Division Motor Research, Pathophysiology and Biomechanics, Department of Trauma, Hand and Reconstructive Surgery, University Hospital, Friedrich Schiller University, Jena, Germany.
Mov Disord. 2010 Feb 15;25(3):265-74. doi: 10.1002/mds.22933.
Increasing interest in rodent models for movement disorders has led to an increasing need for more accurate and precise methods for both delineating the nature of abnormal movements and measuring their severity. These studies describe application of simultaneous high-speed video kinematics with multichannel electromyography (EMG) to characterize the movement disorder exhibited by tottering mutant mice. These mice provide a uniquely valuable model, because they exhibit paroxysmal dystonia superimposed on mild baseline ataxia, permitting the examination of these two different problems within the same animals. At baseline with mild ataxia, the mutants exhibited poorly coordinated movements with increased variation of stance and swing times, and slower spontaneous walking velocities. The corresponding EMG showed reduced mean amplitudes of biceps femoris and vastus lateralis, and poorly modulated EMG activities during the step cycle. Attacks of paroxysmal dystonia were preceded by trains of EMG bursts with doublets and triplets simultaneously in the biceps femoris and vastus lateralis followed by more sustained coactivation. These EMG characteristics are consistent with the clinical phenomenology of the motor phenotype of tottering mice as a baseline of mild ataxia with intermittent attacks of paroxysmal dystonia. The EMG characteristics of ataxia and dystonia in the tottering mice also are consistent with EMG studies of other ataxic or dystonic animals and humans. These studies provide insights into how these methods can be used for delineating movement disorders in mice and for how they may be compared with similar disorders of humans.
对用于运动障碍的啮齿动物模型的日益关注，导致人们对更准确和精确的方法的需求不断增加，这些方法既用于描绘异常运动的性质，也用于测量其严重程度。这些研究描述了应用高速视频运动学与多通道肌电图 (EMG) 相结合的方法，以描述蹒跚突变小鼠表现出的运动障碍。这些小鼠提供了一种非常有价值的模型，因为它们表现出阵发性肌张力障碍，同时伴有轻度基线共济失调，允许在同一动物中检查这两种不同的问题。在轻度共济失调的基线时，突变体表现出运动不协调，步幅和摆动时间变化增加，自发行走速度较慢。相应的 EMG 显示二头肌和股外侧肌的平均振幅降低，在步周期中 EMG 活动调制不良。阵发性肌张力障碍发作前，二头肌和股外侧肌的 EMG 爆发会出现双连发和三连发，随后是更持续的共同激活。这些 EMG 特征与 tottering 小鼠运动表型的临床现象学一致，即轻度共济失调的基线，间歇性阵发性肌张力障碍发作。tottering 小鼠的共济失调和肌张力障碍的 EMG 特征也与其他共济失调或肌张力障碍动物和人类的 EMG 研究一致。这些研究提供了有关如何在小鼠中描绘运动障碍以及如何将其与人类的类似障碍进行比较的方法的深入了解。
