Meunier S, Garnero L, Ducorps A, Mazières L, Lehéricy S, du Montcel S T, Renault B, Vidailhet M
Department of Clinical Neurophysiology, Pitié-Salpêtriére Hospital, Paris, France.
Ann Neurol. 2001 Oct;50(4):521-7. doi: 10.1002/ana.1234.
Dystonia has a wide clinical spectrum from early-onset generalized to late-onset sporadic, task-specific forms. The genetic origin of the former has been clearly established. A critical role of repetitive skilled motor tasks has been put forward for the latter, while underlying vulnerability traits are still being searched for. Using magnetoencephalography, we looked for structural abnormalities reflecting a preexisting dysfunction. We studied finger representations of both hands in the primary sensory cortex, as compared in 23 patients with unilateral task-specific dystonia and 20 control subjects. A dramatic disorganization of the nondystonic hand representation was found in all patients, and its amount paralleled the severity of the dystonic limb motor impairment. Abnormalities were also observed in the cortex coding the dystonic limb representation, but they were important only in the most severely affected patients. The abnormal cortical finger representations from the nondystonic limb appear to be endophenotypic traits of dystonia. That finger representations from the dystonic limb were almost normal for the less severely affected patients may be due to intrinsic beneficial remapping in reaction against the primary disorder.
肌张力障碍的临床谱很广，从早发性全身性到晚发性散发性、任务特异性形式。前者的遗传起源已明确确立。对于后者，重复性熟练运动任务的关键作用已被提出，而潜在的易患性特征仍在探寻中。我们使用脑磁图寻找反映先前存在功能障碍的结构异常。我们研究了23例单侧任务特异性肌张力障碍患者和20名对照受试者的双手在初级感觉皮层中的手指表征。在所有患者中均发现非肌张力障碍手表征的显著紊乱，其程度与肌张力障碍肢体运动障碍的严重程度平行。在编码肌张力障碍肢体表征的皮层中也观察到异常，但仅在受影响最严重的患者中较为明显。来自非肌张力障碍肢体的异常皮层手指表征似乎是肌张力障碍的内表型特征。对于受影响较轻的患者，肌张力障碍肢体的手指表征几乎正常，这可能是由于针对原发性疾病的内在有益重新映射。
