Chorea can be associated with autoimmune diseases such as antiphospholipid syndrome and has been associated with the isolated presence of antiphospholipid antibodies (aPL). Chorea is a rare neurological manifestation of antiphospholipid syndrome. The pathophysiological mechanisms underlying aPL-related chorea are still debated. One postulated mechanism is aPL or other autoantibody binding to brain-blood vessel endothelium, resulting in endothelial dysfunction secondary to a proinflammatory cascade, with sequalae of inflammation and local microthrombosis. Another postulated mechanism considers immune-mediated attack (aPL or antibasal ganglia antibodies) against specific basal ganglia epitopes. Here, we report a patient with isolated aPL-related chorea that followed a relapsing-remitting course. We highlight the role of brain metabolic imaging with fluorodeoxy glucose positron-emission tomography in the diagnostic workup of chorea and the challenges in the practical management of aPL-related chorea with symptomatic treatments.
- MOVEMENT DISORDERS
- CLINICAL NEUROLOGY
- PET, FUNCTIONAL IMAGING
Data availability statement
Data sharing not applicable as no datasets generated and/or analysed for this study.
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Contributors MF was involved in conception, data analysis, design and drafting the manuscript. MF, BH and TA were involved in the clinical care of the patient. BH and TA were involved in critical review and final approval of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Disclaimer All authors satisfy the ICMJE criteria for authorship.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed by Neil Scolding, Bristol, UK.