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目的:视神经脊髓炎(NMO)又称Devic病,是视神经和脊髓同时或相继受累的急性或亚急性病变。它是一种独立的疾病实体还是MS的一种亚型,仍不清楚。本研究通过比较一些NMO和脊髓型MS的病例的临床特点,来探讨两者的关系。方法:回顾性分析20例NMO患者和40例脊髓型MS患者,对两组的人口统计学、临床表现、实验室检查(CSF和MRl、EP)及临床预后等进行比较。结果:NMO患者的神经系统表现局限于视神经和脊髓,多呈现横贯性脊髓损害(P<0.001),而脊髓型MS多为不完全横贯性损害(P<0.001)。NMO组出院时EDSS评分为5.3+/-3.1,明显高于脊髓性MS组(2.2+/-2.3,P<0.001)。NMO组CSF细胞计数平均值明显大于脊髓型MS组(P<0.001),蛋白质水平前者高于后者,但缺乏统计学意义。头MRI异常见于12.5%的NMO患者和83.3%的脊髓型MS患者(P<0.001)。脊髓大型纵向融合病灶见于64.7%的NMO患者和21.6%的脊髓型MS患者(P=0.005)。NMO组常累及髓内中心(P=0.003),而MS组则多局限于侧后索(P=0.003)。NMO组VEP异常率达100%,明显高于MS组(38.5%,P<0.001)。BAEP异常率脊髓型MS组高于NMO组,并提示中枢受累(P=0.372)。NMO组可分为单相型和复发型。女性、发病年龄晚、索引事件间隔时间较长可能与NMO复发病程有关。单相型临床表现比复发型重,但长期预后较好,复发型常在3年内经过一系列复发导致严重残疾。结论:根据临床数据,NMO和脊髓型MS应被视为两个独立的疾病实体。NMO发病时年龄大,女性居多,临床表现严重,预后差。头和脊髓MRI、CSF及诱发电位的特征可以帮助区别两种疾病。但是由于MS和NMO都没有特异性的生化指标,发现这样的生化指标将大大推动这一疾病的诊断和研究。此外,应进行大量多中心、大样本的长期随访研究,来对NMO和MS的各种治疗方法进行系统评价。
OBJECTIVE: Optic neuromyelitis (NMO), also known as Devic disease, is an acute or subacute disease involving the optic nerve and spinal cord simultaneously or successively. Whether it is an independent disease entity or a subtype of MS remains unclear. This study by comparing some of the clinical features of cases of NMO and spinal cord MS, to explore the relationship between the two. Methods: Twenty patients with NMO and 40 patients with spinal cord MS were retrospectively analyzed. The demographics, clinical manifestations, laboratory tests (CSF, MR1, EP) and clinical outcomes were compared between the two groups. Results: The neurological manifestations of patients with NMO were limited to the optic nerve and spinal cord, mostly transversal spinal cord injury (P <0.001), while spinal cord MS was mostly incomplete transverse injury (P <0.001). The EDSS score at discharge of the NMO group was 5.3 +/- 3.1, significantly higher than that of the spinal cord MS group (2.2 +/- 2.3, P <0.001). The mean CSF cell count in NMO group was significantly higher than that in spinal cord MS group (P <0.001), and the former was higher than the latter, but it was not statistically significant. Head MRI abnormalities were found in 12.5% of NMO patients and 83.3% of patients with spinal cord MS (P <0.001). Large spinal longitudinal fusion lesions were found in 64.7% of NMO patients and 21.6% of patients with spinal cord MS (P = 0.005). The NMO group often involved the intramedullary center (P = 0.003), whereas the MS group was more limited to the posterior cords (P = 0.003). The abnormal rate of VEP in NMO group was 100%, which was significantly higher than that in MS group (38.5%, P <0.001). The abnormal rate of BAEP was higher in the MSM group than in the NMO group, suggesting that the central involvement (P = 0.372). NMO group can be divided into single-phase type and recurrent type. Females, late onset of disease, longer index events may be related to the course of NMO relapse. Single-phase clinical manifestations than the recurrence of heavy, but the long-term prognosis is good, recurrence often within a few years after a series of relapses lead to severe disability. CONCLUSIONS: Based on clinical data, NMO and spinal cord MS should be considered as two separate disease entities. NMO onset age, mostly women, severe clinical manifestations, the prognosis is poor. The characteristics of the head and spinal cord MRI, CSF and evoked potentials can help distinguish the two diseases. However, since neither MS nor NMO has specific biochemical indicators, it is found that such biochemical indexes will greatly promote the diagnosis and research of this disease. In addition, a large number of long-term, multicenter, large-sample follow-up studies should be conducted to systematically evaluate various treatment modalities for NMO and MS.