Variably protease-sensitive prionopathy presenting within ALS/FTD spectrum

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dc.contributor.authorVicente-Pascual, Mikel
dc.contributor.authorRossi, Marcello
dc.contributor.authorGámez, Josep
dc.contributor.authorLladó, Albert
dc.contributor.authorValls, Josep
dc.contributor.authorGrau-Rivera, Oriol
dc.contributor.authorÁvila Polo, Rainiero
dc.contributor.authorLlorens, Franc
dc.contributor.authorZerr, Inga
dc.contributor.authorFerrer, Isidre
dc.contributor.authorNos, Carlos
dc.contributor.authorParchi, Piero
dc.contributor.authorSánchez-Valle, Raquel
dc.contributor.authorGelpí, Ellen
dc.date.accessioned2021-03-17T13:41:10Z
dc.date.available2021-03-17T13:41:10Z
dc.date.issued2018
dc.description.abstractWe report clinico‐pathological features of a 65‐year‐old woman and a 56‐year‐old man with a 5‐year clinical history who had clinical and neuropathological characteristics of upper and lower motor neuron disease consistent with amyotrophic lateral sclerosis, and a frontotemporal atrophy pattern in case 2 without TDP‐43 pathology. Instead, spongiform change and pathological prion protein deposits were observed in several brain regions. No prion protein gene mutations were found. Western blot analysis showed a five‐band profile compatible with variably protease‐sensitive prionopathy. We conclude that this disease can display prolonged disease duration and clinico‐pathological features within the ALS/FTLD spectrum.ca_ES
dc.description.sponsorshipThis work was funded by Department of Public Health Generalitat de Catalunya grant ; Spanish “Ministerio de Economía y Competitividad, Subprograma Técnicos de Apoyo 2014” grant ; Spanish Fondo de Investigaciones Sanitarias grant FIS PI16‐01673‐FEDER; Spanish Ministry of Health ‐ Instituto Carlos III grant Miguel Servet ‐ CP16/00041; Fundació La Marató de TV3 grant 20141610.ca_ES
dc.identifier.doihttps://doi.org/10.1002/acn3.632
dc.identifier.issn2328-9503
dc.identifier.urihttp://hdl.handle.net/10459.1/70778
dc.language.isoengca_ES
dc.publisherAmerican Neurological Associationca_ES
dc.relation.isformatofReproducció del document publicat a https://doi.org/10.1002/acn3.632ca_ES
dc.relation.ispartofAnnals of Clinical and Translational Neurology, 2018, vol. 5, núm. 10, p. 1297-1302ca_ES
dc.rightscc-by-nc-nd (c) Vicente-Pascual et al., 2018ca_ES
dc.rights.accessRightsinfo:eu-repo/semantics/openAccessca_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subject.otherEnzims proteolíticsca_ES
dc.titleVariably protease-sensitive prionopathy presenting within ALS/FTD spectrumca_ES
dc.typeinfo:eu-repo/semantics/articleca_ES
dc.type.versioninfo:eu-repo/semantics/publishedVersionca_ES
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