Misu Tatsuro, Fujihara Kazuo, Nakashima Ichiro, Miyazawa Isabelle, Okita Naoshi, Takase Sadao, Itoyama Yasuto
Department of Neurology, Tohoku University School of Medicine, Sendai, Japan.
Brain. 2002 Nov;125(Pt 11):2460-8. doi: 10.1093/brain/awf260.
We evaluated the clinical and laboratory features of the optic-spinal form of multiple sclerosis (OSMS) with no brain lesions on repeated MRI--termed pure OSMS. By reviewing the medical records of 118 Japanese clinically definite multiple sclerosis patients seen between 1988-1999, we found 10 patients (8.5%), nine of whom were women, with only relapsing optic neuritis (ON) and myelitis (MY) clinically and consistently normal brain MRI during follow-ups of >or=5 years. Three patients suffered severe ON and MY, but the other seven had mild disease (six were graded 1 in the Disability Status Scale). Despite frequent relapses, mild pure OSMS was characterized by younger onset and mild spinal symptoms as in 'benign' classical multiple sclerosis (CMS). MRI often revealed multiple cervico-thoracic cord lesions of variable lengths. Oligoclonal IgG bands (OB) were negative in all cases. HLA-DPB10501, whose association with OSMS has been reported, was positive only in six patients (including three patients with severe pure OSMS). Four patients with DRB11501-DQB1*0602, to which CMS is closely linked, had mild disease. Though pure OSMS was heterogeneous with regard to clinical severity and human leukocyte antigen (HLA) class II alleles, this form of multiple sclerosis was characterized by a definite female preponderance and negative OB that distinguished it from CMS.
我们评估了在多次磁共振成像（MRI）上无脑病变的视神经脊髓型多发性硬化症（OSMS）——即纯OSMS——的临床和实验室特征。通过回顾1988年至1999年间诊治的118例日本临床确诊的多发性硬化症患者的病历，我们发现了10例患者（8.5%），其中9例为女性，在随访≥5年期间临床上仅有复发性视神经炎（ON）和脊髓炎（MY），且脑部MRI始终正常。3例患者患有严重的ON和MY，但其他7例病情较轻（6例在残疾状态量表中评分为1级）。尽管频繁复发，但轻度纯OSMS的特点是起病年龄较轻，脊髓症状较轻，如同“良性”经典型多发性硬化症（CMS）。MRI常显示多条不同长度的颈胸段脊髓病变。所有病例的寡克隆IgG带（OB）均为阴性。据报道与OSMS相关的HLA - DPB10501仅在6例患者中呈阳性（包括3例严重纯OSMS患者）。4例携带与CMS密切相关的DRB11501 - DQB1*0602的患者病情较轻。尽管纯OSMS在临床严重程度和人类白细胞抗原（HLA）II类等位基因方面具有异质性，但这种多发性硬化症的特点是女性明显占优势且OB阴性，这使其有别于CMS。
