Kiechl-Kohlendorfer U, Ellemunter H, Kiechl S
Department of Paediatrics, University of Innsbruck, Austria.
Neuropediatrics. 1999 Apr;30(2):96-8. doi: 10.1055/s-2007-973468.
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.
三名年龄在5至16岁的患者出现舞蹈症，这是原发性抗磷脂综合征唯一的或主要的临床表现。在两例患者中，症状在发病后五至八个月自行缓解。在一名患者中，临床病程因瓣膜炎而复杂化。在皮质类固醇治疗下，舞蹈症消失，心脏受累情况稳定。原发性抗磷脂综合征可能是儿童舞蹈症综合征中一个未得到充分认识的鉴别诊断。抗心磷脂抗体和/或狼疮抗凝物的评估应是此类患者诊断检查的必要部分。原发性抗磷脂综合征的早期诊断可能会改善临床管理和预后。
