Publications

Assar H, Topakian R, Weis S, Rahimi J, Trenkler J, Höftberger R, Aboulenein Djamshidian F, Ströbel T, Budka H, Yull H, Head MW, Ironside JW, Kovacs GG. A case of variably protease sensitive prionopathy treated with doxycyclin. JNNP 2015 86(7): 816-8. 

Budka H, Will RG. The end of the BSE saga: do we still need surveillance for human prion diseases? Swiss Medical Weekly 2015; 145: w14212.

Head MW, Yull HM, Törö K, Keller E, Rozsa C, Ironside JW, Kovacs GG. Pathological and biochemical investigation of a woman diagnosed with genetic Creutzfeldt-Jakob disease shortly after parturition. Neuropathol Appl Neurobiol. 2015; 41(5): 676-80

Sanchez- Juan P, Bishop MT, Kovacs GG, Calero M, Aulchenko YS, Ladogana A, Boyd A, Lewis V, Ponto C, Calero O, Poleggi A, Carracedo Á, van der Lee SJ, Ströbel T, Rivadeneira F, Hofman A, Haïk S, Combarros O, Berciano J, Uitterlinden AG, Collins SJ, Budka H, Brandel JP, Laplanche JL, Pocchiari M, Zerr I, Knight RS, Will RG, van Duijn CM. A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk. PLoS One 2015; 10(4): e0123654. 

Schmitz M, Ebert E, Stoeck K, Karch A, Collins S, Calero M, Sklaviadis T, Laplanche JL, Golanska E, Baldeiras I, Satoh K, Sanchez-Valle R, Ladogana A, Skinningsrud A, Hammarin AL, Mitrova E, Llorens F, Kim YS, Green A, Zerr I. Validation of 14-3-3 Protein as a Marker in Sporadic Creutzfeldt-Jakob Disease Diagnostic. Mol Neurobiol. 2015 May 7.

Matthew T. Bishop, Abigail B. Diack, Diane L. Ritchie, James W. Ironside, Robert G. Will, Jean C. Manson. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt–Jakob disease Brain 2013: 136; 1139–1145 doi:10.1093/brain/awt032

Mabel Cruz, Ignacio Mahillo-Fernandez, Alberto Rábano, Åke Siden, Miguel Calero, Henning Laursen, Kåre Mølbak, Javier Almazán, Jesus de Pedro-Cuesta on behalf of the EUROSURGYCJD Research Group. Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance Emerging Themes in Epidemiology 2013,10:5 http://www.ete-online.com/content/10/1/5

Sanchez-Juan P, Bishop MT, Aulchenko YS, Brandel JP, Rivadeneira F, Struchalin M, Lambert JC, Amouyel P, Combarros O, Sainz J, Carracedo A, Uitterlinden AG, Hofman A, Zerr I, Kretzschmar HA, Laplanche JL, Knight RSG , Will RG, van Duijn CM. Genome-wide study links phosphatidylinositol MTMR7 gene to variant Creutzfeldt-Jakob risk. Neurobiol Aging. 2012 Jul;33(7):1487.e21-8. doi: 10.1016/j.neurobiolaging.2011.10.011. Epub 2011 Dec 2.

Alcalde-Cabero E, Almazán-Isla J, Brandel JP, Breithaupt M, Catarino J, Collins S, Haybäck J, Höftberger R, Kahana E, Kovacs GG, Ladogana A, Mitrova E, Molesworth A, Nakamura Y, Pocchiari M, Popovic M, Ruiz-Tovar M, Taratuto AL, van Duijn C, Yamada M, Will RG, Zerr I, de Pedro Cuesta J. Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010. Euro Surveill. 2012;17(15):pii=20144. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=20144

Jesús de Pedro Cuesta, María Ruiz Tovar, Hester Ward, Miguel Calero, Andrew Smith, Concepción Alonso Verduras, Maurizio Pocchiari, Marc L. Turner, Frode Forland, Daniel Palm, Robert G. Will. Sensitivity to Biases of Case-Control Studies on Medical Procedures, Particularly Surgery and BloodTransfusion, and Risk of Creutzfeldt-Jakob Disease Neuroepidemiology 2012;39:1–18 DOI: 10.1159/000339318

The EUROCJD Group Response to the Article by Adanipar et al.: The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey Dement Geriatr Cogn Disord Extra 2011;1:429–432

Abigail B. Diack, Diane Ritchie, Matthew Bishop, Victoria Pinion, Jean-Philippe Brandel, Stephane Haik, Fabrizio Tagliavini, Cornelia Van Duijn, Ermias D. Belay, Pierluigi Gambetti, Lawrence B. Schonberger, Pedro Piccardo, Robert G. Will and Jean C. Manson. Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries Emerging Infectious Diseases www.cdc.gov/eid Vol. 18, No. 10, October 2012

