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).

Chorea

“…(HIV) and acquired immunodeficiency syndrome (AIDS). 16,22,23 Some conditions may cause hemichorea in the absence of an apparent brain lesion. Examples include autoimmune chorea (eg, Sydenham chorea), paraneoplastic syndromes, and variant Creutzfeldt-Jakob disease.…”

“…However, vascular lesions in other territories, such as the thalamus or temporoparietal cortex, have also been implicated in causing chorea 21 . Other etiologies that may cause hemichorea with focal lesions are nonketotic hyperglycemia and opportunistic infections in the setting of human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) 16,22,23 . Some conditions may cause hemichorea in the absence of an apparent brain lesion.…”

Emergencies in movement disorders

“…Chorea is a hyperkinetic movement disorder characterized by a continuous stream of involuntary muscular contractions that are random, short, and migratory (29). The development of acute chorea can be caused by a variety of factors, such as exposure to drugs, metabolic derangements, vascular illness, infectious/ postinfectious and autoimmune diseases.…”