Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood.
Three patients, aged five to 16 years, developed chorea as the only or main clinical manifestation of primary antiphospholipid syndrome. In two cases, complaints were self-limited five to eight months after onset. In one patient, the clinical course was complicated by valvulitis. Under corticosteroid treatment, chorea disappeared and cardiac involvement stabilised. Primary antiphospholipid syndrome is a probably under-recognised differential diagnosis of choreatic syndromes in childhood. Assessment of anticardiolipin antibodies and/or lupus anticoagulant should be an obligatory part of the diagnostic work-up of such patients. Early diagnosis of primary antiphospholipid syndrome may improve clinical management and prognosis.
Department of Paediatrics, University of Innsbruck, Austria.
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Kiechl-Kohlendorfer
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H
Ellemunter
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Kiechl
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D017153:Antibodies, Anticardiolipin / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D001241:Aspirin / Q000627:therapeutic use; D002675:Child, Preschool; D002819:Chorea / Q000209:etiology*; D005260:Female; D006351:Heart Valves / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D012075:Remission, Spontaneous; D013921:Thrombocytopenia / Q000209:etiology; D016896:Treatment Outcome; D014463:Ultrasonography
D000305:Adrenal Cortex Hormones; D017153:Antibodies, Anticardiolipin; D000974:Antibodies, Antinuclear; D016682:Lupus Coagulation Inhibitor; D001241:Aspirin