Cognitive and Behavioral Profile of Progressive Supranuclear Palsy and Its Phenotypes

Overview

Journal J Neurol

Specialty Neurology

Date 2021 Mar 11

PMID 33704556

Citations 13

Authors

Andrea Horta-Barba

Javier Pagonabarraga

Saul Martinez-Horta

Laura Busteed

Berta Pascual-Sedano

Ignacio Illan-Gala

Juan Marin-Lahoz

Ignacio Aracil-Bolanos

Jesus Perez-Perez

Frederic Sampedro

Helena Bejr-Kasem

Jaime Kulisevsky

Affiliations

Soon will be listed here.

Abstract

Background: Although several progressive supranuclear palsy (PSP) phenotypes have recently been described, studies identifying cognitive and neuropsychiatric differences between them are lacking.

Methods: An extensive battery of cognitive and behavioural assessments was administered to 63 PSP patients, 25 PD patients with similar sociodemographic characteristics, and 25 healthy controls. We analysed differences in phenomenology, frequency and severity of cognitive and neuropsychiatric symptoms between PSP, PD and HC, and between PSP subtypes.

Results: Regarding phenotypes, 64.6% met criteria for Richardson's syndrome (PSP-RS), 10.7% PSP with predominant Parkinsonism (PSP-P), 10.7% with PSP progressive gait freezing (PSP-PGF), and 10.7% PSP with predominant speech/language disorder (PSP-SL). Impairment was more severe in the PSP group than in the PD and HC groups regarding motor scores, cognitive testing and neuropsychiatric scales. Cognitive testing did not clearly differentiate between PSP phenotypes, but PSP-RS and PSP-SL appeared to have more cognitive impairment than PSP-PGF and PSP-P, mainly due to an increased impairment in frontal executive domains. Regarding neuropsychiatric disturbances, no specific behavior was more common in any of the PSP subtypes.

Conclusion: Motor deficits delineate the phenotypes included in currently accepted MDS-PSP criteria. Cognition and behavioural disturbances are common in PSP and allow us to distinguish this disorder from other neurological diseases, but they do not differentiate between PSP phenotypes.

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