Sanchez-Juan P, Bishop MT, Croes E, Knight RSG, Will RG, van Duijn CM, Manson JC. A polymorphism in the regulatory region of PRNP in associated with is associated with increased risk of sporadic Creutzfeldt-Jakob disease. BMC Medical Genetics 2011; 12:73

Kovacs GG, Sanchez-Juan P, Ströbel T, Schuur M, Poleggi A, Nocentini S, Giannattasio C, Belay G, Bishop M, Capellari S, Parchi P, Gelpi E, Gal A, Bakos A, Molnar MJ, Heinemann U, Zerr I, Knight RSG, Mitrova E, van Duijn C, Budka H.  Cathepsin D (C224T) polymorphism in sporadic and genetic Creutzfeldt-Jakob disease. Alzheimer Disease & Associated Disorders 2010 24(1):104-107

Brandel J-P, Heath CA, Head MW, Levavasseur E, Knight R, Laplanche J-L, Langeveld JPM, Ironside JW, Hauw J-J, Mackenzie J, Alpérovitch A, Will RG, Haïk S. Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain Annals of Neurology 2009 65 (3) 249-256

Ladogana, A., Sanchez-Juan, P., Mitrová, E., Green, A., Cuadrado-Corrales, N., Sánchez-Valle, R., Koscova, S., Aguzzi, A., Sklaviadis, Th., Kulczycki,J., Gawinecka,  J., Saiz, A., Calero, M., van Duijn, CM., Pocchiari,  M., Knight, R., Zerr, I. Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies J Neurol 2009, 256:1620-1628

Kovacs GG, Horvath S, Ströbel T, Puskas M, Bakos A, Summers DM, Will RG, Budka H: Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiat 2009 80:1410-1411

Mara Popović  Humane prenosljive spongiformne encefalopatije in njihova epidemiologija. Evropski veterinarski teden 2009 v Sloveniji  65-70

B. Meissner, K. Kallenberg, P. Sanchez-Juan, D. Collie, D. M. Summers, S. Almonti, S. J. Collins, P. Smith, P. Cras, G. H. Jansen, J. P. Brandel, M. B. Coulthart, H. Roberts, B. Van Everbroeck, D. Galanaud, V. Mellina, R. G. Will, I. Zerr.  MRI lesion profiles in sporadic Creutzfeldt–Jakob disease.  Neurology 2009; 72:1994-2001

Zerr, I., Kallenberg, K., Summers, DM., Romero, C., Taratuto, A., Heinemann, U.,  Breithaupt, M., Varges, D., Meissner, B., Ladogana, A., Schuur, M., Haik, S., Collins, SJ., Jansen, GH., Stokin, GB., Pimentel, J., Hewer, E., Collie, D., Smith, P., Roberts, H., Brandel, JP., van Duijn, C., Pocchiari, M., Begue, C., Cras, P., Will, RG., Sanchez-Juan, P.  Updated clinical diagnostic criteria for sporadic CJD. Brain, 2009: 132(Pt 10):2659-68

Meissner, B., Kallenberg, K., Ramljak, S., Krasnianski, A., Heinemann, U., Krebs, B., Budka, H., Barcic, A., Kretzschmar, H.A., Knauth, M., Schulz-Schaeffer, W.J., Zerr I. MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease. J Neurology, 2009 256(3):355-63

Mitrová,E.: Dynamika výskytu CJch na Slovensku v rokoch 1975-2008.   Epidemiol. Mikrobiol. Imunol., 2009, 58; 4:172-177

Pocchiari, M., Poleggi, A., Principe, S., Graziano, S. & Cardone, F. (2009). Genomic and post-genomic analyses of human prion diseases. Genome Med 1, 63

Parchi, P., Notari, S., Weber, P., Schimmel, H., Budka, H., Ferrer, I., Haik, S., Hauw, J. J., Head, M. W., Ironside, J. W., Limido, L., Rodriguez, A., Strobel, T., Tagliavini, F. & Kretzschmar, H. A. (2009). Inter-laboratory assessment of PrPSc typing in creutzfeldt-jakob disease: a Western blot study within the NeuroPrion Consortium. Brain Pathol 19, 384-391

Pocchiari, M. (2009). Prevalence of variant CJD in the UK. BMJ 338, b435. 

