Chorea Revealing Antiphospholipid Syndrome: Implications for Stroke Secondary Prevention
Objective:
To present a case in which new-onset chorea revealed an underlying antiphospholipid syndrome (APS) in a patient previously diagnosed with atrial fibrillation (AF) - related ischemic strokes, leading to a pivotal change in secondary stroke prevention.
Background:
Antiphospholipid syndrome is an autoimmune, prothrombotic condition characterized by arterial and venous thrombosis, pregnancy morbidity, and a broad spectrum of neurological manifestations. Up to 30% of patients develop neurological symptoms due to cerebral ischemia, endothelial injury, or antibody-mediated neuronal dysfunction. Among these, ischemic stroke and cognitive impairment predominate, while chorea, occurring in only 1-4% of the cases, represents a distinctive and often underrecognized manifestation. The proposed pathophysiology includes both microvascular ischemia of the basal ganglia and direct neuronal effects of antiphospholipid antibodies, emphasizing the link between immune dysregulation and movement disorders.
Design/Methods:
A 79-year-old man with hypertension, atrial fibrillation (status post-left atrial appendage occlusion), and prior right middle cerebral artery territory ischemic strokes, most recently in April 2023, presented in January 2024 with two months of progressive involuntary movements involving the trunk and limbs. Symptoms improved with benzodiazepines and alcohol. Examination showed diffuse generalized choreiform movements, mild gait ataxia, and dysdiadochokinesia.
Results:
Hypercoagulable testing revealed elevated anticardiolipin and β2-glycoprotein antibodies (IgM/IgG/IgA), confirming APS diagnosis. Anticoagulation was switched from apixaban to warfarin for optimal secondary stroke prevention. Deutetrabenazine was initiated and titrated gradually to 24mg daily, resulting in marked improvement of both chorea and coordination.
Conclusions:
This case highlights the importance of recognizing APS as a potential unifying etiology in patients with recurrent thrombotic strokes and new-onset chorea. Early identification can prompt critical changes in secondary prevention strategies, transforming management, and improving neurological outcomes.
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