Association of PSP phenotypes with survival: A brain-bank study
| Autor: | Esteban Muñoz, Oriol Grau, Nuria Caballol, Alexandra Pérez-Soriano, Celia Painous, Ellen Gelpi, Mar Guasp, Francesc Valldeoriola, Alicia Garrido, Ana Cámara, Guenter H. Höglinger, Gesine Respondek, Almudena Sánchez-Gómez, María José Martí, Yaroslau Compta, Laura Molina-Porcel |
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| Rok vydání: | 2021 |
| Předmět: |
Male
0301 basic medicine Oncology classification [Supranuclear Palsy Progressive] physiopathology [Supranuclear Palsy Progressive] Richardson's syndrome Survival PSP-PGF Disease diagnosis [Supranuclear Palsy Progressive] Cohort Studies 0302 clinical medicine diagnosis [Parkinsonian Disorders] Corticobasal degeneration classification [Parkinsonian Disorders] PSP Middle Aged Prognosis Phenotype mortality [Parkinsonian Disorders] Neurology physiopathology [Parkinsonian Disorders] Practice Guidelines as Topic Cohort Female Supranuclear Palsy Progressive PSP-P medicine.medical_specialty Tissue Banks mortality [Supranuclear Palsy Progressive] Sensitivity and Specificity 03 medical and health sciences Atrophy Parkinsonian Disorders Internal medicine medicine Humans ddc:610 Pathological Aged Retrospective Studies business.industry medicine.disease Survival Analysis eye diseases 030104 developmental biology Clinical diagnosis Brain bank Neurology (clinical) Geriatrics and Gerontology business 030217 neurology & neurosurgery |
| Zdroj: | Parkinsonism & related disorders 84, 77-81 (2021). doi:10.1016/j.parkreldis.2021.01.015 |
| ISSN: | 1353-8020 |
| DOI: | 10.1016/j.parkreldis.2021.01.015 |
| Popis: | Introduction The MDS-PSP criteria expand the phenotypic spectrum of PSP by adding to Richardson's syndrome (PSP-RS) other presentations such as PSP-parkinsonism (PSP–P), PSP-pure-gait-freezing (PSP-PGF), PSP-speech-language (PSP-SL), PSP-frontal (PSP–F), PSP-postural-instability (PSP-PI) and PSP-corticobasal-syndrome (PSP-CBS). Evidence about the prognostic differences between PSP phenotypes is scarce and focused on PSP-RS vs. non-PSP-RS. Using a brain-bank cohort we assessed PSP survival not only in PSP-RS vs. non-PSP-RS, but also in PSP-RS + cortical vs. subcortical phenotypes. Besides, we assessed sensitivity and specificity of the MDS-PSP criteria in of PSP and other degenerative parkinsonisms. Methods We retrospectively applied the MDS-PSP diagnostic criteria to 32 definite PSP cases and 30 cases with other degenerative parkinsonian syndromes (Parkinson's disease [PD; n = 11], multiple system atrophy [MSA; n = 11], corticobasal degeneration [CBD; n = 8]). We conducted survival statistics in neuropathologically confirmed PSP cases considering PSP-RS vs. non-PSP-RS and PSP-RS + PSP-cortical (PSP–F + PSP-SL + PSP-CBS) vs. PSP-subcortical (PSP–P + PSP-PGF) phenotypes. We also adjusted survival analyses for PSP tau scores. Results Diagnostic sensitivity was 100% and specificity ranged from 47% to 87% when excluding cases that met the “suggestive of PSP” definition early in their disease course but with other clinical features better matching with a non-PSP pathological diagnosis. Survival was significantly shorter in PSP-RS vs. non-PSP-RS cases, but it was more markedly shorter in PSP-RS + PSP-cortical vs. PSP-subcortical, independently of PSP tau scores, which were not associated with survival. Conclusions PSP-subcortical phenotypes appear to have longer survival than PSP-RS and cortical phenotypes. This might be of prognostic relevance when informing patients upon clinical diagnosis. |
| Databáze: | OpenAIRE |
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