Stehmann Christiane, Sarros Shannon, Senesi Matteo, Lewis Victoria, Simpson Marion, McLean Catriona, Masters Colin, Collins Steven
Australian National Creutzfeldt–Jakob Disease Registry, Department of Medicine (RMH) and The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
The Alfred Hospital, Department of Anatomical Pathology, 55 Commercial Rd, Melbourne Vic 3004 Australia
Commun Dis Intell (2018). 2019 Jul 16;43. doi: 10.33321/cdi.2019.43.32.
Nationwide surveillance of human prion diseases (also known as transmissible spongiform encephalopathies), the most common being Creutzfeldt–Jakob disease (CJD), is performed by the Australian National Creutzfeldt–Jakob Disease Registry (ANCJDR), based at the University of Melbourne. National surveillance encompasses the period since 1970, with prospective surveillance occurring from 1993 onwards. Over this prospective surveillance period considerable developments have occurred, especially in relation to pre-mortem diagnostics, the delineation of new disease subtypes and a heightened awareness of prion diseases in the health care setting. The surveillance practices of the ANCJDR have evolved and adapted accordingly. Since the ANCJDR began offering cerebrospinal fluid (CSF) 14-3-3 protein testing in Australia in September 1997, the annual number of referrals has steadily increased to a maximum of 508 in 2017. The number of CSF test referrals in 2017 represents a 20% increase compared to that of 2016. In 2017, there was an overall stabilisation of the annual incidence rate of confirmed prion disease in Australia at expected levels; 72 persons with suspected human prion disease were added to the national register, with 72% of all suspected CJD cases undergoing neuropathological examination. The majority of the 72 suspected cases added to the register are as of 31 December 2017 still classified as “incomplete” (47 cases), while four cases were excluded by either detailed clinical follow-up (1 case) or neuropathological examination (3 cases); 19 cases were classified as definite and two as probable prion disease. No cases of variant CJD (vCJD) were confirmed.
澳大利亚全国克雅氏病登记处（ANCJDR）设在墨尔本大学，负责对全国范围内的人类朊病毒病（也称为传染性海绵状脑病，最常见的是克雅氏病，即CJD）进行监测。全国监测涵盖自1970年以来的时间段，前瞻性监测从1993年开始。在这个前瞻性监测期间，出现了相当大的进展，特别是在生前诊断、新疾病亚型的划分以及医疗环境中对朊病毒病的认识提高方面。ANCJDR的监测做法也相应地不断演变和调整。自1997年9月ANCJDR在澳大利亚开始提供脑脊液（CSF）14-3-3蛋白检测以来，每年的转诊数量稳步增加，2017年达到最多508例。2017年脑脊液检测转诊数量比2016年增加了20%。2017年，澳大利亚确诊朊病毒病的年发病率总体稳定在预期水平；72例疑似人类朊病毒病患者被纳入国家登记册，所有疑似CJD病例中有72%接受了神经病理学检查。截至2017年12月31日，新增登记册的72例疑似病例中，大多数仍被归类为“不完整”（47例），而4例通过详细的临床随访（1例）或神经病理学检查（3例）被排除；19例被归类为确诊，2例被归类为可能的朊病毒病。未确诊变异型克雅氏病（vCJD）病例。
