# metadata.dc.description.abstract[].value
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.

# metadata.dc.relation.ispartof[].value
Annals of Clinical and Translational Neurology, 2018, vol. 5, num. 10, p. 1297-1302

# metadata.dc.rights[].value
cc-by-nc-nd (c) Vicente-Pascual, Mikel et al., 2018

# metadata.dc.source[].value
Articles publicats en revistes (Patologia i Terapèutica Experimental)