TY - T1的多系统萎缩表现为运动神经元病(P6.069) JF -神经学乔-神经学六世- 90 - 15补充SP - P6.069 AU -劳伦·琼斯盟蕾妮·纳尔逊AU -首页詹姆斯·鲍尔AU -约瑟夫松本盟Paola Sandroni AU -爱德华多Benarroch AU -马里亚纳苏亚雷斯AU -菲利普低非盟-沃尔夫冈歌手盟伊丽莎白浣熊Y1 - 2018/04/10 UR - //www.ez-admanager.com/content/90/15_Supplement/P6.069.abstract N2 -目的:研究MSA患者最初被诊断患有运动神经元疾病,并确定这是否影响诊断和MSA的进程。背景:上、下运动神经元的存在多系统萎缩(MSA)的迹象指出因为害羞和德尔格强调痉挛状态和自发性收缩早期的描述;然而,运动神经元疾病的存在可能导致MSA的误诊。设计/方法:所有患者诊断为MSA在梅奥诊所,罗切斯特和自主功能测试在1998年和2012年之间进行回顾性综述。患者,最初指运动神经元疾病的诊断是相比与其他初始诊断。结果:从685年MSA患者,8最初诊断为肌萎缩性脊髓侧索硬化症,主侧硬化,或为运动神经元病。六个病人的最终诊断为MSA-parkinsonism, 2 MSA-cerebellar共济失调。2例尸检了,确认MSA。出现症状是电动机在6例包括腿的弱点,瀑布、构音障碍、吞咽困难。7个患者肌电图;2演示了颤和/或束状势而1抽筋排放。 The mean time from symptom onset to diagnosis for those initially diagnosed with motor neuron disease was 2.97 years compared to 4.148 years in all MSA patients (p = 0.0765). In patients with an initial motor neuron disease diagnosis, those with EMG findings had a mean time to MSA diagnosis of 4.11 years compared to 2.28 with normal EMGs (p = 0.0773). Overall survival for patients with an initial diagnosis of motor neuron disease was 4.67 years compared 7.61 years in all other MSA patients ( p = 0.0101).Conclusions: Our findings suggest that MSA patients may initially present with subtle findings of motor neuron disease. Overall, the few patients presenting with an initial diagnosis of motor neuron disease had shorter survival.Disclosure: Dr. Jones has nothing to disclose. Dr. Nelson has nothing to disclose. Dr. Bower has nothing to disclose. Dr. Matsumoto has nothing to disclose. Dr. Sandroni has nothing to disclose. Dr. Benarroch has nothing to disclose. Dr. Suarez has nothing to disclose. Dr. Low has nothing to disclose. Dr. Singer has nothing to disclose. Dr. Coon has nothing to disclose. ER -