Sikorska, B., Liberski, P. P., Sobow, T., Budka, H. & Ironside, J. W. (2009). Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker disease. Neuropathol Appl Neurobiol 35, 46-59 

Anja Colja Venturini, Maja Bresjanac, Tanja Vranac1, Simon Koren, Mojca Narat, Mara Popović and Vladka Жurin Šerbec. Anti-idiotypic antibodies: a new approach in prion research. BMC Immunology 2009, 10:16 doi:10.1186/1471-2172-10-16  

Georgsson G, Ólafsson E, Guðmundsson G.  Scrapie of sheep and Creutzfeldt-Jakob disease in Iceland. The Icelandic Medical Journal 2008 94: 541-548

Papacostas S, Malikides A, Petsa M, Kyriakides T.  Ten-year mortality from Creutzfeldt-Jakob disease in Cyprus.  Eastern Mediterranean Health Journal 14 (3) 2008 715-719

M. Pocchiari, A. Ladogana, S. Graziano and M. Puopolo. Creutzfeldt-Jakob disease: hopes for therapy. Editorial European Journal of Neurology 2008, 15: 435–436

Matthew T. Bishop1, Diane L. Ritchie, Robert G. Will, James W. Ironside1, Mark W. Head, Val Thomson, Moira Bruce, Jean C. Manson. No Major Change in vCJD Agent Strain after Secondary Transmission via Blood Transfusion PLoS ONE August 2008, 3(8): 2878

Mitrová,E.: Súčasná situácia v diagnostike CJch v SR: vyskytol sa aj nvCJch? Neurológia, 2008; 2:86-89

Mitrová,E.: Nekonvenčný svet prionových chorôb: malá retrospektíva a perspektívy, alebo 2+1  Nobelové ceny.  (Unconventional world of prion diseases: some retrospectives and perspectives or 2 + 1 Nobel prizes.) Edit. Herba, 2008:1-157

Mahillo-Fernandez I, de Pedro Cuesta J, Bleda MJ, Cruz M, Molbak K, Laursen H, Falkenhorst G, Martinez-Martin P, Siden A.  Surgery and Risk of Sporadic Creutzfeldt - Jakob disease in Denmark and Sweden: Registry-Based Case-Control Studies Neuroepidemiology 2008 31:229-240

Miele G, Seegar H, Marino D, Eberhard R, Heikenwalder M, Stoeck K, Basagni M, Knight R, Green A, Chianini F, Wuthrich R, Hock C, Zerr I, Aguzzi A. Urinary alpha-1-antichymotrypsin: A biomarker of prion infection. PLoS 2008;3(12):1-9

Mitrová,E., Janáková, A.: Psychiatrické príznaky pri CJch z pohľadu diferenciálnej diagnostiky.  Slovenský lekár, 2008, 1-2:10-14.

Green A, Sanchez-Juan P, Ladogana A, Cuadrado-Corrales N, Sanchez-Valle R, Mitrova E, Stoeck K, Sklaviadis T, Kulczycki J, Heinemann U, Hess K, Slivarichova D, Saiz A, Calero M, Mellina V, Knight R, van Duijn C M, Zerr I.  CSF analysis in patients with sporadic CJD and other transmissible spongiform encephalopathies.  European Journal of Neurology 2007;  14:  121-124

Sanchez-Juan P, Sanchez-Valle R, Green A, Ladogana A, Cuadrado-Corrales N, Mitrova E, Stoeck K, Sklaviadis T, Kulczycki J, Hess K, Krasnianski A, Equestre M, Slivarichova D, Saiz A, Calero M, Pocchiari M, Knight R, van Duijn C M, Zerr I.  Influence on timing on CSF tests value for Creutzfeldt-Jakob disease diagnosis.  J Neurol 2007;  254:901-906

Sanchez-Juan P, Houben MPWA, Hoff JI, Jansen C, Sie MPS, van Rujn MJE, Ironside JW, Will RG, van Duijn CM, Rozemuller A. The first case of variant Creutzfeldt-Jakob disease in the Netherlands. J Neurol 2007; 254: 958-960

The European surveillance system for Creutzfeldt-Jakob disease, Prof RG Will, Dr RSG Knight, CJD Support Network Newsletter, March 2007, Issue 16

P Sanchez-Juan, SN Cousens, RG Will, CM van Duijn. Source of Variant Creutzfeldt-Jakob Disease outside the United Kingdom. Emerging Infectious Diseases 2007 13:8 1166–1169. 

Sanchez-Juan P, Bishop MT, Green A, Giannattasio C, Arias-Vasquez A, Poleggi A, Knight RSG and van Duijn CM. No evidence for association between tau gene haplotypic variants and susceptibility to Creutzfeldt-Jakob disease. BMC Medical Genetics 2007, 8:77

McHugh JC, Bradley D, Hutchinson M, Brett F, Heffernan J, Howley R, Farrell M, Tubridy N. Variant Creutzfeldt-Jakob disease: first two indigenous cases in Republic of Ireland. Case report and perspective. Eur J Neurol. 2007 Apr;14(4):467-9.

