Hayashi M, Koide H
Department of Pediatrics, Saitama Medical School, Japan.
Brain Dev. 1997 Dec;19(8):556-8. doi: 10.1016/s0387-7604(97)00070-3.
A 12-year-old boy had suffered from idiopathic torsion dystonia since the age of 8 years, which had never been relieved with pharmacologic treatment. His mother had exhibited primary tremor upon writing from the age of 10 years, but had not yet developed dystonia. Surface electromyography revealed paradoxical muscular contraction of Westphal in both patients, although the main abnormal findings in the proband and his mother were continuous tonic discharges in the arms and 4-5-Hz grouped discharges in the neck, respectively. The simultaneous occurrence of dystonia and writing tremor within one family, and the presence of the paradoxical contraction in both cases suggest that a certain type of dystonia and writing tremor may be pathogenetically linked.
一名12岁男孩自8岁起患有特发性扭转性肌张力障碍，药物治疗从未使其缓解。他的母亲从10岁起写字时就出现原发性震颤，但尚未发展为肌张力障碍。表面肌电图显示两名患者均有韦斯法尔矛盾性肌肉收缩，尽管先证者及其母亲的主要异常表现分别为手臂持续强直性放电和颈部4-5赫兹的成组放电。一个家庭中同时出现肌张力障碍和书写震颤，且两例均存在矛盾性收缩，这表明某种类型的肌张力障碍和书写震颤可能在发病机制上存在关联。
