Tremor in primary adult-onset dystonia: prevalence and associated clinical features
Tremor in primary adult-onset dystonia: prevalence and associated clinical features
复制标题
DOI：
10.1136/jnnp-2012-303782
复制
发表时间：
2013-04-01
影响因子：
11
通讯作者：
Berardelli, Alfredo
中科院分区：
文献类型：
--
作者：
Defazio, Giovanni;Gigante, Angelo Fabio;Berardelli, Alfredo
目的探讨原发性成人肌张力障碍（PAOD）患者震颤的发生率及主要临床特征，方法对意大利8个运动障碍中心的429例PAOD患者进行横断面研究。虽然肌张力障碍发作时的性别和年龄在有震颤和没有震颤的肌张力障碍患者中相似，但有震颤的患者更常受局灶性颈部肌张力障碍的影响，而较少受局灶性眼睑痉挛的影响。肌张力障碍有更大的倾向，以传播患者震颤。根据运动障碍协会共识声明，43例患者的震颤被归类为肌张力障碍性震颤（DT），23例患者的震颤伴肌张力障碍（TAWD）。6例患者同时存在两种类型的震颤。考虑到发病时年龄和相应肌张力障碍类型的身体分布的潜在混杂因素，DT患者和TAWD患者的所有临床特征均相当，但肌张力障碍扩散的趋势除外，DT患者的趋势更大。结论震颤是原发性迟发性肌张力障碍的一个相对常见的特征，约17%的患者发生震颤。震颤和肌张力障碍扩散之间的关联表明这种形式的震颤可能是肌张力障碍的表现。DT和TAWD的表型特征的相似性超过了差异，这表明这两种形式的震颤可能是同一种疾病的表现。肌张力障碍伴震颤患者与原发性震颤患者在性别和震颤体部分布上的差异也提示肌张力障碍伴震颤与原发性震颤是不同的实体。
Objective To investigate the frequency and the main clinical features of tremor in primary adult-onset dystonia (PAOD).Methods This cross-sectional study was conducted on 429 patients with PAOD from eight Italian movement disorder centres.Results Of the 429 dystonic patients, 72 (16.7%) had tremor. Although sex and age at dystonia onset were similar in dystonic patients who had tremor and those who did not, patients who had tremor were affected more often by focal cervical dystonia and less often by focal blepharospasm. Dystonia had a greater tendency to spread in patients with tremor. According to the Movement Disorder Society Consensus Statement, tremor was classified as dystonic tremor (DT) in 43 patients and tremor associated with dystonia (TAWD) in 23 patients. Six patients had both types of tremor. Taking into account potential confounding by age at onset and body distribution of the corresponding dystonia type, all the clinical features in patients with DT and in those with TAWD were comparable except the tendency of dystonia to spread, which was greater in patients with DT. Conclusions Tremor is a relatively common feature occurring in about 17% of patients with primary late-onset dystonia. The association between tremor and dystonia spread suggests that this form of tremor may be a dystonic manifestation. Similarities in phenotypic features of DT and TAWD predominated over differences, suggesting that the two forms of tremor may be manifestations of the same disease. Differences in gender and body distribution of tremor between patients with dystonia and tremor and those of patients with essential tremor also suggest that tremor in dystonia and essential tremor are different entities.