Cardoso Francisco
Movement Disorders Clinic, Neurology Service, Department of Internal Medicine, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil.
Handb Clin Neurol. 2011;100:221-9. doi: 10.1016/B978-0-444-52014-2.00014-8.
Sydenham's chorea (SC) is the neurologic expression of acute rheumatic fever (ARF). Despite the declining incidence of ARF worldwide, it remains the most common cause of acute chorea in children. It is characterized by a combination of motor and nonmotor features. In addition to chorea, among the first are decreased muscle tone and tics. Nonmotor features include obsessions, compulsions, attention deficit, emotional lability, decreased verbal fluency, and executive dysfunction. Most patients present with nonneurologic features of ARF, such as carditis and arthritis. The pathogenesis is thought to involve streptococcus-induced antibodies which cross-react with antigens of the basal ganglia. The diagnosis is made on purely clinical grounds since there is no biological marker of the illness. The management is based on use of antichoreic agents, such as valproic acid and neuroleptics, and prophylaxis of new bouts of streptococcus infection with antibiotics. Although the motor features of SC come into spontaneous remission in the majority of patients, a significant proportion of individuals remain with persistent chorea.
Sydenham舞蹈病（SC）是急性风湿热（ARF）的神经系统表现。尽管全球范围内ARF的发病率在下降，但它仍是儿童急性舞蹈病最常见的病因。其特征是运动和非运动特征的结合。除舞蹈病外，首先出现的是肌张力降低和抽搐。非运动特征包括强迫观念、强迫行为、注意力缺陷、情绪不稳定、言语流畅性降低和执行功能障碍。大多数患者会出现ARF的非神经系统特征，如心脏炎和关节炎。其发病机制被认为涉及链球菌诱导的抗体，这些抗体与基底神经节的抗原发生交叉反应。由于该疾病没有生物学标志物，诊断完全基于临床依据。治疗基于使用抗舞蹈病药物，如丙戊酸和抗精神病药物，以及用抗生素预防链球菌感染的新发作。尽管大多数患者的SC运动特征会自发缓解，但仍有相当一部分人会持续存在舞蹈病。
