TY - T1的临床表型的纯原发性侧索硬化症(S31.002) JF -神经学乔-神经学六世- 90 - 15补充SP - S31.002盟上路Om米塔尔AU -威廉·首页胡盟-基思·约瑟夫盟- j . Eric Ahlskog AU -埃里克·索伦森盟安哈哈桑Y1 - 2018/04/10 UR - //www.ez-admanager.com/content/90/15_Supplement/S31.002.abstract N2 -目的:描述纯主侧索硬化症的临床表型(PLS)背景:请是一种神经退行性综合征,其特征是皮质脊髓的/皮质延髓的症状/体征,消极的家族史,无肌电图的证据前角细胞involvementDesign /方法:回顾性的图表总结患者的临床诊断的请从1996 - 2016年在梅奥诊所,MN罗彻斯特。所有患者至少有一个正常的梅奥EMG四年后出现症状,请完成标准(普林格尔;戈登)结果:79请患者,48个正常肌电图,这个抽象的主题。去年EMG时间范围从4到17年从症状出现。请与小肌电图变化报告在一个单独的抽象。表型包括31名患者下肢发病(paraparetic类型);11 hemibody-onset (hemiparetic类型),而6始于bulbar-onset(球类型)。发作时期对所有类型范围从40到67年。在最常见的表型,paraparetic,平均随访11.6年(4 - 25年);26岁的患者进展从腿到上肢的意思是七年(1年)。 Bladder symptoms developed during follow-up in 17 patients, mean 9 years (0–20 years). Progression to dysarthria occurred in 14 patients mean 8.6 years, range 2–16 years. Dysphagia developed in seven patients after 5–9 years and pseudobulbar symptoms in 6 patients after 4–16 years. A cane was necessary in the majority of the paraparetic phenotype, whereas a minority needed a walker by mean 7.5 years; five were wheelchair-bound, mean 10.6 years (range, 7–16 years). In the hemparetic and bulbar phenotypes, progression to a cane, walker, wheelchair was somewhat similar in frequency.Conclusions: Pure PLS most commonly presents as an ascending paraparetic syndrome but also manifests asymmetrically (hemiparetic) or descending from bulbar onset. Among all the phenotypes, progression typically included dysarthria, dysphagia bladder and pseudobulbar symptoms.Disclosure: Dr. Mittal has nothing to disclose. Dr. Hu has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Hoffman LaRoche, Abbvie, AARP. Dr. Hu has received research support from Avid Radiopharmaceuticals, Fujirebio. Dr. Josephs has nothing to disclose. Dr. Ahlskog has nothing to disclose. Dr. Sorenson has nothing to disclose. Dr. Hassan has nothing to disclose. ER -