Recurrent hemichorea in an adolescent with systemic lupus erythematosus and previous ipsilateral cerebral infarction
Ya-Wen Yu1, Cheng-Yu Chung2, Yee-Hsuan Chiou1,3
1Division of Pediatric Allergy Immunology and Rheumatology, Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.
Insights
Systemic lupus erythematosus (SLE) can cause chorea, a movement disorder linked to antiphospholipid antibodies. This case highlights unexplained hemichorea in an SLE patient despite normal brain imaging, suggesting further research is needed.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) is an autoimmune disease known to cause various neurological complications.
- Chorea, characterized by involuntary, rapid, jerky movements, is a recognized but often inconsistently presenting neurological manifestation in SLE.
- Antiphospholipid (aPL) antibodies are frequently implicated in the pathogenesis of neurological disorders associated with SLE, including chorea.
Observation:
- A 15-year-old female with a history of SLE and cerebral infarction presented with recurrent left-sided hemichorea.
- Elevated antiphospholipid antibody levels were detected in the patient.
- Serial brain magnetic resonance imaging (MRI) scans, including perfusion studies, did not reveal acute lesions or explain the asymmetric hemichorea during episodes.
Findings:
- The case demonstrates a discrepancy between clinical presentation (hemichorea) and neuroimaging findings in SLE.
- Recurrent hemichorea in this SLE patient with elevated aPL antibodies occurred without detectable acute cerebral lesions on MRI.
- The pathophysiology of chorea in SLE, particularly its asymmetric manifestation and association with aPL antibodies, remains incompletely understood.
Implications:
- This case underscores the challenges in diagnosing and understanding chorea in SLE patients, especially when neuroimaging is inconclusive.
- Further research into the mechanisms underlying chorea in SLE is crucial for improving diagnostic accuracy and patient prognosis.
- Investigating novel imaging or diagnostic markers may be necessary to elucidate the pathophysiology of movement disorders in SLE.
Abstract:
Systemic lupus erythematosus (SLE) can present with movement disorders, among which chorea is closely associated with antiphospholipid (aPL) antibodies. Brain imaging results obtained in patients with chorea are generally inconsistent with the clinical manifestation of chorea; moreover, medical tests for hemichorea, which are expected to reveal distinct localization, may show negative findings. Herein, we present a case of a 15-year-old girl with SLE who had a history of left cerebral infarction; tests revealed elevated aPL levels, and she developed recurrent left hemichorea 2 years later. Brain magnetic resonance imaging (MRI) results revealed no acute lesions during each episode of involuntary movements, and an MRI perfusion scan failed to provide an explanation for the asymmetric presentation. Although various hypotheses have been proposed regarding the mechanism underlying the occurrence of chorea, some scenarios still remain unexplained. Further investigation on the pathophysiology of chorea in SLE may be warranted to clarify its prognosis.
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