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Screening for autoantibodies in children with opsoclonus-myoclonus-ataxia

2002; Elsevier BV; Volume: 27; Issue: 5 Linguagem: Inglês

10.1016/s0887-8994(02)00457-5

1873-5150

Michael R. Pranzatelli, Elizabeth D. Tate, Alisa Wheeler, Nancy Bass, Arnold P. Gold, May L. Griebel, Maria Gumbinas, Peter Heydemann, Philip J. Holt, Pierre Jacob, Suresh Kotagal, Chester J Minarcik, Howard S Schub,

Myasthenia Gravis and Thymoma

Various paraneoplastic autoantibodies have been linked to discrete neurologic syndromes and tumors in adults, but little is known about their incidence in children. We report a cross-sectional study of known paraneoplastic antibodies in 59 children with opsoclonus-myoclonus-ataxia, 86% of whom were moderately or severely symptomatic, and 68% of whom had relapsed at the time of testing. This total number of patients includes 18 children with low-stage neuroblastoma (tested after tumor resection), six of whom had never been treated with immunosuppressants. All were seronegative for anti-Hu, anti-Ri, and anti-Yo, the three paraneoplastic antibodies most associated with opsoclonus-myoclonus or ataxia in adults. These data contrast with reports of anti-Hu-positive sera in children with high-stage tumors and suggest that anti-Hu, anti-Ri, and anti-Yo do not explain relapses in pediatric opsoclonus-myoclonus-ataxia. They underscore the need to search for unique autoantibodies, as well as cellular mechanisms of pediatric paraneoplastic disease.