小脑共济失调和步态障碍的神经生理学。
Neurophysiology of cerebellar ataxias and gait disorders.
发表日期:2023
作者:
Mario Manto, Mariano Serrao, Stefano Filippo Castiglia, Dagmar Timmann, Elinor Tzvi-Minker, Ming-Kai Pan, Sheng-Han Kuo, Yoshikazu Ugawa
来源:
CLINICAL PHARMACOLOGY & THERAPEUTICS
摘要:
小脑疾病的形式包括散发性和遗传性疾病。本章的目的是概述过去20年间用于临床和科研目的的在小脑患者中的神经生理学测试的进展和新兴技术。从临床上看,患者表现出多种组合的前庭小脑综合症、小脑认知情感综合症和小脑运动综合症,这些将在本章中进行讨论。小脑患者显示出异常的准备电位和不匹配负性(mismatch negativity)。现在正在将小脑脑电图应用于小脑疾病中,以解开与小脑皮质疾患有关的电生理模式的障碍。眨眼条件反射在小脑疾病中明显受损:遗传性共济失调、小脑中风和小脑肿瘤切除手术后,获得条件性眨眼反应的能力减少。此外,眨眼条件反射受损是小脑退行性疾病的一个早期标志。运动控制的一般规则表明,在日常生活复杂环境中执行自愿运动需要最优策略。学习运动控制的过程需要高度适应性,因为感觉运动适应是执行准确目标导向运动的关键。小脑患者在在线视运动适应任务中表现出障碍。小脑-运动皮层抑制(CBI)是一种神经生理学生物标志物,显示了小脑丘脑皮质通路完整性与共济失调严重程度之间的逆相关关系。共济步态特点包括步态宽度增加,踝关节运动范围减小,步态变异性增加,肢体间和节段间协调缺失,足地位和躯干控制受损。综上所述,这些技术为更好地评估小脑疾病提供了一个神经生理学框架。©2023年国际临床神经生理学联合会。由Elsevier B.V.出版。
There are numerous forms of cerebellar disorders from sporadic to genetic diseases. The aim of this chapter is to provide an overview of the advances and emerging techniques during these last 2 decades in the neurophysiological tests useful in cerebellar patients for clinical and research purposes. Clinically, patients exhibit various combinations of a vestibulocerebellar syndrome, a cerebellar cognitive affective syndrome and a cerebellar motor syndrome which will be discussed throughout this chapter. Cerebellar patients show abnormal Bereitschaftpotentials (BPs) and mismatch negativity. Cerebellar EEG is now being applied in cerebellar disorders to unravel impaired electrophysiological patterns associated within disorders of the cerebellar cortex. Eyeblink conditioning is significantly impaired in cerebellar disorders: the ability to acquire conditioned eyeblink responses is reduced in hereditary ataxias, in cerebellar stroke and after tumor surgery of the cerebellum. Furthermore, impaired eyeblink conditioning is an early marker of cerebellar degenerative disease. General rules of motor control suggest that optimal strategies are needed to execute voluntary movements in the complex environment of daily life. A high degree of adaptability is required for learning procedures underlying motor control as sensorimotor adaptation is essential to perform accurate goal-directed movements. Cerebellar patients show impairments during online visuomotor adaptation tasks. Cerebellum-motor cortex inhibition (CBI) is a neurophysiological biomarker showing an inverse association between cerebellothalamocortical tract integrity and ataxia severity. Ataxic gait is characterized by increased step width, reduced ankle joint range of motion, increased gait variability, lack of intra-limb inter-joint and inter-segmental coordination, impaired foot ground placement and loss of trunk control. Taken together, these techniques provide a neurophysiological framework for a better appraisal of cerebellar disorders.© 2023 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.