O'Riordan J I, Gallagher H L, Thompson A J, Howard R S, Kingsley D P, Thompson E J, McDonald W I, Miller D H
Department of Clinical Neurology, National Hospital of Neurology and Neurosurgery, London, UK.
J Neurol Neurosurg Psychiatry. 1996 Apr;60(4):382-7. doi: 10.1136/jnnp.60.4.382.
Since Devic's original description of neuromyelitis optica in 1894 there has been much debate regarding its aetiology. A specific cause has been identified in a minority of cases but in most the question has arisen whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This study was undertaken to help clarify this issue.
Neuromyelitis optica was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody status was documented. Patients underwent CSF examination and MRI of brain and spinal cord.
Twelve patients, with a mean age of presentation of 35.1 years, were seen. Eleven were women; vision was reduced to counting fingers or worse in 10 patients and seven became confined to a wheelchair. Examination of CSF showed local synthesis of oligoclonal bands in only two patients and a neutrophil pleocytosis in two. A possible aetiology was identified in five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and possible acute disseminated encephalomyelitis (two). Six had non-specific increases in various autoantibodies. Eleven patients underwent MRI of the brain and spinal cord. In 10 there were diffuse abnormalities involving cervical and thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in five; in five there were multiple deep white matter lesions, and one patient had minor age related changes.
It is proposed that Devic's neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI features different from those found in classic multiple sclerosis. In most cases a specific aetiology is not identified, but an immunological mechanism of tissue damage seems likely.
自1894年德维克首次描述视神经脊髓炎以来，关于其病因一直存在诸多争论。少数病例已确定了具体病因，但在大多数病例中，问题在于德维克的视神经脊髓炎是否为多发性硬化的一种变异型。本研究旨在帮助阐明这一问题。
视神经脊髓炎定义为：（1）严重横贯性脊髓炎；（2）急性单侧或双侧视神经病变；（3）无脊髓或视神经以外的临床受累；（4）单相或多相疾病。记录临床和自身抗体情况。患者接受脑脊液检查及脑和脊髓的磁共振成像（MRI）检查。
共观察到12例患者，平均发病年龄为35.1岁。其中11例为女性；10例患者视力降至仅能数指或更差，7例需依靠轮椅行动。脑脊液检查显示，仅2例患者存在寡克隆带局部合成，2例有中性粒细胞增多。5例确定了可能的病因：特定结缔组织病（2例）、肺结核（1例）、可能的急性播散性脑脊髓炎（2例）。6例各种自身抗体有非特异性升高。11例患者接受了脑和脊髓的MRI检查。10例患者颈段和胸段脊髓有弥漫性异常，急性期广泛肿胀。5例患者脑MRI正常；5例有多发深部白质病变，1例有与年龄相关的轻微改变。
有人提出，德维克的视神经脊髓炎是一种独特的疾病，具有一些与经典多发性硬化不同的临床、脑脊液和MRI特征。在大多数病例中未确定具体病因，但组织损伤的免疫机制似乎很可能存在。
