Association of PSP phenotypes with survival: A brain-bank study. (March 2021)

• Record Type: Journal Article
• Authors: Guasp, Mar; Molina-Porcel, Laura; Painous, Celia; Caballol, Nuria; Camara, Ana; Perez-Soriano, Alexandra; Sánchez-Gómez, Almudena; Garrido, Alicia; Muñoz, Esteban; Marti, Maria Jose; Valldeoriola, Francesc; Grau, Oriol; Gelpí, Ellen; Respondek, Gesine; Höglinger, Guenter H.; Compta, Yaroslau
• Is Part Of: Parkinsonism & related disorders. Volume 84(2021)
• Page Start: 77
• Page End: 81
• Publication Date: 2021-03
• Subjects: PSP -- Phenotype -- Richardson's syndrome -- PSP-P -- PSP-PGF -- Prognosis -- Survival; Parkinson's disease -- Periodicals; Movement disorders -- Periodicals; Movement Disorders -- Periodicals; Nerve Degeneration -- Periodicals; Nervous System Diseases -- Periodicals; Parkinson Disease -- Periodicals; Tremor -- Periodicals; Parkinson, Maladie de -- Périodiques; Parkinson's disease; 616.833
• DOI: 10.1016/j.parkreldis.2021.01.015
• Languages: English
• ISSNs: 1353-8020

Abstract

Abstract: 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. Highlights: The MDS PSP diagnostic criteria have 100% sensitivity and 87% specificity when excluding s.o.PSP with non-PSP features. Survival is significantly shorter in PSP-RS than in the other phenotypes considered together. This difference is more remarkable when comparing PSP-RS and PSP cortical phenotypes to the subcortical PSP phenotypes. This information can be relevant for prognosis upon clinical diagnosis.