Laterality of onset in idiopathic torsion dystonia
Laterality of onset in idiopathic torsion dystonia
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特发性扭转肌张力障碍的偏侧性发病
作者：
R. Inzelberg;N. Ziber;E. Kahana;A. Korczyn
特发性扭转肌张力障碍（ITD）是一种显性遗传性疾病。确定可能导致肌张力障碍的因素或预防其在遗传易感个体中的发生是很重要的。由于肌张力障碍可能由外周触发诱发，我们研究了优先使用肢体是否会影响肌张力障碍的发展。在以色列进行的一项全国性调查中，对49例ITD患者进行了运动优势侧与首次出现张力障碍症状的肢体之间的相关性分析。45例（92%）确定运动侧为优势侧。在29例侧侧肢体发病的患者中，24例表现为右侧运动优势，其中21例为右侧肢体肌张力障碍发病。在所有5例左侧运动优势的患者中，第一个症状出现在左肢体（90%的符合率）。研究了肢体受累模式。可以排除检测偏差。在ITD患者中，运动优势与肢体发作的侧边性之间的高度显著关系表明，优先使用肢体可能引发肌张力障碍的发作。
Idiopathic torsion dystonia (ITD) is a dominantly inherited disorder with incomplete penetrance. It is important to identify factors that may cause dystonia or prevent its occurrence in a genetically predisposed individual. Because dystonia may be precipitated by peripheral triggers, we have investigated whether the preferential use of a limb affects the development of dystonia. Analysis of the correlation between the side of motor dominance and the limbs in which dystonic symptoms first appeared was performed in 49 patients with ITD ascertained in a country‐wide survey in Israel. The dominant motor side was determined in 45 cases (92%). Among 29 patients with lateralized limb onset, 24 showed right‐side motor dominance, of whom 21 had dystonia onset in a right limb. The first sign was in a left limb for all five cases with left‐side motor dominance (90% coincidence). The pattern of limb involvement was studied. Detection bias could be ruled out. The highly significant relationship between the motor dominance and the laterality of limb onset in ITD patients suggests that the preferred use of a limb may trigger the onset of dystonia.