Torres-Russotto Diego, Perlmutter Joel S
Department of Neurology, Washington University in St. Louis, St. Louis, Missouri, USA.
J Hand Surg Am. 2008 Nov;33(9):1657-8. doi: 10.1016/j.jhsa.2008.09.001.
Hand dystonia can cause substantial functional disability and is frequently misdiagnosed. In general, dystonia is a disabling disorder of motor control characterized by excessive muscle contractions that can produce involuntary movements and abnormal postures. Prevalence of dystonia can be as high as 1 in 2500 people. Dystonias can be classified based on etiology, age of onset, anatomical part affected, and their tempo. Etiologically, they are classified as primary when the dystonia is the main sign and the cause is genetic or unknown and secondary when there are other disease manifestations and the cause may be identifiable. Dystonias that start before 27 years of age are called childhood-onset dystonia; they usually start in the lower limbs, trunk, or upper extremities and frequently spread to the rest of the body. Adult-onset dystonias usually begin in the upper half of the body, and the risk of progression to other body parts depends on the anatomic site of onset. Anatomically, dystonias can be focal (1 body part), segmental (2 or more contiguous body parts), multifocal (2 noncontiguous areas), hemidystonia, or generalized. Based on their tempo, dystonias can be constant, intermittent, or situational, the latter including task-specific dystonias. The rest of the review focuses on the clinical manifestations, differential diagnosis, pathophysiology, and treatment of hand dystonia.
手部肌张力障碍可导致严重的功能残疾,且常被误诊。一般来说,肌张力障碍是一种运动控制方面的致残性疾病,其特征为肌肉过度收缩,可产生不自主运动和异常姿势。肌张力障碍的患病率高达每2500人中就有1人患病。肌张力障碍可根据病因、发病年龄、受累解剖部位及其发作节奏进行分类。从病因学角度来看,当肌张力障碍为主要症状且病因是遗传或不明时,被归类为原发性;当存在其他疾病表现且病因可能可确定时,则归类为继发性。在27岁之前发病的肌张力障碍称为儿童期起病的肌张力障碍;其通常始于下肢、躯干或上肢,并常蔓延至身体其他部位。成人起病的肌张力障碍通常始于身体上半部分,进展至身体其他部位的风险取决于发病的解剖部位。从解剖学角度,肌张力障碍可分为局灶性(1个身体部位)、节段性(2个或更多相邻身体部位)、多灶性(2个不相邻区域)、偏侧肌张力障碍或全身性。根据发作节奏,肌张力障碍可为持续性、间歇性或情境性,后者包括特定任务性肌张力障碍。本文综述的其余部分聚焦于手部肌张力障碍的临床表现、鉴别诊断、病理生理学及治疗。