Case summaryHistory A 5-year-old Japanese boy was admitted to another hospital with palpable purpura, abdominal pain, and bilateral ankle pain and swelling 1 day after receiving an inactivated influenza vaccination. One year previously, he had been admitted to the same hospital because of HenochSchönlein purpura (HSP), which had never recurred until his present illness. He had not been recently given any drugs or contracted any infectious disease except for the influenza vaccine. On admission, he had normal serum C3, C4, and CH50 titers, normothrombocytemia, and no antiphospholipid antibodies (aPLs) including lupus anticoagulant or anti-cardiolipin antibodies. Hemagglutination inhibition tests for influenza A revealed an elevation of anti-influenza A H3N2 antibody (×128). Intravenous corticosteroid therapy improved his condition rapidly; however, his abdominal pain and purpura recurred following decreases in steroid dosage. Two weeks following the onset of his illness, he was transferred to our hospital. Clinical findings on admissionPhysical examination revealed a generalized palpable purpuric eruption, left ankle joint swelling and pain, and abdominal pain and tenderness. His blood pressure, pulse rate, and temperature were normal. Laboratory findingsLaboratory studies revealed normal values for the following: complete blood cell count, platelet count, erythrocyte sedimentation rate, electrolytes, liver enzymes, blood urea nitrogen, creatinine, urinalysis, C-reactive protein, IgG, IgA, IgM, IgE, egg-specific IgE and anti-streptolysin O titer. Tests for hepatitis B surface antigen, antinuclear antibody, circulating immune complexes, rheumatoid factor, and VDRL were negative. Bacterial and viral cultures from a throat swab grew no pathogenic organisms. Hemagglutination inhibition tests for influenza A revealed an elevation of anti-influenza A H3N2 titer (×256). The C4 and CH50 levels were slightly depressed to 11 mg/dl (normal 13-34 mg/dl) and 26.5 IU/l (normal >30.0 IU/l), respectively. Lupus anticoagulant, detected on the basis of a prolonged activated partial thromboplastin time, a positive result of the mixing study and a confirmatory test with hexagonal phase phospholipid, was present, and anti-cardiolipin IgG antibody was slightly increased to 14 U/ml (ELISA: normal <10 U/ml). Treatment and subsequent courseOral corticosteroid therapy promptly improved his abdominal symptoms again, and was tapered gradually during the subsequent month without recurrence of abdominal symptoms. One month later, while titer of anti-cardiolipin IgG antibody had decreased into the normal ranges, lupus anticoagulant was still present. The C4 and CH50 levels had increased into the normal ranges. He was discharged on the 40th hospital day without any sequelae. Two months after being discharged, the lupus anticoagulant had disappeared.
Although central nervous system involvement is an important manifestation of systemic lupus erythematosus (SLE), chorea is a relatively uncommon complication. A strong association between chorea and the presence of antiphospholipid antibodies (aPLs) has been reported in patients with SLE, lupus-like disease, or primary antiphospholipid syndrome. We describe a patient with lupus nephritis and cerebral infarction, who subsequently developed recurrent hemichorea associated with increased aPLs levels. A 7-year-old boy suffered from lupus nephritis and a left middle cerebral artery infarction associated with aPLs. He subsequently experienced two episodes of right hemichorea associated with increased aPLs levels without any evidence of further neurological lesions by brain computed tomography or magnetic resonance imaging. The previous left cerebral artery infarction might have increased the susceptibility of the left basal ganglia to the effects of aPLs that contributed to the development of the right hemichorea in this patient.
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