Department of Neurology, Medical University of Warsaw, Banacha 1a, 02-091 Warsaw, Poland.
Department of Bioethic, Medical University of Warsaw, Warsaw, Poland.
Neurol Neurochir Pol. 2019;53(5):335-340. doi: 10.5603/PJNNS.a2019.0046. Epub 2019 Oct 17.
Gilles de la Tourette syndrome (GTS) is a childhood onset disorder characterised by motor and vocal tics. Different types of motor tics may occur in GTS, including dystonic tics (DTs). Although DTs have been recognised as part of GTS symptomatology, little is known about their risk factors or about how often and at what age they appear in affected individuals.
The aim of our study was to investigate lifetime prevalence and clinical correlations of DTs in a Polish cohort of GTS patients.
We performed a prospective, one-registration study in a cohort of 207 consecutive ambulatory patients (mean age: 16.5 ± 9.4 years, 131 children, 162 males) with GTS. Duration of GTS was 9.0 ± 8.0 years (range: 1-39 years). DTs were diagnosed during the interview. DTs were defined as slower and lasting longer than typical clonic tics, abnormal dystonia-like movements that led to a sustained, but not fixed, posture.
DTs occurred at some point in the lifetime of 73.9% (n = 153) of patients. The prevalence of DTs in adults and children was almost the same (p = 0.963). Age at onset of DTs was 9.9 ± 5.2 years with the most frequent onset in children (7-11 years, 74.4%, n = 64), followed by adolescence (12-18 years; 17.4%, n = 15) and adulthood (≥ 18 years, 8.1%, n = 7). DTs occurred 3.7 ± 4.2 years after tic onset. On average, patients suffered from 1.8 ± 1.7 types of DTs. The most frequent manifestations of DTs were: eyes (tightening resembling blepharospasm 84.3%, n = 129 and oculogyric crisis 45.8%, n = 70), trunk (dystonic postures 59.5%, n = 91), jaw (bruxism 34.6%, n = 53), neck (30.7%, n = 47), upper limb (26.1%, n = 40), and foot (20.9%, n = 32). Multivariate logistic regression analysis showed significant associations of DTs with the total number of simple, and the total number of complex, tics.
DTs are early and frequent symptoms of GTS. They tend to localise in the facial area. DTs occur more frequently in individuals with a higher number of tics and probably add to the global impairment caused by tics.
妥瑞氏症候群(Tourette syndrome,GTS)是一种以运动和发声抽动为特征的儿童期发病障碍。GTS 可能出现不同类型的运动性抽动,包括肌张力障碍性抽动(dystonic tics,DTs)。尽管 DTs 已被认为是 GTS 症状的一部分,但对于其危险因素或在受影响个体中出现的频率和年龄,知之甚少。
本研究的目的是在波兰 GTS 患者队列中调查 DTs 的终身患病率和临床相关性。
我们对 207 例连续门诊患者(平均年龄:16.5±9.4 岁,131 例儿童,162 例男性)进行了前瞻性、单次登记研究。GTS 的病程为 9.0±8.0 年(范围:1-39 年)。在访谈期间诊断 DTs。DTs 被定义为比典型的痉挛性抽动更慢、持续时间更长的异常肌张力样运动,导致持续但非固定的姿势。
73.9%(n=153)的患者在其一生中的某个时间点出现过 DTs。成人和儿童 DTs 的患病率几乎相同(p=0.963)。DTs 的发病年龄为 9.9±5.2 岁,最常见于儿童(7-11 岁,74.4%,n=64),其次是青少年(12-18 岁,17.4%,n=15)和成年期(≥18 岁,8.1%,n=7)。DTs 在抽动发病后 3.7±4.2 年出现。平均而言,患者患有 1.8±1.7 种 DTs。DTs 最常见的表现是:眼部(类似于眼睑痉挛的紧绷感 84.3%,n=129 和眼球震颤危机 45.8%,n=70)、躯干(肌张力障碍姿势 59.5%,n=91)、下颌(磨牙 34.6%,n=53)、颈部(30.7%,n=47)、上肢(26.1%,n=40)和足部(20.9%,n=32)。多变量逻辑回归分析显示,DTs 与单纯性 tic 总数和复杂性 tic 总数显著相关。
DTs 是 GTS 的早期和常见症状。它们倾向于在面部区域定位。在 tic 数量较多的个体中,DTs 发生的频率更高,可能会增加 tic 引起的整体损伤。