Vicente-Pascual, MikelRossi, MarcelloGámez, JosepLladó, AlbertValls, JosepGrau-Rivera, OriolÁvila Polo, RainieroLlorens, FrancZerr, IngaFerrer, IsidreNos, CarlosParchi, PieroSánchez-Valle, RaquelGelpí, Ellen2023-01-252023-01-252018-09-212328-9503http://hdl.handle.net/10668/13121We 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.enAttribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/research article30349865open access10.1002/acn3.632PMC6186932https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/acn3.632https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186932/pdf