Mahat Sanjay, Asuncion Ria Monica D.
Nepal Cancer Hospital
University of Santo Tomas, Medicine and Surgery
Kuru is the prototype human prion disease first reported in publications by Dr. D. Carleton Gajdusek and Vincent Zigas in 1957 in the Fore tribes of Papua New Guinea. The word “kuru” means to tremble due to fever or cold. It is a non-inflammatory neurodegenerative disease and is a form of transmissible spongiform encephalopathy transmitted through the act of cannibalism. The symptoms of kuru progress rapidly with cerebellar and extrapyramidal signs and symptoms, with death occurring within one to two years of onset of symptoms. The disease is now extinct, but its discovery in the 20th century was pivotal for understanding the other prion diseases. Other prion diseases include Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), variant CJD (vCJD), and Gerstmann-Straussler-Scheinker (GSS) disease.
库鲁病是人类朊病毒疾病的原型,1957年由D. 卡尔顿·加德塞克博士和文森特·齐加斯首次在出版物中报道,该病存在于巴布亚新几内亚的福尔部落。“库鲁”一词意为因发烧或寒冷而颤抖。它是一种非炎症性神经退行性疾病,是通过食人行为传播的可传播性海绵状脑病的一种形式。库鲁病的症状发展迅速,伴有小脑和锥体外系体征及症状,症状出现后一到两年内会死亡。这种疾病现已绝迹,但它在20世纪被发现对于理解其他朊病毒疾病至关重要。其他朊病毒疾病包括克雅氏病(CJD)、致死性家族性失眠症(FFI)、变异型克雅氏病(vCJD)和格斯特曼-施特劳斯勒-舍恩克综合征(GSS)病。