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Neurology:Unverricht-Lundborg病病程以及进展因素研究

2017-09-21 zhangfan MedSci原创

Unverricht-Lundborg病肌阵挛认知障碍
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研究发现，确诊年龄越早，发生严重肌阵挛的时间越早，持续性癫痫是疾病症状加重的预测指标

近日研究人员对Unverricht-Lundborg病（EPM1，亦称波罗的海肌阵挛）的病程以及严重程度的危险因素进行了研究，对症状的时间过程以及疾病的进展因素进行了探讨。

研究对59名携带CSTB扩增突变的意大利患者进行特征回顾性评价，每5年对患者进行一次评估以考察其肌阵挛的累积概率随时间变化、相关的认知障碍以及工作能力障碍，利用时间等级检验和Cox多元比例风险回归模型考察早期因素的影响。

致残性肌阵挛一般发生在疾病确诊32年后，认知功能障碍发生较晚。发病12内会发生首次严重的肌阵挛，在致残性肌阵挛以及认知下降后超过10年会发生持久性癫痫。大部分患者在致残性肌阵挛以及认知下降之前即丧失了劳动能力。

研究发现，确诊年龄越早，发生严重肌阵挛的时间越早，持续性癫痫是疾病症状加重的预测指标。

原始出处：

Laura Canafoglia et al. Variable course of Unverricht-Lundborg disease Early prognostic factors. Neurology. September 20,2017.

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    2018-08-19 yinhl1978

    #Neurol#

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    2018-03-11 caobinglei8079

    #Unverricht-Lundborg病#

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    2018-03-05 surilei

    #ERR#

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    2017-09-23 方舒

    学习

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    2017-09-23 diushouji

    #ICH#

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