Neurological manifestations in Wilson's disease:: Report of 119 cases
Neurological manifestations in Wilson's disease:: Report of 119 cases
复制标题
DOI：
10.1002/mds.21170
复制
发表时间：
2006-12-01
影响因子：
8.6
通讯作者：
Barbosa, Egberto Reis
中科院分区：
文献类型：
--
作者：
Machado, Alexandre;Chien, Hsin Fen;Barbosa, Egberto Reis
我们描述了1963年至2004年期间119例WD患者（93例索引病例和26例受影响的家庭成员）的神经系统表现。症状发作时的平均年龄为19.6岁（范围，7-37岁）。审查了患者首次神经系统检查的病历。最常见的神经系统表现为构音障碍（91%）、步态障碍（75%）、肌张力障碍（69%）、强直（66%）、震颤（60%）和吞咽困难（50%）。较不常见的表现是舞蹈病（16%）和手足徐动症（14%）。罕见的神经系统表现为癫痫发作（4.2%）和锥体束征（3%）。(C)2006年，《社会运动》创刊。
We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%). (C) 2006 Movement Disorder Society.