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http://hdl.handle.net/2445/139916
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DC Field | Value | Language |
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dc.contributor.author | Vicente-Pascual, Mikel | - |
dc.contributor.author | Rossi, Marcello | - |
dc.contributor.author | Gámez, Josep | - |
dc.contributor.author | Lladó Plarrumaní, Albert | - |
dc.contributor.author | Valls Solé, Josep | - |
dc.contributor.author | Grau-Rivera, Oriol | - |
dc.contributor.author | Ávila Polo, Rainiero | - |
dc.contributor.author | Llorens Torres, Franc | - |
dc.contributor.author | Zerr, Inga | - |
dc.contributor.author | Ferrer, Isidro (Ferrer Abizanda) | - |
dc.contributor.author | Nos, Carlos | - |
dc.contributor.author | Parchi, Piero | - |
dc.contributor.author | Sánchez del Valle Díaz, Raquel | - |
dc.contributor.author | Gelpi, Ellen | - |
dc.date.accessioned | 2019-09-12T17:15:59Z | - |
dc.date.available | 2019-09-12T17:15:59Z | - |
dc.date.issued | 2018-09 | - |
dc.identifier.issn | 2328-9503 | - |
dc.identifier.uri | http://hdl.handle.net/2445/139916 | - |
dc.description.abstract | We report clinico-pathological features of a 65-year-old woman and a 56-yearold 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. | - |
dc.format.extent | 6 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | American Neurological Association | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1002/acn3.632 | - |
dc.relation.ispartof | Annals of Clinical and Translational Neurology, 2018, vol. 5, num. 10, p. 1297-1302 | - |
dc.relation.uri | https://doi.org/10.1002/acn3.632 | - |
dc.rights | cc-by-nc-nd (c) Vicente-Pascual, Mikel et al., 2018 | - |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/es | - |
dc.source | Articles publicats en revistes (Patologia i Terapèutica Experimental) | - |
dc.subject.classification | Enzims proteolítics | - |
dc.subject.classification | Patologia | - |
dc.subject.classification | Esclerosi lateral amiotròfica | - |
dc.subject.other | Proteolytic enzymes | - |
dc.subject.other | Pathology | - |
dc.subject.other | Amyotrophic lateral sclerosis | - |
dc.title | Variably protease-sensitive prionopathy presenting within ALS/FTD spectrum | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 689423 | - |
dc.date.updated | 2019-09-12T17:15:59Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 30349865 | - |
Appears in Collections: | Articles publicats en revistes (Medicina) Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Patologia i Terapèutica Experimental) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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689423.pdf | 523.3 kB | Adobe PDF | View/Open |
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