5 Discussion
This case report presents a 12year old girl, whose SLE diagnosis was revealed by a new onset chorea as part of acute neurological manifestation of SLE along with, bicytopenia, elevated titers of anti-dsDNA antibodies, positive antinuclear antibodies, and positive antiphospholipid antibodies.
While chorea can manifest as a symptom in various diseases, when a child exhibits generalized chorea as the sole symptom, Sydenham’s chorea is typically the initial diagnosis in regions including Nepal with a high prevalence of Rheumatic Heart Disease.5The observation of leukopenia, lymphopenia, and an elevated erythrocyte sedimentation rate (ESR) raised concerns, leading to the search for an alternative diagnosis that could encompass the multisystem manifestations. Hence, the investigations for autoimmune disease were performed, ultimately revealing a diagnosis of Systemic Lupus Erythematosus (SLE).
SLE is a common diagnosis in the pediatric population.However it mostly presents in the form of lupus nephritis.6 The American College of Rheumatology (ACR) nomenclature for neuropsychiatry syndromes in SLE includes 12 central nervous system syndromes and 7 peripheral nervous system syndromes.There is a wide variation in the frequency of NPSLE in various studies. Mild cognitive dysfunction and mood disorders are frequently seen as manifestations of NPSLE (6-80%) whereas movement disorders including chorea are extremely rare (< 1% of SLE patients.7
Neuropsychiatric(NP) manifestations are a rare presentation of Lupus.In a study carried out in Turkey among 1107 juvenile SLE patients 149 patients had  NP involvement (13.5%). The most common NPSLE findings were headache (50.3%), seizure (38.3%), and acute confusional state (33.6%). A study found the prevalence of movement disorders in SLE to be 9.4%.8
Chorea as the sole initial presentation of SLE remains rare across all age groups. It manifests in only around 1–3% of cases of SLE.9
The exact mechanism behind lupus-associated chorea remains uncertain, but it is potentially linked to damage in the vascular, neuronal, and glial components. There is a high incidence of antiphospholipid antibodies (aPL) in studies of SLE patients with neuropsychiatric manifestations, between 25 and 90%, perhaps implicating a role of these antibodies in the pathogenesis.10Vascular injury may involve both inflammatory and thrombotic processes. The presence of aPL appears to have a significant impact on the pathogenesis of neuropsychiatric systemic lupus erythematosus (SLE)11
The European League Against Rheumatism (EULAR) recommends antiplatelet therapy for SLE patients with movement disorders and positive aPL antibodies. Anticoagulation agents are only suggested for patients with thrombotic manifestations. As there are no specific guidelines for children with neuropsychiatric SLE, the management should be individualized and based on the decision of the treating clinician.12
As chorea is a frequent manifestation of rheumatic fever, the diagnosis of systemic lupus erythematosus (SLE) can be delayed, particularly in regions where rheumatic fever is widespread. Despite its rarity, it is essential to consider the possibility of childhood SLE in the differential diagnosis of any child presenting with unexplained neurological symptoms.