Abstract
An extraordinary incidence of genetic Creutzfeldt-Jakob disease (gCJD) appearing in clusters in the Slovak Republic was described in the 1990's. The aim of the study was to analyse data of CJD cases obtained from surveillance in Eastern Slovakia (ES) (2004-2016), the region outside the described geographical clusters. The database set in the project was the source for epidemiological and clinical analysis of CJD cases. The incidence of CJD in ES (2004-2016) was 1.7/million person-years (95% CI 1-2.4); the incidence increase in the last five years (2012-2016) was comparable to the whole country. Twenty seven of 29 reported CJD cases were available for analysis (mean age 59 years, F/M 15/12). The proportion of gCJD (E200K mutation) cases remained dominant (78%), with 9 familiar cases originating in 4 families. Analysis of the clinical features revealed shorter duration of the symptomatic phase in sporadic CJD (sCJD) (3.4 months) versus gCJD (5.15 months). Cognitive/behavioural changes, insomnia, and sensory disturbance were more pronounced in the early symptoms of gCJD. Periodic EEG discharges were more frequent in sCJD (83%) than gCJD (56%), all 19 available MR findings were CJD specific and localisation of abnormalities varied ...Continue Reading
References
Feb 1, 1979·Annals of Neurology·C L MastersD M Asher
Nov 1, 1979·Annals of Neurology·P BrownD C Gajdusek
Nov 4, 1992·JAMA : the Journal of the American Medical Association·J M BertoniD C Gajdusek
Jan 1, 1986·Journal of Clinical Neurophysiology : Official Publication of the American Electroencephalographic Society·S R LevyR R Young
May 8, 2003·Clinics in Laboratory Medicine·Maura N Ricketts, Paul Brown
Aug 7, 2003·BMC Public Health·Ivan Laprevotte, Alain Hénaut
Aug 12, 2004·Neurology·Y ShigaY Itoyama
Sep 28, 2004·Annals of Neurology·Bernhard J SteinhoffHans A Kretzschmar
May 11, 2005·Neurology·A LadoganaI Zerr
Sep 28, 2005·Human Genetics·Gábor G KovácsUNKNOWN EUROCJD
Jun 26, 2008·Journal of Neurology·N KandiahN C Tan
Jul 19, 2008·AJNR. American Journal of Neuroradiology·R K FulbrightI Prohovnik
Jul 2, 2010·Acta Neuropathologica·Gabor G KovacsArmand Perret-Liaudet
Aug 13, 2011·Journal of Neurology·Shmuel A AppelIlan Blatt
Oct 27, 2011·Central European Journal of Public Health·Dana SlivarichováLadislava Wsólová
Jul 30, 2014·European Journal of Epidemiology·Eva MitrováIvana Tomečková