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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 | 2019-09-12T17:15:59Z |
| dc.date | 2019-09-12T17:15:59Z |
| dc.date | 2018-09 |
| dc.date | 2019-09-12T17:15:59Z |
| dc.identifier | 2328-9503 |
| dc.identifier | 689423 |
| dc.identifier | 30349865 |
| dc.identifier.uri | http://hdl.handle.net/2445/139916 |
| dc.description | 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 | 6 p. |
| dc.format | application/pdf |
| dc.language | eng |
| dc.publisher | American Neurological Association |
| dc.relation | Reproducció del document publicat a: https://doi.org/10.1002/acn3.632 |
| dc.relation | Annals of Clinical and Translational Neurology, 2018, vol. 5, num. 10, p. 1297-1302 |
| dc.relation | https://doi.org/10.1002/acn3.632 |
| dc.rights | cc-by-nc-nd (c) Vicente-Pascual, Mikel et al., 2018 |
| dc.rights | http://creativecommons.org/licenses/by-nc-nd/3.0/es |
| dc.rights | info:eu-repo/semantics/openAccess |
| dc.subject | Enzims proteolítics |
| dc.subject | Patologia |
| dc.subject | Esclerosi lateral amiotròfica |
| dc.subject | Proteolytic enzymes |
| dc.subject | Pathology |
| dc.subject | 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 |
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