Creutzfeldt-Jakob Disease in Austria: An Autopsy-Controlled Study

Abstract

Background: Definite diagnosis of prion diseases or transmissible spongiform encephalopathies (TSEs) requires neuropathology, usually at autopsy. Epidemiology of human TSEs has relied on definite as well as ‘probable’ cases in which neuropathological confirmation is lacking, usually because of low autopsy rates in most countries. Methods: In Austria, an active surveillance program for human prion diseases was established in 1996. Since then, more than 900 referrals were analyzed. Postmortem investigation of brain tissue is mandatory in every suspect case of TSE. Thus, epidemiological data on TSEs from Austria may serve as autopsy-controlled reference for countries with lower autopsy rates. Results: The total number of TSE cases in Austria since 1969 is 206. The average yearly mortality for the active surveillance period from 1996 to 30 June 2006 is 1.39 per million, with the highest rates for Vienna (2.37) compared with other provinces. Eighty-five percent of definite TSEs were classified as sporadic Creutzfeldt-Jakob disease (sCJD). We observed a significant linear increase in the mean age at death of 0.6 years per calendar year. Clinical diagnostic surveillance criteria had a sensitivity and specificity of 82.7 and 80.0% for probable CJD, respectively, and a positive predictive value of 80.5% for probable and 38.9% for ‘possible’ CJD. Alternative neuropathological diagnoses in suspect cases included Alzheimer’s disease with or without Lewy body pathology, vascular encephalopathy, metabolic encephalopathies and viral or limbic encephalitis. Conclusion: The steady increase in mortality rates, especially in old age groups, most likely reflects improved case ascertainment due to active surveillance causing higher awareness of the medical community. In comparison with other European countries, it is reassuring to note that the overall death rate of TSEs does not differ from the Austrian autopsy-controlled data, thus confirming the value of clinical surveillance criteria.

Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series

“…AD was the second most frequent aetiology, followed by DLB. Similar findings have been reported in previous studies [3,4,5,25,26,27]. Vascular encephalopathy was relatively frequent among non-prion RPD, as observed in other postmortem series [4,26].…”

Population-Based Epidemiological Study of Neurological Diseases in Taiwan: I. Creutzfeldt-Jakob Disease and Multiple Sclerosis

“…Compared with recent epidemiological data of CJD from Europe, Australia and Canada [15,20,23] , our data showed a similar age distribution in most cases occurring after 50 years of age. The annual incidence rate estimated in this study was lower than in Europe and Canada (0.63 vs. 0.93-1.67/million).…”

“…Although there is no autopsy rate available in our data, autopsy-controlled data revealed that clinical diagnostic surveillance criteria for probable CJD had a sensitivity and specificity of 82.7 and 80.0% [23] . In comparison with other European countries, it is reassuring to note that the overall death rate of CJD does not differ from the autopsy-controlled data, thus confirming the value of clinical surveillance criteria [23] . The NHIRD with SAD certificates could be a reliable source for evaluating the epidemiology of CJD in Taiwan.…”

An update on the epidemiology and key issues associated with the diagnosis and management of Creutzfeldt–Jakob disease cases in NSW

“…rates up to 2.37 cases per million population). 13 A similar relationship between surveillance intensity and disease incidence has also been reported in other countries. 14 …”