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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\u2010band profile compatible with variably protease\u2010sensitive prionopathy. We conclude that this disease can display prolonged disease duration and clinico\u2010pathological features within the <jats:styled-content style=\"fixed-case\">ALS<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content> spectrum.<\/jats:p>","DOI":"10.1002\/acn3.632","type":"journal-article","created":{"date-parts":[[2018,9,22]],"date-time":"2018-09-22T12:41:20Z","timestamp":1537620080000},"page":"1297-1302","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Variably protease\u2010sensitive prionopathy presenting within ALS\/FTD spectrum"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5445-7853","authenticated-orcid":false,"given":"Mikel","family":"Vicente\u2010Pascual","sequence":"first","affiliation":[{"name":"Department of Neurology Hospital Cl\u00ednic de Barcelona Barcelona Spain"},{"name":"Neurological Tissue Bank of the 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