Department of Neurology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Brain. 2013 Jul;136(Pt 7):2050-62. doi: 10.1093/brain/awt147.
A large body of evidence points to a role of basal ganglia dysfunction in the pathophysiology of dystonia, but recent studies indicate that cerebellar dysfunction may also be involved. The cerebellum influences sensorimotor adaptation by modulating sensorimotor plasticity of the primary motor cortex. Motor cortex sensorimotor plasticity is maladaptive in patients with writer's cramp. Here we examined whether putative cerebellar dysfunction in dystonia is linked to these patients' maladaptive plasticity. To that end we compared the performances of patients and healthy control subjects in a reaching task involving a visuomotor conflict generated by imposing a random deviation (-40° to 40°) on the direction of movement of the mouse/cursor. Such a task is known to involve the cerebellum. We also compared, between patients and healthy control subjects, how the cerebellum modulates the extent and duration of an ongoing sensorimotor plasticity in the motor cortex. The cerebellar cortex was excited or inhibited by means of repeated transcranial magnetic stimulation before artificial sensorimotor plasticity was induced in the motor cortex by paired associative stimulation. Patients with writer's cramp were slower than the healthy control subjects to reach the target and, after having repeatedly adapted their trajectories to the deviations, they were less efficient than the healthy control subjects to perform reaching movement without imposed deviation. It was interpreted as impaired washing-out abilities. In healthy subjects, cerebellar cortex excitation prevented the paired associative stimulation to induce a sensorimotor plasticity in the primary motor cortex, whereas cerebellar cortex inhibition led the paired associative stimulation to be more efficient in inducing the plasticity. In patients with writer's cramp, cerebellar cortex excitation and inhibition were both ineffective in modulating sensorimotor plasticity. In patients with writer's cramp, but not in healthy subjects, behavioural parameters reflecting their capacity for adapting to the rotation and for washing-out of an earlier adaptation predicted the efficacy of inhibitory cerebellar conditioning to influence sensorimotor plasticity: the better the online adaptation, the smaller the influence of cerebellar inhibitory stimulation on motor cortex plasticity. Altered cerebellar encoding of incoming afferent volleys may result in decoupling the motor component from the afferent information flow, and also in maladjusted sensorimotor calibration. The loss of cerebellar control over sensorimotor plasticity might also lead to building up an incorrect motor program to specific adaptation tasks such as writing.
大量证据表明基底神经节功能障碍在肌张力障碍的病理生理学中起作用,但最近的研究表明小脑功能障碍也可能涉及其中。小脑通过调节初级运动皮层的感觉运动可塑性来影响感觉运动适应。原发性震颤患者的运动皮层感觉运动可塑性适应性不良。在这里,我们研究了肌张力障碍患者的假定小脑功能障碍是否与这些患者的适应性不良可塑性有关。为此,我们比较了患者和健康对照组在涉及由鼠标/光标运动方向施加随机偏差(-40°至 40°)产生的视觉运动冲突的伸展任务中的表现。众所周知,这种任务涉及小脑。我们还比较了患者和健康对照组之间,小脑如何调节运动皮层中正在进行的感觉运动可塑性的程度和持续时间。通过重复经颅磁刺激来兴奋或抑制小脑皮层,然后通过成对关联刺激在运动皮层中诱导人工感觉运动可塑性。原发性震颤患者比健康对照组更慢地达到目标,并且在反复适应轨迹以适应偏差之后,他们在没有施加偏差的情况下执行伸展运动的效率不如健康对照组。这被解释为受损的冲洗能力。在健康受试者中,小脑皮层兴奋可防止成对关联刺激在初级运动皮层中诱导感觉运动可塑性,而小脑皮层抑制可使成对关联刺激在诱导可塑性方面更有效。在原发性震颤患者中,小脑皮层兴奋和抑制均无法调节感觉运动可塑性。在原发性震颤患者中,但在健康受试者中,反映其适应旋转和早期适应冲洗能力的行为参数预测了抑制性小脑调节对感觉运动可塑性的影响的功效:在线适应越好,小脑抑制刺激对运动皮层可塑性的影响越小。传入冲动的小脑编码改变可能导致运动成分与传入信息流解耦,并导致感觉运动校准不当。小脑对感觉运动可塑性的控制丧失也可能导致针对特定适应任务(例如书写)建立不正确的运动程序。