Fardet L, Généreau T, Mikaeloff Y, Fontaine B, Seilhean D, Cabane J
Service de Médecine Interne, pavillon de l'Horloge 2 étage, Centre Hospitalier Universitaire Saint Antoine, Paris, France.
Acta Neurol Scand. 2003 Sep;108(3):193-200. doi: 10.1034/j.1600-0404.2003.02178.x.
Multiple sclerosis (MS) is by far the most popular diagnosis for patients with multifocal neurological disease. Owing to demyelinating inflammatory non-necrotic plaques of the white matter, MS can give remitting symptoms of virtually every part of the central nervous system. Corticosteroids are usually helpful. Devic's neuromyelitis optica (DNMO) is a neurological disease involving only the optic nerves and the spinal cord, where demyelination evolves towards necrosis and atrophy; the prognosis is poor and no satisfactory treatment is known. The objectives of this study are to describe clinical, biological, pathological and radiological data of patients with DNMO and to differentiate DNMO from MS.
We studied the files of 14 patients diagnosed with possible DNMO in three French hospitals between 1980 and 1999 and reviewed the literature.
Nine patients were included as definite DNMO. Five were excluded because they did not fulfil the diagnostic criteria. For the nine patients with definite DNMO, DNMO was either monophasic or multiphasic. The prognosis was generally poor: two patients died and five others developed severe disability such as blindness, para or quadriplegia or both. Cerebrospinal fluid study and neuroimaging were essential to confirm the diagnosis of DNMO. Various immunosuppressive treatments generally failed to benefit the patients.
In the literature (as well as our 14 initial patients) only a few cases of patients described as suffering from DNMO fulfilled the diagnostic criteria. The others showed evidence that another disease like MS was involved. We stress that inclusion and exclusion criteria have to be kept in mind to differentiate clearly DNMO from MS and other central nervous system white matter diseases.
到目前为止，多发性硬化症（MS）是多灶性神经疾病患者最常见的诊断。由于白质的脱髓鞘炎性非坏死性斑块，MS可导致中枢神经系统几乎每个部位出现缓解性症状。皮质类固醇通常有帮助。视神经脊髓炎（DNMO）是一种仅累及视神经和脊髓的神经疾病，其中脱髓鞘会发展为坏死和萎缩；预后较差，且尚无令人满意的治疗方法。本研究的目的是描述DNMO患者的临床、生物学、病理学和放射学数据，并将DNMO与MS区分开来。
我们研究了1980年至1999年间在三家法国医院诊断为可能患有DNMO的14例患者的病历，并回顾了相关文献。
9例患者被确诊为DNMO。5例因不符合诊断标准而被排除。对于9例确诊为DNMO的患者，DNMO可为单相或多相。总体预后较差：2例患者死亡，另外5例出现严重残疾，如失明、截瘫或四肢瘫或两者皆有。脑脊液检查和神经影像学检查对于确诊DNMO至关重要。各种免疫抑制治疗通常对患者无效。
在文献中（以及我们最初的14例患者中），只有少数被描述为患有DNMO的患者符合诊断标准。其他患者显示有证据表明涉及另一种疾病，如MS。我们强调，必须牢记纳入和排除标准，以明确区分DNMO与MS及其他中枢神经系统白质疾病。
