Fukazawa Toshiyuki
Department of Neurology, Nishimaruyama Hospital, Sapporo.
Rinsho Shinkeigaku. 2006 Nov;46(11):863-5.
Although the diagnosis of optico-spinal MS (OSMS) is solely based on the unique lesion distribution, the OSMS is clinically characterized by distinctive features which are mostly shared by the clinical characteristics of relapsing neuromyelitis optica (NMO). Conversely, Western investigators appear to consider relapsing NMO as a distinct entity from MS, and distinct characteristics and independent diagnostic criteria for NMO were proposed. However, the key characteristics of OSMS and NMO seem to be quite similar, and therefore the prototype of these disorders are identical which we would like to call OSMS/NMO. Most of the described characteristics of the OSMS/NMO appear to reflect the fulminant nature of each attack and the expansion of each lesion, which we called "attack-related severity". Recently, we found that clinical characteristics seem to be distinctively different between OSMS patients with and without longitudinally extending spinal cord lesions while clinical features of OSMS patients without extending spinal cord lesions are similar to those of CMS patients. To understand the pathomechanisms of OSMS and NMO, the "attack-related severity" must be an important key factor as well as the unique lesion distribution.
虽然视神经脊髓型多发性硬化症(OSMS)的诊断完全基于独特的病变分布,但OSMS的临床特征具有独特性,这些特征大多与复发型视神经脊髓炎(NMO)的临床特征相同。相反,西方研究者似乎认为复发型NMO是一种与MS不同的疾病实体,并提出了NMO的独特特征和独立诊断标准。然而,OSMS和NMO的关键特征似乎非常相似,因此我们将这些疾病的原型称为OSMS/NMO。OSMS/NMO所描述的大多数特征似乎反映了每次发作的暴发性本质和每个病变的扩展,我们将其称为“发作相关严重程度”。最近,我们发现有纵向延伸脊髓病变和无纵向延伸脊髓病变的OSMS患者的临床特征似乎有明显差异,而无脊髓病变延伸的OSMS患者的临床特征与慢性多发性硬化症(CMS)患者相似。为了理解OSMS和NMO的发病机制,“发作相关严重程度”以及独特的病变分布必定是重要的关键因素。