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西德纳姆舞蹈病

Sydenham's Chorea.

作者信息

Risavi Brian L, Iszkula Erik, Yost Bryan

机构信息

Department of Emergency Medicine, UPMC Hamot, University of Pittsburgh Medical Center (UPMC), Erie, Pennsylvania.

出版信息

J Emerg Med. 2019 Jun;56(6):e119-e121. doi: 10.1016/j.jemermed.2019.02.012. Epub 2019 Apr 16.

Abstract

BACKGROUND

Sydenham's chorea is the most common acquired movement disorder of adolescence. This clinical manifestation of acute rheumatic fever has a clear and documented relationship with Group A streptococcal infections. The symptoms are involuntary choreiform movements that can affect the face and all extremities. The pathophysiology remains unclear.

CASE REPORT

A 12-year-old female was brought to the emergency department with a 2-week history of involuntary muscle spasms of her right arm and leg. Her parents reported intermittent slurred speech and difficulty grasping utensils. Physical examination revealed an awake, alert, age-appropriate female with normal cranial nerves. Patient was found to have choreoathetoid movements on the right extremities with dystonia of right leg with ambulation. Neurology consultation, computed tomography of the head, and magnetic resonance imaging of the brain did not show any acute pathology. Echocardiogram did show mild tricuspid regurgitation, suggestive of rheumatic fever. Anti-streptolysin O titer was markedly elevated, along with DNAse-B antibodies. The patient had marked improvement of movement disorder at just over 1 week later. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Sydenham's chorea is a rare but important movement disorder often related to Group A streptococcus and rheumatic fever. The incidence of rheumatic fever has been decreasing in North America but continues to be much more prevalent in developing countries as well as immigrant populations. This diagnosis is rare and can occasionally be misdiagnosed as a "fidgety" child or as a psychiatric manifestation. Sydenham's chorea is important to diagnose because acute treatment and prophylactic antibiotics can help improve symptoms and minimize cardiac damage.

摘要

背景

Sydenham舞蹈病是青少年时期最常见的后天性运动障碍。这种急性风湿热的临床表现与A组链球菌感染有着明确且有记录的关联。症状为不自主的舞蹈样动作,可累及面部和所有肢体。其病理生理学仍不清楚。

病例报告

一名12岁女性因右臂和右腿不自主肌肉痉挛2周被送至急诊科。她的父母报告称其言语间歇性含糊不清且抓握餐具困难。体格检查发现该女性神志清醒、警觉,颅神经正常。检查发现患者右上肢有舞蹈徐动样动作,行走时右腿有肌张力障碍。神经科会诊、头部计算机断层扫描和脑部磁共振成像均未显示任何急性病变。超声心动图显示轻度三尖瓣反流,提示风湿热。抗链球菌溶血素O滴度以及DNA酶B抗体明显升高。1周多后患者的运动障碍有明显改善。

为什么急诊医生应该了解这个疾病?:Sydenham舞蹈病是一种罕见但重要的运动障碍,常与A组链球菌和风湿热相关。在北美,风湿热的发病率一直在下降,但在发展中国家以及移民人群中仍然更为普遍。这种诊断很罕见,偶尔会被误诊为“坐立不安”的儿童或精神疾病表现。Sydenham舞蹈病的诊断很重要,因为急性治疗和预防性抗生素有助于改善症状并使心脏损害最小化。

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