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在1型和2型脊髓大脑动症中综合自主性严重性评分

Dibashree Tamuli¹, Manpreet Kaur², Ashok K Jaryal³

¹Department of Physiology, All India Institute of Medical Sciences, New Delhi, India; Department of Zoology, Nalbari College, Assam, India.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia

|May 31, 2023

概括

第1类和第2类脊髓小脑动症 (SCA) 显著损害自主神经系统功能,特别是心血管反射. 大多数患者经历中度自主性衰竭,严重衰竭在SCA1.1中更常见.

科学领域:

神经学神经学
自主神经科学自主神经科学

背景情况:

脊髓小脑缩症 (SCA) 是一组遗传性神经退行性疾病.
自主功能障碍越来越多地被认为是SCAs中显著的并发症.

研究的目的:

为了评估自主神经系统完整性在脊髓大脑动症 (SCA) 类型1和2.
通过一系列测试和复合自主严重性评分 (CASS) 来评估自主功能.

主要方法:

在SCA1 (n=31),SCA2 (n=40) 和健康对照 (n=40) 中评估了心血管 (依赖和独立于巴罗反射) 和运动功能.
计算了复合自主严重性评分 (CASS) 以对自主异常进行分级.

主要成果:

无论是SCA1和SCA2患者都显示出巴罗反射依赖的自主反应性降低,包括显著的缩血压下降和较低的30:15比率在头向上倾斜时与对照组相比.
在SCA1与SCA2.2的Sudomotor评估中,发现SCA1与SCA2.2的距离腿汗水反应延长的延迟时间.
在SCA1的80.65%和SCA2的85%患者中,中度的全身性自主性衰竭是普遍存在的;严重的自主性衰竭在SCA1 (6.45%) 比SCA2 (2.50%) 更常见.

结论:

心血管自主功能在SCA1和SCA2显著受损,特别影响着巴罗反射循环的完整性.
在大多数SCA1和SCA2患者中,CASS评分表明中度自主性衰竭.

关键词:

巴罗反射依赖的自主功能障碍心血管自主功能的心血管自主功能综合自主严重性评分综合自主严重性评分脊髓大脑小骨性动脉衰竭南极运动自主功能自主功能

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