Goldenberg J, Ferraz M B, Fonseca A S, Hilário M O, Bastos W, Sachetti S
Department of Medicine, Escola Paulista de Medicina, São Paulo, Brasil.
Rev Paul Med. 1992 Jul-Aug;110(4):152-7.
Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called "pure" chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil.
西德纳姆舞蹈病(小舞蹈病、圣维特舞蹈病、风湿性脑炎)由托马斯·西德纳姆于1686年描述,被认为是风湿热的主要表现之一(1, 2, 3, 4)。临床上,其特征为不自主运动、肌张力减退、构音障碍、情绪障碍,较少见的还有其他神经学表现,如无力、头痛、癫痫发作和感觉异常(1,4)。运动障碍可为全身性或单侧性,单侧时构成偏侧舞蹈症(3)。舞蹈病可在急性发作时与其他风湿热表现同时出现,或以孤立形式出现,即所谓的“纯”舞蹈病(5, 6, 7)。尽管其与先前A组β溶血性链球菌病理生理感染的关系已得到充分证实(8),但其病因和病理生理机制仍不清楚。也有证据表明免疫机制参与其发病过程,如发现血清抗尾状核抗体和抗丘脑底核抗体(9)以及舞蹈病患者脑脊液中IgG水平升高(10)。在发达国家,由于风湿热发病率降低以及舞蹈病作为其表现形式的频率下降(3, 11),后者已变得罕见。然而,在发展中国家,风湿热仍然是一个公共卫生问题。在巴西,近年来观察到舞蹈病发病率作为风湿热临床表现的一部分有所增加(12)。本研究报告了1980年1月至1990年12月期间在巴西圣保罗市的两个大学中心对187例西德纳姆舞蹈病患者进行随访的临床和实验室检查结果。