Kirke Diana N, Frucht Steven J, Simonyan Kristina
Department of Neurology, Icahn School of Medicine at Mount Sinai, One Gustave L. Levy Place, Box 1137, New York, NY, 10029, USA.
J Neurol. 2015 Jun;262(6):1548-56. doi: 10.1007/s00415-015-7751-2. Epub 2015 May 1.
Laryngeal dystonia (LD) is a task-specific focal dystonia of unknown pathophysiology affecting speech production. We examined the demographics of anecdotally reported alcohol use and its effects on LD symptoms using an online survey based on Research Electronic Data Capture (REDCap™) and National Spasmodic Dysphonia Association's patient registry. From 641 participants, 531 were selected for data analysis, and 110 were excluded because of unconfirmed diagnosis. A total of 406 patients (76.5 %) had LD and 125 (23.5 %) had LD and voice tremor (LD/VT). The consumption of alcohol was reported by 374 LD (92.1 %) and 109 LD/VT (87.2 %) patients. Improvement of voice symptoms after alcohol ingestion was noted by 227 LD (55.9 % of all patients) and 73 LD/VT (58.4 %), which paralleled the improvement observed by patient's family and/or friends in 214 LD (57.2 %) and 69 LD/VT (63.3 %) patients. The benefits lasted 1-3 h in both groups with the maximum effect after 2 drinks in LD patients (p = 0.002), whereas LD/VT symptoms improved independent of the consumed amount (p = 0.48). Our data suggest that isolated dystonic symptoms, such as in LD, are responsive to alcohol intake and this responsiveness is not attributed to the presence of VT, which is known to have significant benefits from alcohol ingestion. Alcohol may modulate the pathophysiological mechanisms underlying abnormal neurotransmission of γ-aminobutyric acid (GABA) in dystonia and as such provide new avenues for novel therapeutic options in these patients.
喉肌张力障碍(LD)是一种病因不明的任务特异性局灶性肌张力障碍,影响言语产生。我们使用基于研究电子数据采集(REDCap™)和国家痉挛性发音障碍协会患者登记处的在线调查,研究了传闻中饮酒的人口统计学特征及其对LD症状的影响。在641名参与者中,531名被选作数据分析,110名因诊断未确认而被排除。共有406例患者(76.5%)患有LD,125例(23.5%)患有LD伴语音震颤(LD/VT)。374例LD患者(92.1%)和109例LD/VT患者(87.2%)报告有饮酒行为。227例LD患者(占所有患者的55.9%)和73例LD/VT患者(58.4%)在饮酒后语音症状有改善,这与患者家属和/或朋友观察到的214例LD患者(57.2%)和69例LD/VT患者(63.3%)的改善情况相似。两组患者的益处持续1 - 3小时,LD患者饮用2杯酒时效果最佳(p = 0.002),而LD/VT症状的改善与饮酒量无关(p = 0.48)。我们的数据表明,孤立的肌张力障碍症状,如LD中的症状,对饮酒有反应,且这种反应性并非归因于VT的存在,已知VT从饮酒中获益显著。酒精可能调节肌张力障碍中γ-氨基丁酸(GABA)异常神经传递的病理生理机制,从而为这些患者提供新的治疗选择途径。