The clinical features of combined central and peripheral demyelination in Chinese patients
The clinical features of combined central and peripheral demyelination in Chinese patients
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DOI：
10.1016/j.jneuroim.2018.02.006
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发表时间：
2018-04-15
影响因子：
3.3
通讯作者：
Li, Hong-Lei
中科院分区：
文献类型：
--
作者：
Wang, Yan-Qin;Chen, Han;Li, Hong-Lei
背景资料：中枢和外周联合脱髓鞘（Combined central and peripheral demyelination，CCPD）是一种少见的疾病，在中国汉族人群中尚未见报道。结果：CCPD患者的临床表现为感觉障碍（86.4%）、肢体瘫痪（77.3%）、颅神经受累（77.3%）、深腱反射异常（72.7%）。CSF数据显示81%的患者CSF蛋白升高。寡克隆IgG条带（08）为阴性。主要累及皮质或前皮质、脑室周围、幕下病变、胸颈脊髓。视觉诱发电位提示视神经脱髓鞘占50%。21例CCPD患者静脉注射丙种球蛋白或激素或两者联合治疗，有效率分别为33.3%、54.5%、71.4%。1例对激素加静脉免疫球蛋白治疗无反应，经环磷酰胺治疗后明显改善。结论：CCPD是一种谱性疾病，不能简单地认为是MS和CIDP的结合。疑似CCPD应接受脑和脊髓MRI以及电生理检查，以获得准确的诊断。
Background: Combined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population.Objectives: To study the clinical features of CCPD in Han Chinese patients.Methods: Twenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis.Results: CCPD patients presented with sensory disturbance (86.4%), plegia (77.3%), cranial nerve involvement (77.3%), abnormal deep tendon reflexes (72.7%). CSF data showed increased CSF protein in 81% patients. Oligoclonal IgG bands (08) were negative. Cortical or juxtacortical, periventricular, infratentorial lesions, thoracic and cervical spinal cord were mostly affected. Visual evoked potentials indicated optic nerves demyelinating in 50% cases. 21 CCPD patients were treated with intravenous immunoglobulin or steroids or both of them, and the efficacy was 33.3%, 54.5%, 71.4%, respectively. One case that showed no response to steroids plus intravenous immunoglobulin treatment was improved significantly after using cyclophosphamide.Conclusions: CCPD is a spectrum disease that can't be regarded as a simple combination of MS and CIDP. A suspected CCPD should receive brain and spinal MRI as well as electrophysiological examination to obtain a precise diagnosis.