Acute chorea: case series from the emergency room of a Brazilian tertiary-level center

ABSTRACT Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. Methods: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. Results: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. Conclusion: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).

【摘要】背景：舞蹈症（chorea）是一种以随机、短暂且游走性不自主肌肉收缩为特征的运动障碍。若症状于数小时至数天内出现，则被定义为急性舞蹈症。目前已明确该病症的三大主要病因：血管性、毒性代谢性及炎症性。目的：本研究旨在明确某三级转诊中心急诊室急性舞蹈症的主要病因患病率与核心临床特征，并提出可指导诊断评估与临床管理的方案。方法：我们回顾性分析了2015至2019年本院神经内科急诊室收治的10例急性舞蹈症患者的临床资料与神经影像学（neuroimaging）数据。结果：卒中为最常见病因（占总病例的50%），且全部为缺血性卒中。值得注意的是，仅1例患者在对侧丘脑下核出现典型缺血性病灶。非血管性病因中，非酮症性高血糖为首要诱因，其次为药物相关性舞蹈症。1例患者的病因考虑为炎症性，极可能为西德纳姆舞蹈症（Sydenham chorea）复发。结论：急性舞蹈症在急诊诊疗中较为少见且颇具挑战性，其病因多具备潜在可治疗性。我们建议采用首字母缩略词DANCE（舞蹈症诊断、急性卒中诊疗流程、血糖水平正常、神经影像学检查、药物暴露史排查）作为初始评估方案，以强调需要及时妥善处理的病因（如溶栓治疗）。