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Embouchure dystonia--Portrait of a task-specific cranial dystonia.

机译:Embouchure肌张力障碍-特定任务的颅肌张力障碍的肖像。

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Focal task-specific dystonia (FTSD) is an unusual disorder of motor control, which typically affects the hand but may also involve the face, jaw, and tongue. We report 89 musicians with dystonia of the embouchure (ED), the muscles of the lower face, jaw, and tongue used to control the flow of air into the mouthpiece of a woodwind or brass instrument. Symptoms of ED began at an average age of 36, were typically painless and only rarely were preceded by trauma. Specific musical techniques commonly triggered dystonia, often in one instrumental register. Task-specific embouchure tremor and lip-pulling ED phenotypes were common among high-register brass players (trumpet and French horn), whereas lip-locking occurred exclusively in low-register brass players (trombone and tuba). Jaw and tongue ED phenotypes occurred predominantly in woodwind players, and once present, frequently spread to speaking or eating. Six percent of all ED patients had coincident writer's cramp, suggesting a possible genetic predisposition to develop dystonia. We assessed two-point sensory discrimination in the upper lip, lower lip, and hand in ED patients, normal musicians, and nonmusician age-matched controls--there were no differences between groups. Once present, symptoms of ED did not remit and often disrupted careers and livelihoods. Better treatments are urgently needed for this unusual disorder of oral motor control.
机译:特定于任务的地方性肌张力障碍(FTSD)是一种异常的运动控制障碍,通常会影响手,但也可能涉及面部,下颌和舌头。我们报告了89位音乐家,他们的手掌肌张力障碍(ED),下脸,下颌和舌头的肌肉用来控制空气流向木管乐器或铜管乐器的吹口。 ED的症状始于平均36岁,通常无痛且很少有外伤。特定的音乐技巧通常会触发肌张力障碍,通常是在一种器乐演奏中。在高配准黄铜演奏者(小号和圆号)中,特定于任务的手袋震颤和拔唇ED表型是常见的,而在低配准黄铜演奏者(长号和大号)中仅发生唇锁。下颌和舌头ED表型主要发生在木管乐器演奏者中,并且一旦出现,经常会传播到说话或进食。所有ED患者中有6%患有同时性的抽筋,提示可能有遗传倾向导致肌张力障碍。我们评估了ED患者,正常音乐人和非音乐人年龄匹配的对照者在上唇,下唇和手部的两点感觉辨别力-两组之间没有差异。一旦出现,ED的症状就不会缓解,并且经常破坏职业和生计。对于这种异常的口腔运动控制异常,迫切需要更好的治疗方法。

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