Objective: To report a case of acute chorea syndrome in a young patient, secondary to Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS).

Background: Movement disorders (MD) may occur in SLE and chorea is the most common presentation. These MDs may also be accompanied by other neuropsychiatric changes. About 90% of patients with chorea and SLE have positive antiphospholipid antibodies (aPL). It is believed that the inflammation related to aPL leads to a breakdown of the blood-brain barrier, allowing the passage of other antibodies to the central nervous system [1].

Method: This is a case report study

Results: A 23-year-old patient female patient was admitted to the emergency department with a 3-day history of involuntary hyperkinetic movements characterized as a generalized choreoathetosis, without other neurological signs. The movements started in the upper limbs and progressed to the other extremities within 48 hours. She had no relevant family history, was not on medication and had a previous diagnosis of a deep vein thrombosis 1 year ago. Laboratory investigations revealed a reagent ANA title of 1:640, showing a nuclear pattern and speckled cytoplasm, and anticardiolipin antibody levels were IgM 11,3MPL/ml and IgG 61,3MPL/ml. The cerebrospinal fluid (CSF) was normal. Her brain MRI showed multiple diffusion restriction foci and high signal flair in the white matter lesions. Therefore, she was diagnosed with SLE and APS according to the ACR/EULAR criteria. Thus, IV methylprednisolone (1g/day for 5 days) was prescribed, with complete remission of hyperkinetic movements. Aspirin and oral anticoagulation were started due to the diagnosis of APS and evidence of thrombotic events.

Conclusion: This case report highlights the recognition of SLE and APS in the differential diagnosis of acute chorea etiology, especially because the response to early treatment may be lifesaving. Chorea may be a manifestation of a primary neurological disease or may be a neurological complication of other systemic disorder [2]. Other than that, chorea occurs in 1.3-4.5% of patients with APS and may be the first manifestation, as it was in the presenting case [3]. Therefore, a complete clinical approach in a patient with acute chorea presenting to the emergency department is needed.

References: 1.Baizabal-Carvallo, J. F., Bonnet, C., & Jankovic, J. (2013). Movement disorders in systemic lupus erythematosus and the antiphospholipid syndrome. Journal of neural transmission (Vienna, Austria : 1996), 120(11), 1579–1589. https://doi.org/10.1007/s00702-013-1023-z
2.Jankovic, J., Hallett, M., Okun, M. S., Comella, C., Fahn, S., & Goldman, J. (2021). Principles and Practice of Movement Disorders. Elsevier. https://doi.org/10.1016/B978-0-323-31071-0.01001-5
3.Fleetwood T, Cantello R and Comi C (2018) Antiphospholipid Syndrome and the Neurologist: From Pathogenesis to Therapy. Front. Neurol. 9:1001. doi: 10.3389/fneur.2018.01001

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MDS Abstracts - https://www.mdsabstracts.org/abstract/acute-chorea-in-the-emergency-department-related-to-systemic-lupus-and-antiphospholipid-syndrome-a-case-report/