R. Matěj, R. Rusina, F. Koukolík. 5 let činnosti Národní referenční laboratoře lidských prionových onemocnění při Oddělení patologie a molekulární medicíny FTNsP: naše zkušenosti a 0.přehled literatury. Cesk Slov Neurol N 2007; 70/103(6): 637-642

Georgsson G, Tryggvason T, Jonasdottir AD, Gudmundsson S, Thorgeirsdottir S. Polymorphism of PRNP codons in the normal Icelandic population. Acta Neurol Scand 2006: 113: 419–425

Saetta AA, Michalopoulos NV, Malamis G, Papanastasiou PI, Mazmanian N, Karlou M, Kouzoupis A, Korkolopoulou P, Patsouris E. Analysis of PRNP gene codon 129 polymorphism in the Greek population. Eur J Epidemiol. 2006;21(3):211-5

Collins SJ, Sanchez-Juan P, Masters CL, Klug GM, van Duijn C, Poleggi A, Pocchiari M, Almonti S, Cuadrado-Corrales N, de Pedro Cuesta J, Budka H, Gelpi E, Glatzel M, Tolnay M, Hewer E, Zerr I, Heinemenn U, Kretzschmar HA, Jansen GH, Olsen E, Mitrova E, Alperovitch A, Brandel J-P, Mackenzie J, Murray K, Will RG. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain 2006 September 2006; 129: 2278-2287.

Boesenberg-Grosse C, Schulz-Schaeffer W J, Bodemer M, Ciesielczyk B, Meissner B, Krasnianski A, Bartl M, Heinemann U, Varges D, Eigenbrod S, Kretzschmar H A, Green A, Zerr I. Brain-derived proteins in the CSF, do they correlate with brain pathology in CJD? BMC Neurology 2006 September 2006; 1471-2377-6-35.

Sanchez-Juan P, Green A, Ladogana A, Cuadrado-Corrales N, Sanchez-Valle R, Mitrova E, Stoeck K, Sklaviadis T, Kulczycki J, Hess K, Bodemer M, Slivarichova D, Saiz A, Calero M, Ingrosso L, Knight R, Janssens A C J W, van Duijn C M, Zerr I. CSF tests in the differential diagnosis of Creutzfeldt - Jakob disease. Neurology 2006; 67; 637-643. 

Kovacs GG, Puopolo M, Ladogana A, Pocchiari M, Budka H, van Duijn C, Collins SJ, Boyd A, Giulivi A, Coulthart M, Delasnerie-Laupretre N, Brandel JP, Zerr I, Kretzschmar H, de Pedro Cuesta J, Calero-Lara M, Glatzel M, Aguzzi A, Bishop M Knight R, Belay G, Will R, Mitrova E. Genetic Prion Disease: the EUROCJD experience. Hum Genet (2005); 118:166-174.

Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, Klug GM, Sutcliffe T, Giulivi A, Alperovitch A, Delasnerie-Laupretre N, Brandel J-P, Poser S, Kretzschmar H, Rietveld I, Mitrova E, de Pedro Cuesta J, Martinez-Martin P, Glatzel M, Aguzzi A, Knight R, Ward H, Pocchiari M, van Duijn CM, Will RG, Zerr I. Mortality from Creutzfedlt-Jakob disease and related disorders in Europe, Australia, and Canada.  Neurology 2005; 64:1586-1591.

Pocchiari M, Puopolo M, Croes EA, Gelpi E, Collins S, Lewis V, Sutcliffe T, Giulivi A, Delasnerie-Laupretre N, Brandel J-P, Alperovitch A, Zerr I, Poser S, Kretzschmar H, Ladogana A, Rietvald I, Mitrova E, Martinez-Martin P, Pedro-Cuesta J, Glatzel M, Aguzzi A, Cooper S, Mackenzie J, van Duijn CM, Will RG. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004 127 2348-2359.

Kovács GG, Kalev O, Budka H. (2004) Contribution of neuropathology to the understanding of human prion disease. Folia Neuropathol Suppl. A. 35-42.

Kovács GG, Lindeck-Pozza E, Chimelli L, Araújo AQC, Gabbai AA, Ströbel T, Glatzel M, Aguzzi A, Budka H. (2004) Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle. Ann Neurol 55: 121-125.

