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Systemic Lupus Erythematosus (SLE) of pediatric onset represents 10% to 20% of all SLE cases. Neuropsychiatric manifestations are found in up to 50% of children with SLE within the first year from the time of diagnosis.  Although chorea is a rare neuropsychiatric manifestation of SLE, when present, it is usually associated with the presence of antiphospholipid antibodies (aPL).   Reports of patients with SLE chorea without aPL antibodies suggest that at least in some cases there is a different pathophysiologic mechanism. We describe the case of a six-year-old boy with a diagnosis of SLE that presented with lupus nephritis and subsequently experienced left hemichorea without MRI evidence of brain lesions.  Antiphospholipid panel was negative.  Brain SPECT revealed moderately decreased perfusion-metabolism in the right posterior parietal and occipital cortex suggestive of vasculitis. Patient was treated for a CNS vasculitis and symptoms of chorea, which resolved within several months.  This case highlights the major clinical differences between adult and pediatric onset SLE and demonstrates that in SLE patients with negative aPL, CNS vasculitis may be the underlying etiology for the neuropsychiatric manifestations, including chorea.

chorea; systemic lupus erythematsus; central nervous system vasculitis; children