ArticleAnnals of clinical and translational neurology2026
Variably Protease-Sensitive Prionopathy: Two New Cases With Motor Neuron-Dementia Syndrome.
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García-Amigot, Anika Simonovska-Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó and 1 more
Abstract readCase Reports
In one paragraphArticle in Annals of clinical and translational neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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What it found
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2 · The registryThe trial behind it
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4 · The recordCorrections and comments
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5 · Who and what moneyAuthors and funding
11 authors.
María Victoria ZelayaDepartment of Pathology, Hospital Universitario de Navarra (HUN), Pamplona, Spain.
Hasier ErañaCIC BioGUNE, Basque Research and Technology Alliance (BRTA), Prion Research Lab, Derio, Spain.ORCID 0000-0001-8776-4211 Javier Sánchez Ruiz de GordoaDepartment of Neurology, Hospital Universitario de Navarra (HUN), Pamplona, Spain.
Fermín García-AmigotDepartment of Medical Genetics, Hospital Universitario de Navarra (HUN), Pamplona, Spain.
Anika Simonovska-SerraDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, and Austrian Reference Center for Human Prion Diseases (OERPE), Vienna, Austria.
María Cristina CaballeroDepartment of Pathology, Hospital Universitario de Navarra (HUN), Pamplona, Spain.
Isidre FerrerBiomedical Network Research Centre of Neurodegenerative Diseases (CIBERNED), Hospitalet de Llobregat, Spain.
Ellen GelpiDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, and Austrian Reference Center for Human Prion Diseases (OERPE), Vienna, Austria.
Ivonne JericóDepartment of Neurology, Hospital Universitario de Navarra (HUN), Pamplona, Spain.
Joaquín CastillaCIC BioGUNE, Basque Research and Technology Alliance (BRTA), Prion Research Lab, Derio, Spain.
Funding
Agencia Estatal de Investigación, Ministerio de Ciencia e Innovación PID2021-122201OB-C21Agencia Estatal de Investigación, Ministerio de Ciencia e Innovación PID2024-160022OB-I00European Regional Development FundFederal Ministry Labour, Social Affairs, Health, Care and Consumer ProtectionHealth Department of Navarre Government 19-2021Severo Ochoa Excellence accreditation CEX2021-001136-S
6 · The paper itselfAbstract
We describe two patients with variably protease-sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD). Neuropathology revealed a spongiform encephalopathy with frontotemporal and pronounced thalamic involvement, associated with fine synaptic abnormal prion protein conformer (PrP
Indexed as
Frontotemporal DementiaMotor Neuron DiseasePrion DiseasesAgedAmyotrophic Lateral SclerosisFemaleHumansMaleMiddle AgedPrion ProteinsPrion Proteinsatypical amyotrophic lateral sclerosisCreutzfeldt‐Jakob diseasepyramidal syndromespinal cordthalamic dementia
Identifiers
PMID41456082
PMCPMC13071102
What OpenQuestion holds
Textmetadata
LicenceCC BY-NC-ND
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