de Villemeur Thierry Billette
Pediatric Neurology Service, Pathologie du Développement, Hôpital Trousseau, Université Pierre et Marie Curie-Paris VI, Paris, France.
Handb Clin Neurol. 2013;112:1191-3. doi: 10.1016/B978-0-444-52910-7.00040-4.
Prion diseases are rare in children. Three types are known: kuru, variant Creutzfeldt-Jakob disease (CJD), and iatrogenic CJD. All three affect children and young adults, and are transmitted by infectious contamination. Kuru was the result of ritual funeral practices similar to cannibalism; variant CJD affects young people who have eaten meat from cows with mad cow disease (mostly in the UK); and iatrogenic CJD is secondary to graft of human tissues performed in the 1980s (dura mater, pituitary extracted growth hormone). The disease appears after 4-30 years of incubation. The initial symptomatology is frequently neurological (cerebellar ataxia, oculomotor disturbance, peripheral nerve pain, pyramidal syndrome) followed by dementia. There is no biological test available that can give a definite diagnosis of prion disease apart from neuropathology, although prion accumulation in vCJD can be demonstrated in pharyngeal tonsil by immunohistochemical techniques. This devastating disease results inevitably in death. No specific treatment is available.
朊病毒病在儿童中较为罕见。已知有三种类型：库鲁病、变异型克雅氏病（CJD）和医源性CJD。这三种类型均会影响儿童和年轻人，且通过传染性污染传播。库鲁病是类似同类相食的仪式性丧葬习俗导致的结果；变异型CJD影响那些食用了患有疯牛病的牛肉的年轻人（主要在英国）；医源性CJD继发于20世纪80年代进行的人体组织移植（硬脑膜、垂体提取的生长激素）。该病在4至30年的潜伏期后出现。最初的症状通常为神经方面的（小脑共济失调、动眼神经紊乱、周围神经疼痛、锥体综合征），随后发展为痴呆。除神经病理学外，尚无能够明确诊断朊病毒病的生物学检测方法，不过通过免疫组织化学技术可在咽扁桃体中检测到变异型CJD中的朊病毒积聚。这种毁灭性疾病最终必然导致死亡。目前尚无特效治疗方法。
