在偶发性和家族性ALS型8的自主功能
Melina Pazian Martins1, Carelis González-Salazar1, Fabricio Diniz de Lima1
1Department of Neurology, School of Medical Sciences, University of Campinas (UNICAMP), Campinas, Brazil.
概括
自主功能障碍,包括心脏和运动问题,存在于零星ALS和家族ALS类型8中. 然而,这两种ALS形式之间的这种dysautonomia的具体症状是不同的.
科学领域:
- 神经学 神经学
- 自主神经系统研究 自主神经系统研究
- 神经退行性疾病 神经退行性疾病
背景情况:
- 肌缩侧面硬化 (ALS) 是一种进展性神经退行性疾病,影响运动神经元.
- 自主功能障碍 (dysautonomia) 越来越多地被认为是ALS的一个组成部分.
- 家庭ALS型8 (fALS8) 是一种罕见的遗传亚型,具有独特的特征.
研究的目的:
- 描述和比较患有散发性ALS (sALS) 和家族性ALS8型 (fALS8) 的患者的自主功能.
- 确定sals和fals8.8之间自主症状概况和神经生理学发现的差异.
主要方法:
- 招募了11名sALS患者,14名fALS8患者和26名年龄和性别匹配的对照.
- 自主功能使用帕金森病自主症状结果尺度 (SCOPA-AUT),心率可变性,定量动轴突反射测试 (QSART) 和皮肤交感反应 (SSR) 来评估.
- 数据使用非参数测试进行分析,显著性设置为P < 0.05.
主要成果:
- 与sALS和对照组相比,fALS8患者报告了更多的温度调节,瞳孔运动和性症状.
- 与对照组相比,sALS和fALS8组在心率变化指标 (Valsalva比率,E:I指数,RR最小值) 中显示出显著差异.
- 在sALS和fALS8中,突发运动功能障碍显而易见,由降低的QSART体积和下肢缺失的SSR表明.
结论:
- 心脏和运动失调是sALS和fALS8.8表型的组成部分.
- 自主症状的不同形状区分了fALS8和sALS.
- 在fALS8和sALS中,自主功能障碍涉及同情神经系统和副同情神经系统的划分.
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