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
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
Externí odkaz: