Category: Autoimmune Movement Disorders
Objective: To describe chorea as the initial manifestation of antiphospholipid syndrome (APS) and its radiological correlation on brain FDG-PET as an autoimmune movement disorder.
Background: APS is a systemic autoimmune disease characterized by arterial and/or venous thrombosis and obstetric morbidity associated with persistent anticardiolipin antibodies, anti-β2 glycoprotein-1 (anti-β2GP1), and/or lupus anticoagulant1. Chorea associated with APS is an uncommon neurological manifestation. Its pathophysiology is thought to involve antibody binding to the vascular endothelium of cerebral vessels, leading to microthrombotic and inflammatory phenomena affecting the basal ganglia2. Brain FDG-PET may be useful in the etiological work-up, showing basal ganglia hypermetabolism and supporting an inflammatory etiology3
Method: An 82-year-old man with a history of atrial fibrillation treated with rivaroxaban and no family history of neurological or psychiatric disease was admitted with a four-month history of progressive cognitive decline and abnormal movements. Neurological examination showed somnolence and temporal disorientation, orolingual dyskinesias and intermittent choreic movements of the right lower limb.
Results: Laboratory studies revealed euvolemic hypoosmolar hyponatremia (126 mEq/L) compatible with SIADH and positivity for anti-β2GP1 IgM antibodies (49.35 U/mL). Brain MRI showed supratentorial microangiopathy, a chronic right pontine infarction and right mesial temporal sclerosis. Lumbar puncture revealed lymphocytic pleocytosis (90 leukocytes/µL), with negative oligoclonal bands and neuronal antibodies. Given the suspicion of autoimmune etiology, treatment with methylprednisolone (1 g/day for 5 days) followed by intravenous immunoglobulins (25 g for 5 days) was administered, with improvement of cognitive symptoms and abnormal movements. Monthly intravenous immunoglobulin therapy was initiated.
Conclusion: APS-associated chorea is a potentially treatable autoimmune cause of chorea. Basal ganglia hypermetabolism on brain FDG-PET may help support the diagnosis of an autoimmune etiology.
References: 1. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost [Internet]. 2006;4(2):295–306. Disponible en: http://dx.doi.org/10.1111/j.1538-7836.2006.01753.x
2. Farag M, Hunt BJ, Andrews TC. Antiphospholipid-related chorea. Pract Neurol [Internet]. 2023;23(2):150–2. Disponible en: http://dx.doi.org/10.1136/pn-2022-003587
3. Lerjefors L, Andretta S, Bonato G, Mainardi M, Carecchio M, Antonini A. Antiphospholipid-related chorea: Two case reports and role of metabolic imaging. Mov Disord Clin Pract [Internet]. 2022;9(4):516–21. Disponible en: http://dx.doi.org/10.1002/mdc3.13432
To cite this abstract in AMA style:
A. Bonilla Tena, G. Lafuente Gómez, A. Contreras Chicote, B. Casa Fages, J. Pérez Sánchez, E. Luque Buzo, V. Pérez Olmo, F. Grandas Pérez. Late onset chorea secondary to antiphospholipid syndrome with radiological correlation on brain FDG-PET: a case report. [abstract]. Mov Disord. 2026; 41 (suppl 1). https://www.mdsabstracts.org/abstract/late-onset-chorea-secondary-to-antiphospholipid-syndrome-with-radiological-correlation-on-brain-fdg-pet-a-case-report/. Accessed October 1, 2026.« Back to 2026 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/late-onset-chorea-secondary-to-antiphospholipid-syndrome-with-radiological-correlation-on-brain-fdg-pet-a-case-report/