Technical report: Transmissible spongiform encephalopathies: A review for pediatricians

Transmissible spongiform encephalopathies (TSEs) are a family of rare, slow ly progressive, and universally fatal neurodegenerative syndromes affecting animals and humans. Until recently, TSEs were of little interest to pediat ricians. However, since the outbreak in adolescents and the association of TSEs with new-variant Creutzfeldt-Jakob disease (nvCJD), interest among ped iatricians and the general public has increased. Even before bovine spongif orm encephalopathy and nvCJD were linked, the recognition that iatrogenic C reutzfeldt-Jakob disease (CJD) had been acquired from administration of cad averic human growth and gonadotropic hormones and from corneal and dura mat er transplants prompted medical vigilance. Furthermore, recent concern abou t the potential for transmission of CJD by blood and blood products has rai sed awareness among public health and regulatory agencies, pediatricians, a nd the public, although no epidemiologic data support this concern. Because of worldwide concern (although no cases have been reported in North Americ a), this review focuses on the potential impact of TSEs, particularly CJD a nd nvCJD, on the pediatric population.