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西德纳姆舞蹈病

Sydenham's chorea.

作者信息

Kulkarni M L, Anees S

机构信息

Department of Pediatrics, J.J.M. Medical College, Davangere.

出版信息

Indian Pediatr. 1996 Feb;33(2):112-5.

PMID:8772928
Abstract

OBJECTIVE

To study the clinical profile in patients with Sydenham's chorea.

DESIGN

Prospective.

SETTING

Medical college and hospital.

SUBJECTS

Sixty cases with Sydenham's chorea, between 1988 and 1994, were studied. Of these 36 were girls; the mean age at presentation was 11.1 yr (range 7-16 yr).

RESULTS

Female predominance was apparent only after 9 years of age. There was a high familial incidence for both chorea and rheumatic fever. Generalized chorea was seen in 40 and hemichorea in 20 patients. The chorea lasted from 5 to 40 days; 13 patients had recurrent episodes. Gait disturbances, dysarthria and weakness were common. Six patients had co-existing heart disease, 4 had arthritis and one had subcutaneous nodules. A raised ESR and positive ASO titer were seen in 37 and 20 patients respectively.

CONCLUSIONS

There was an increased incidence of Sydenham's chorea after 9 years of age in girls possibly suggesting the influence of female sex hormones. A high incidence of neurologic manifestations were noted. Acute phase reactants were raised in almost half the patients. Sodium valproate, whenever used, was effective in controlling the chorea.

摘要

目的

研究 Sydenham 舞蹈病患者的临床特征。

设计

前瞻性研究。

地点

医学院及附属医院。

研究对象

对1988年至1994年间的60例 Sydenham 舞蹈病患者进行研究。其中36例为女孩;就诊时的平均年龄为11.1岁(范围7 - 16岁)。

结果

仅在9岁以后女性优势才明显显现。舞蹈病和风湿热的家族发病率都很高。40例患者出现全身性舞蹈病,20例出现偏侧舞蹈病。舞蹈病持续5至40天;13例患者有复发发作。步态障碍、构音障碍和肌无力很常见。6例患者合并有心脏病,4例有关节炎,1例有皮下结节。分别有37例和20例患者血沉升高和抗链球菌溶血素O滴度呈阳性。

结论

9岁以后女孩中 Sydenham 舞蹈病的发病率增加,这可能提示女性性激素的影响。观察到神经学表现的高发病率。几乎一半的患者急性期反应物升高。丙戊酸钠无论何时使用,对控制舞蹈病均有效。

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