI) revealed long lesions extending from the medulla oblongata to the lower thoracic cord. In addition, gadolinium-enhanced MRI revealed a right optic nerve lesion in the optic canal. High titer of anti-aquaporin-4 (AQP4) antibody was detected in the patient's serum (1:131,072). A combination therapy comprising steroid pulse therapy and plasmapheresis improved her clinical symptoms, and the administration of oral prednisolone (20 mg/ day) was effective in preventing the recurrence of NMO. In patients with myelitis/transverse myelopathy associated with autoimmune disorders such as Sjögren syndrome, examining the titer values of anti-AQP4 antibody is indispensable in determining the appropriate therapy.
我们报告了一例31岁女性患者，其患有与干燥综合征和远端肾小管酸中毒相关的视神经脊髓炎（NMO）。她因颈髓横贯性脊髓病和右眼视神经炎住院。她临床诊断为干燥综合征，抗SS - A抗体滴度高（1:500），抗SS - B抗体滴度高（1:498）。她还表现出低钾血症、代谢性酸中毒以及由与干燥综合征相关的远端肾小管酸中毒引起的肾钙质沉着症。T2加权磁共振成像（MRI）显示从延髓到胸髓下段的长节段病灶。此外，钆增强MRI显示视神经管内右侧视神经病变。在患者血清中检测到高滴度抗水通道蛋白4（AQP4）抗体（1:131,072）。包括类固醇冲击疗法和血浆置换的联合治疗改善了她的临床症状，口服泼尼松龙（20毫克/天）给药有效预防了NMO复发。在患有与自身免疫性疾病如干燥综合征相关的脊髓炎/横贯性脊髓病患者中，检测抗AQP4抗体滴度值对于确定合适的治疗方法不可或缺。