Brandel JP, Preece M, Brown P et al. Distribution of codon 129 genotype in human growth hormone-related CJD patients in France and the UK. Lancet 2003; 362:128-30 
Nurmi MH, Bishop M, Strain L, Brett F, McGuigan C, Hutchison M et al. The normal population distribution of PRNP codon 129 polymorphism. Acta Neurol Scand 2003; 108:374-378

Jarius C.*, Kovács GG.*, Belay G., Hainfellner JA., Mitrova E., Budka H. (2003) Distinctive cerebellar immunoreactivity for the prion protein in familial (E200K) Creutzfeldt-Jakob disease. Acta Neuropathol 105(5):449-454 

Mitrova E, Belay G. Creutzfeldt-Jakob disease with E200K Mutation in Slovakia: Characterization and development. Acta Virologica 2002 46:31-39

Ward H, Everington D, Croes EA, Alperovitch A, Delasnerie-Laupretre N, Zerr I, Poser S, van Duijn CM. Sporadic Creutzfeldt-Jakob Disease and surgery: A case-control study using community controls. Neurology 2002; 59:543-548

Alperovitch A, Will RG. Predicting the size of the vCJD epidemic in France. C R Biologies 2002; 325:33-36

van Everbroeck B, Green AJE, Vanmechelen E, Vanderstichele H, Pals P, Sanchez-Guerra M, Cuadrado Corrales N, Martin J-J, Cras P. Phosphorylated tau in cerebrospinal fluid as a marker for Creutzfeldt-Jakob disease. JNNP 2002;73:79-82

The EUROCJD Group.  Genetic epidemiology of Creutzfeldt-Jakob disease in Europe.  Rev Neurol (Paris) 2001; 157: 633-637.  

Zerr I, Brandel J-P, Masullo C, Wientjens D, De Silva R, Zeidler M et al. European surveillance on Creutzfeldt-Jakob disease:  a case-control study for medical risk factors. J Clin Epid 2000;53:747-54.

Zerr I, Pocchiari M, Collins S, Brandel J-P, de Pedro Cuesta J, Knight RS et al. Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease.  Neurology 2000;55(6):811-5.

Alperovitch A, Zerr I, Pocchiari M, Mitrova E, de Pedro Cuesta J, Hegyi I et al. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 1999;353:1673-4. 

Will RG, Alperovitch A, Poser S, Pocchiari M, Hofman A, Mitrova E et al. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. Ann Neurol 1998;43:763-7. 

van Duijn CM, Delasnerie-Laupretre N, Masullo C, Zerr I, De Silva R, Wientjens DPWM et al. Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95. Lancet 1998;351:1081-5. 

Will RG. Update on surveillance of Creutzfeldt-Jakob disease in Europe. In Office International des Epizooties, ed. Epidemiological studies and research on transmissible spongiform encephalopathies, pp 10-12. Paris: Office International des Epizooties, 1998. 

Wientjens DPWM, Delasnerie-Laupretre N, Hofman A, Poser S, Pocchiari M, Will RG. Incidence of Creutzfeldt-Jakob disease in Europe. Neurology 1996;46:A290.

Wientjens DPWM, Davanipour Z, Hofman A, Kondo K, Matthews WB, Will RG et al. Risk factors for Creutzfeldt-Jakob disease:  a reanalysis of case-control studies. Neurology 1996;46:1287-91.

Will RG. Incidence of Creutzfeldt-Jakob disease in the European Community. In Gibbs CJJr, ed. Bovine Spongiform Encephalopathy:  The BSE Dilemma, pp 364-74. NY: Springer-Verlag, 1996.

Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921-5. 

Wientjens DPWM, Delasnerie-Laupretre N, Hofman A, Poser S, Pocchiari M, Will RG. Incidence of Creutzfeldt-Jakob disease in Europe. Neurology 1996;46:A290.

Wientjens DPWM, Davanipour Z, Hofman A, Kondo K, Matthews WB, Will RG et al. Risk factors for Creutzfeldt-Jakob disease:  a reanalysis of case-control studies. Neurology 1996;46:1287-91.

Will RG. Incidence of Creutzfeldt-Jakob disease in the European Community. In Gibbs CJJr, ed. Bovine Spongiform Encephalopathy:  The BSE Dilemma, pp 364-74. NY: Springer-Verlag, 1996.

Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921-5. 

Delasnerie-Laupretre N, Poser S, Pocchiari M, Wientjens DPWM, Will RG. Creutzfeldt-Jakob disease in Europe. Lancet 1995;346:898. 

Alperovitch A, Brown P, Weber T, Pocchiari M, Hofman A, Will RG. Incidence of Creutzfeldt-Jakob disease in Europe 1993. Lancet 1994;343:918