西德纳姆舞蹈病:临床和实验室结果。187例分析。

Revista paulista de medicina Pub Date : 1992-07-01
J Goldenberg, M B Ferraz, A S Fonseca, M O Hilário, W Bastos, S Sachetti
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引用次数: 0

摘要

1686年,托马斯·西德纳姆(Thomas Sydenham)描述了西德纳姆舞蹈病(小舞蹈病、圣·维图斯舞蹈病、风湿性脑炎),被认为是风湿热的主要表现之一(1,2,3,4)。临床表现为不自主运动、强张、构音障碍、情绪障碍,其他神经系统表现如虚弱、头痛、癫痫发作和感觉异常等较少出现(1,4)。运动障碍可能是全身性的或单侧的,在这种情况下构成局部舞蹈病(3)。在急性发作期间,舞蹈病可能与其他风湿热表现相关,或以孤立形式出现,即所谓的“纯”舞蹈病(5,6,7)。其病因和病理生理机制尚不清楚。虽然它与先前的a组-溶血性链球菌感染的病理生理关系已经得到证实(8)。也有证据表明免疫机制参与了其发病机制,如发现血清抗尾状核和抗丘脑底抗体(9),以及舞蹈病患者脑脊液中IgG水平升高(10)。在发达国家,由于风湿热发病率的减少和以舞蹈病为表现的频率的减少(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,在过去几年中,作为风湿热临床表现的一部分,已经观察到舞蹈病发病率的增加(12)。本研究报告了1980年1月至1990年12月在巴西圣保罗市的两所大学中心随访的187例西德纳姆舞蹈病的临床和实验室结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sydenham chorea: clinical and laboratory findings. Analysis of 187 cases.

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.

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