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[舞蹈病与亨廷顿舞蹈症]

[Chorea and Huntington's disease].

作者信息

Dürr A

机构信息

INSERM U 289 et Fédération de neurologie Hôpital de la Salpêtrière, Paris.

出版信息

Rev Prat. 1997 May 15;47(10):1083-7.

PMID:9208671
Abstract

The causes of chorea are numerous and it is convenient to distinguish hereditary chorea and acquired syndromes with chorea. Huntington's disease represents the most typical disorder with chorea and the clinical diagnosis must be considered if there is chorea intellectual decline and autosomal dominant inheritance. The availability of molecular analysis for diagnostic confirmation shows that the clinical diagnosis in experienced centers is very accurate. Predictive testing involves several ethical problems, especially related to the severity of the disease and its absence of treatment. The other causes of hereditary chorea are less frequent and specific clinical contexts are evoqual of acquired choreas.

摘要

舞蹈症的病因众多,区分遗传性舞蹈症和后天性舞蹈症综合征较为简便。亨廷顿舞蹈病是最典型的伴有舞蹈症的疾病,如果存在舞蹈症、智力衰退和常染色体显性遗传,就必须考虑临床诊断。用于确诊的分子分析方法表明,经验丰富的中心所做的临床诊断非常准确。预测性检测涉及若干伦理问题,尤其是与疾病的严重程度及其无有效治疗方法有关的问题。遗传性舞蹈症的其他病因较少见,特定的临床情况与后天性舞蹈症相似。

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