Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report.
Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report.
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DOI：
10.1186/1471-2377-9-56
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发表时间：
2009-10-23
期刊：
影响因子：
2.6
通讯作者：
Nankovic S
中科院分区：
文献类型：
--
作者：
Petelin Gadze Z;Hajnsek S;Basic S;Sporis D;Pavlisa G;Nankovic S
视神经肌炎（NMO）是一种特发性、严重的中枢神经系统炎性脱髓鞘疾病，可引起严重的视神经炎和肌痉挛发作。多发性硬化症（MS）和NMO之间的早期鉴别是重要的，因为这两种疾病的最佳治疗可能有很大差异。我们报告一个病例，最初表现为纵向广泛性横肌麻痹（LETM），有痉挛性上肢轻瘫和痉挛性截瘫，C2/C3感觉水平和尿失禁，以及广泛的炎性脊髓病变，从C2水平到圆锥。5个月后，患者再次发生横肌痉挛，电生理学检查结果与视神经炎一致，NMO-IgG（水通道蛋白-4 IgG）血清阳性，因此符合NMO诊断标准。在用皮质类固醇冲击疗法和静脉注射免疫球蛋白治疗疾病发作后，我们在治疗中包括口服硫唑嘌呤联合口服泼尼松。由于没有明显的临床改善，我们决定使用环磷酰胺治疗，这导致了良好的临床改善和脐带肿胀逐渐减少。在这个NMO病例报告中，我们想强调我们的患者从C2水平到圆锥的脊髓炎性变化的广泛性。在结论中，重要的是要说，准确，早期诊断和MS的区别是至关重要的，以促进免疫抑制治疗的攻击预防开始。
Neuromyelitis optica (NMO) is an idiopathic, severe, inflammatory demyelinating disease of the central nervous system, that causes severe optic neuritis and myelitis attacks. Early discrimination between multiple sclerosis (MS) and NMO is important, as optimum treatment for both diseases may differ considerably. We report a case of a patient who initially presented as longitudinally extensive transverse myelitis (LETM), having spastic upper extremities diparesis and spastic paraplegia, C2/C3 sensory level and urinary incontinence, as well as extensive inflammatory spinal cord lesions from C2 level to conus. After 5 months the patient had another attack of transverse myelitis, had electrophysiological findings consistent with optic neuritis, was seropositive for NMO-IgG (aquaporin-4 IgG) and thus fulfilled NMO diagnostic criteria. Following treatment of disease attacks with pulse corticosteroid therapy and intravenous immunoglobulins, we included oral azathioprine in a combination with oral prednisone in the therapy. Since there was no significant clinical improvement, we decided to use cyclophosphamide therapy, which resulted in good clinical improvement and gradual decrease of cord swelling. In this NMO case report we wanted to emphasize the extensiveness of inflammatory spinal cord changes in our patient, from C2 level to conus. In the conclusion it is important to say that accurate, early diagnosis and distinction from MS is critical to facilitate initiation of immunosuppressive therapy for attack prevention.