DEVICS-NEUROMYELITIS-OPTICA - A CLINICOPATHOLOGICAL STUDY OF 8 PATIENTS
DEVICS-NEUROMYELITIS-OPTICA - A CLINICOPATHOLOGICAL STUDY OF 8 PATIENTS
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DOI：
10.1002/ana.410340211
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发表时间：
1993-08-01
影响因子：
11.2
通讯作者：
KORNFELD, M
中科院分区：
文献类型：
--
作者：
MANDLER, RN;DAVIS, LE;KORNFELD, M
我们报告了8例Devic视神经肌萎缩症的临床、影像学和实验室特征。所有患者均有严重的脊髓病和视神经炎。没有患者的大脑、脑干或小脑受到影响，即使是在患病数年后。各种免疫抑制治疗未能使患者受益，其中5人死亡。这5例患者的尸检显示了严重的坏死性脊髓病，血管壁增厚，无淋巴细胞浸润。在适当的临床环境中，头部磁共振成像显示白色物质异常的缺乏有助于在生活中识别Devic综合征。由于Devic氏脊髓病是坏死性的，而不是脱髓鞘性的，这种综合征的预后很差。
We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to benefit the patients, 5 of whom died. Autopsies of these 5 patients demonstrated a severe necrotizing myelopathy with thickening of blood vessel walls and no lymphocyte infiltrates. In the appropriate clinical setting, the lack of white matter abnormalities demonstrated by magnetic resonance imaging of the head facilitates the recognition of Devic's syndrome during life. Inasmuch as Devic's myelopathy is necrotizing, rather than demyelinating, the prognosis of this syndrome is poor.