Straube A, Mennicken J B, Riedel M, Eggert T, Müller N
Department of Neurology, Ludwig Maximilians University, Munich, Germany.
Mov Disord. 1997 Jul;12(4):536-46. doi: 10.1002/mds.870120410.
Gilles de la Tourette's syndrome (GTS) is presumed to be an inherited disorder with an unclear pathophysiology. An involvement of the basal ganglia is suspected. Besides vocal tics, one of the main symptoms is the presence of motor tics. As eye movements are a specialized part of the motor system, we investigated whether they differed in some typical way in GTS patients. To study the control of saccades in GTS, different paradigms were used to elicit saccades, which were either externally triggered and visually guided or internally triggered and without visual target. GTS patients (n = 10) showed a significant increase of the latency of antisaccades, a highly impaired performance of sequences of memory-guided saccades, and an isolated reduction of the peak velocity in the antisaccades. Overall the results were similar to those found with similar paradigms in patients with Huntington's disease (HD). In analogy to the known pathology of HD, these findings can be attributed to ascending loops from the basal ganglia that inappropriately activate the frontal cortex, especially some of the eye movement-related areas there. This impairment seems to be a rather specific effect, since some oculomotor features that are preserved by structures of the frontal cortex, such as the effect of a fixation target on saccadic latency ("gap effect"), were normal.
抽动秽语综合征(GTS)被认为是一种病理生理学尚不清楚的遗传性疾病。人们怀疑基底神经节参与其中。除了发声抽动外,主要症状之一是存在运动性抽动。由于眼球运动是运动系统的一个特殊部分,我们研究了GTS患者的眼球运动是否在某些典型方面存在差异。为了研究GTS患者的扫视控制,我们使用了不同的范式来引发扫视,这些扫视要么是外部触发并由视觉引导,要么是内部触发且没有视觉目标。GTS患者(n = 10)表现出反扫视潜伏期显著增加、记忆引导扫视序列的表现严重受损以及反扫视峰值速度单独降低。总体而言,这些结果与在亨廷顿舞蹈病(HD)患者中使用类似范式所发现的结果相似。与HD已知的病理学类似,这些发现可归因于基底神经节的上行环路不恰当地激活了额叶皮质,尤其是其中一些与眼球运动相关的区域。这种损害似乎是一种相当特殊的效应,因为额叶皮质结构所保留的一些眼球运动特征,如注视目标对扫视潜伏期的影响(“间隙效应”)是正常的。