ABSTRACT
In Spain, human transmissible spongiform encephalopathies (TSEs) have been undergoing continuous surveillance for over 25 years. In 1995, the system was launched as an EU Concerted Action, with EU surveillance network procedures being incorporated from 2002 onwards. The aim of this report was to describe performance and outcomes of this surveillance system across the period 1993–2018. Neurology and public health specialists from every region reported cases to a central hub at the Carlos III Health Institute, Madrid. In all, eight accidentally transmitted cases and five definite variant Creutzfeldt-Jakob disease (vCJD) patients were reported. All vCJD cases were diagnosed between 2005 and 2008. Two of these were family/dietary-related and spatially linked to a third. Yearly incidence of sporadic CJD per million was 1.25 across the period 1998–2018, and displayed a north-south gradient with the highest incidence in La Rioja, Navarre and the Basque Country. Genetic TSEs were observed to be clustered in the Basque Country, with a 4-fold incidence over the national rate. A total of 120 (5.6%) non-TSE sporadic, conformational, rapidly progressing neurodegenerative and vascular brain disorders were reported as suspect CJD. We conclude that TSEs in Spain displayed geographically uneven, stable medium incidences for the sporadic and genetic forms, a temporal and spatial family cluster for vCJD, and decreasing numbers for dura-mater-associated forms. The vCJD surveillance, framed within the EU network, might require continuing to cover all prion disorders. There is need for further strategic surveillance research focusing on case definition of rapid-course, conformational encephalopathies and surgical risk.
Statements
The Spanish CJD Study and Surveillance Group (Grupo para el Estudio y Vigilancia de las EETH del Consejo Interterritorial del Sistema Nacional de Salud) is composed of:
— Spanish National Register of Human Transmissible Spongiform Encephalopathies (Almazán J, Avellanal F, Calero M, De Pedro-Cuesta J, Martínez P, Ruiz M, Tello O, García López FJ.), National Epidemiology Centre and Unit of Spongiform Encephalopathies, National Microbiology Centre, Health Institute Carlos III, Madrid.
— Department of Pathology, University Hospital Fundación Alcorcón (Guerrero C, Rebolledo A), Alcorcón.
— CIEN Foundation, Foundation Research Centre of Neurological Diseases (Calero M, Rábano A).
— Regional epidemiologic and clinical coordinators in 2020: Andalusia: Heras JA, Varo A, Guillén J; Aragon: Alonso JP, Juanas FE, Ladrero O, López del Val J; Asturias: Huerta, I, Margolles M, Asensi A; Balearic Islands: Grau P, Nicolau A, Sureda B; Canary Islands: Izquierdo A, C. Muñoz, Pérez MC; Cantabria: Galán M, Martínez F, Polo JM; Castile-La Mancha: Cabeza C, Gutiérrez G, Humanes S; Castile and León: Goñi M, Marcos H; Catalonia: Domínguez A, Nos C; Valencian Region: Escudero J, Guiral S, Marín C; Extremadura: Casado I, Fuentes A, López M. Ramírez JM, Sánchez JF; Galicia: Losada I, Malvar A, Navarro C, Robles A; Madrid: Del Ser T, Frank A, Muñoz J, Pichiule M, Ramírez R; Murcia: Barranco MI, Castán Y, Contreras MA, García-Fulgueiras A, Pina R; Navarre: Barricarte A, Castilla J, Tuñón I; Basque Country: Arteagoitia JM, Zarranz JJ; La Rioja: Blanco A, Gil A, Martínez E, Marzo ME, Perucha M; Ceuta: Carrillo FJ, Rivas AI; Melilla: Castrillejo D, Duque G.
Acknowledgments
Authors would like to acknowledge the generous contribution made by multiple persons in the form of support for and collaboration with the Spanish CJD Study and Surveillance Group, and to the following in particular: notifying clinicians and pathologists and temporary members active at the registry (Alcalde E, Cuadrado JI, Mahíllo I, Martínez-Martín P, Plitt C, Ruiz Bremón A, Sevillano MD, Cuadrado Corrales N); colleagues at the National Epidemiology Centre (Tello O and Cano R); members of the Coordination Centre for Health Alerts and Emergencies of the Spanish Ministry of Health (Sierra MJ, Simón F), and Mrs. Lindsay T, Ward HJ, and Professors Will RG and Ironside J at the Edinburgh CJD Unit for advice and support. Finally, the authors would also like to thank Dr Ana Cabello for early perception of atCJD cases, and Michael Benedict for his English language revision of this paper.
Ethics
Review by Ethics Committee was not needed due to Public Health involvement.
Disclosure of potential conflicts of interest
All authors declare absence of conflict of interest.
Author contributions
Concept and design: Jesús de Pedro-Cuesta, Javier Almazán-Isla, Fernando J García López
Acquisition, analysis or interpretation of data: Javier Almazán-Isla, Fuencisla Avellanal, María Ruiz-Tovar, Miguel Calero, Jesús de Pedro-Cuesta
Drafting of the manuscript: Jesús de Pedro-Cuesta, Fernando J. García López, Laura Tejedor-Romero, Javier Almazán-Isla, Miguel Calero
Critical revision of the manuscript for important intellectual content: Jesús de Pedro-Cuesta, Javier Almazán-Isla, Laura Tejedor-Romero, María Ruiz-Tovar, Fuencisla Avellanal, Alberto Rábano, Miguel Calero, Fernando J. García López and all regional epidemiologic and clinical coordinators.
Statistical analysis: Javier Almazán-Isla
Obtained funding: Jesús de Pedro-Cuesta
Administrative, technical, or material support: Javier Almazán-Isla, Fuencisla Avellanal, María Ruiz-Tovar, Fernando J García López
Supervision: Jesús de Pedro-Cuesta