Hemichorea as the First and Sole Manifestation in Lupus: Case-Based Review

Considering her age, raised ESR and prolonged APTT, SLE with anti-phospholipid antibody syndrome was strongly suspected and further evaluation revealed positive anti-nuclear antibody (ANA) by indirect immunofluorescence (IF)- 3+ nuclear homogenous pattern in 1:100 dilution, raised anti-double stranded DNA (Anti dsDNA)- 125 IU/ml (0-25 IU/ml), low C3- 47mg/dl (90-180mg/dl), low C4- 3.07mg/dl (10-40mg/dl) and positive lupus anticoagulant 1.94 (0-1.3). Magnetic resonance imaging (MRI) Brain was normal and Digital subtraction angiography (DSA) did not show any evidence of CNS vasculitis. [...]a final diagnosis of SLE with neuropsychiatric lupus in the form of left hemichorea and secondary antiphospholipid antibody syndrome was made. The American College of Rheumatology (ACR) nomenclature for neuropsychiatry syndromes in SLE includes 12 central nervous system syndromes and 7 peripheral nervous system syndromes.2 The classification of pathogenic mechanisms responsible for various neuropsychiatry syndromes into ischemic and neuroinflammatory may be an oversimplification of this clinically challenging entity. In prior case reports where chorea has been the presenting feature of SLE, the onset of other clinical features of SLE has varied from being present during the initial evaluation of chorea to a few weeks after the onset of chorea.9-13 The exact pathogenesis of chorea in SLE may be multifactorial involving 1) ischemia of small and large vessels in CNS mediated by anti-phospholipid antibodies and immune complexes, and 2) inflammatory neuronal injury secondary to complement activation, inflammatory cytokines, and increased permeability of blood-brain barrier causing autoantibodies to migrate to the intrathecal space.