TY - T1的临床表型原发性侧索硬化症与小肌异常(P4.462) JF -神经学乔-神经学六世- 90 - 15补充SP - P4.462 AU - 11米塔尔盟-威廉·胡AU -基思·约瑟夫盟j . Eric Ahlskog AU -埃里首页克·索伦森盟安哈哈桑Y1 - 2018/04/10 UR - //www.ez-admanager.com/content/90/15_Supplement/P4.462.abstract N2 -目的:描述原发性侧索硬化症(PLS)的临床表型与小肌异常。背景:请皮质脊髓的/皮质延髓的障碍没有临床症状较低的运动神经元参与长期随访。然而,小,稳定肌电图异常有时记录。设计/方法:Restrospective图表回顾最后请诊断患者从1996 - 2016年在梅奥诊所,MN罗彻斯特。所有的病人都至少有一个Mayo-EMG症状出现后4年。小肌电图异常(即患者。1 - 2肌肉去神经,扩大中)但没有lower-motor-neuron进展进行评估,最终实现标准的戈登。结果:79请患者,31日小,稳定的去神经肌电图的证据。时间最后的肌电图:从症状出现4 - 28年。这些患者的表型类似于纯请与正常的肌电图。大多数(21/31)lower-extremity-onset (paraparetic);七hemiparetic-onset,三,bulbar-onset。 Onsets ranged from 42–72 years. In the paraparetic group with mean follow-up of 10.8 years (4 to 30 years), 20 patients had symptom progression to upper limbs by mean 5.7 years (1–22 years); bladder symptoms in 11 patients, mean 9.7 years (0–25 years); dysarthria, 16 patients at mean 7.4 years (0.5–22 years); dysphagia, 10 patients at mean 6.8 years( 5–13 years); pseudobulbar symptoms, 6 patients at mean 6.9 years (4–18 years). Cane or walker was necessary at a mean 6–8 years after onset among those with adequate follow-up, including five, wheel-chair-bound. Similar needs for gait aids were noted in the hemiparetic-onset group, whereas 1/3 bulbar-onset patients needed a walker at five years.Conclusions: PLS with minor, stable EMG signs of denervation appears clinically similar to pure PLS. The most common phenotype was paraparetic but also included hemiparetic and bulbar-onset. As with pure PLS, progression included variable degrees of dysarthria, dysphagia, bladder and pseudobulbar symptoms. Thus, minor, stable EMG lower-motor-neuron changes do not preclude PLS diagnosis.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 -