Ghika J, Bogousslavsky J, Henderson J, Maeder P, Regli F
Service de Neurologie, CHUV, Lausanne, Switzerland.
J Neurol. 1994 Aug;241(9):537-42. doi: 10.1007/BF00873516.
We report the cases of three patients with a thalamic infarct in the territory of the posterior choroidal artery involving the posterior thalamic nuclei. These patients developed delayed complex hyperkinetic motor syndromes, associating ataxia, tremor, dystonia, myoclonus and chorea, which we call "the jerky dystonic unsteady hand". One patient had a severe myoclonic and ataxic-dystonic choreoathetosis; another showed a so-called rubral tremor (myoclonic ataxia with resting, action, and wing-beating tremor) with dystonia; and the third one had a dystonic and ataxic hand with intermittent mild action myoclonus. All of them had sensory dysfunction; two had also presented with a painful Dejérine-Roussy syndrome. All had CT or MRI-proven infarcts in the territory of the posterior cerebral artery involving the posterior choroidal territory with an abnormal signal in the posterior area of the thalamus (pulvinar nucleus) but sparing the other thalamic, subthalamic and midbrain structures. These delayed myoclonic complex hyperkinetic syndromes have not been reported before, and we did not observe them in other topographic forms of thalamic infarcts. They may thus represent a new entity of movement disorders due to lesions in the posterior thalamic nuclei, with specificity for posterior choroidal artery infarcts.
我们报告了3例脉络膜后动脉供血区丘脑梗死累及丘脑后核的病例。这些患者出现了延迟性复杂性运动过度综合征,伴有共济失调、震颤、肌张力障碍、肌阵挛和舞蹈症,我们称之为“急促性肌张力障碍性手部不稳”。1例患者出现严重的肌阵挛性及共济失调性肌张力障碍性舞蹈手足徐动症;另1例表现为所谓的红核震颤(伴有静息性、动作性及扑翼样震颤的肌阵挛性共济失调)合并肌张力障碍;第3例患者有肌张力障碍性及共济失调性手部,伴有间歇性轻度动作性肌阵挛。所有患者均有感觉功能障碍;2例还出现了疼痛性丘脑综合征。所有患者的CT或MRI均证实大脑后动脉供血区梗死累及脉络膜后区,丘脑后部(枕核)有异常信号,但其他丘脑、丘脑底核及中脑结构未受累。此前尚未报道过这些延迟性肌阵挛性复杂性运动过度综合征,我们在其他丘脑梗死的部位也未观察到。因此,它们可能代表了一种因丘脑后核病变导致的新型运动障碍,对脉络膜后动脉梗死具有特异性。