A Reliable and Rapid Language Tool for the Diagnosis, Classification, and Follow-Up of Primary Progressive Aphasia Variants.

Stéphane Epelbaum,Bruno Dubois,Sophie Tezenas Du Montcel,Emmanuel Roze,Yasmina Michel Saade,Marie Nogues,Sophie Ferrieux,Céline Arbizu,Carole Azuar,Marc Teichmann,Constance Flamand Roze

doi:10.3389/fneur.2020.571657

Abstract

Background: Primary progressive aphasias (PPA) have been investigated by clinical, therapeutic, and fundamental research but examiner-consistent language tests for reliable reproducible diagnosis and follow-up are lacking.Methods: We developed and evaluated a rapid language test for PPA (“PARIS”) assessing its inter-examiner consistency, its power to detect and classify PPA, and its capacity to identify language decline after a follow-up of 9 months. To explore the reliability and specificity/sensitivity of the test it was applied to PPA patients (N = 36), typical amnesic Alzheimer's disease (AD) patients (N = 24) and healthy controls (N = 35), while comparing it to two rapid examiner-consistent language tests used in stroke-induced aphasia (“LAST”, “ART”).Results: The application duration of the “PARIS” was ~10 min and its inter-rater consistency was of 88%. The three tests distinguished healthy controls from AD and PPA patients but only the “PARIS” reliably separated PPA from AD and allowed for classifying the two most frequent PPA variants: semantic and logopenic PPA. Compared to the “LAST” and “ART,” the “PARIS” also had the highest sensitivity for detecting language decline.Conclusions: The “PARIS” is an efficient, rapid, and highly examiner-consistent language test for the diagnosis, classification, and follow-up of frequent PPA variants. It might also be a valuable tool for providing end-points in future therapeutic trials on PPA and other neurodegenerative diseases affecting language processing.

Highlights

Primary progressive aphasias (PPA) are neurodegenerative diseases characterized by isolated or highly predominant language impairment
We evaluate the external validity of the Progressive Aphasia Rating Scale (PARIS) there is no standard aphasia battery designed to diagnose PPA
The application duration of the PARIS was not assessed in the follow-up visit, which could have provided an indicator reflecting the influence of aphasia severity on the application duration of the PPA

Summary

Introduction

Primary progressive aphasias (PPA) are neurodegenerative diseases characterized by isolated or highly predominant language impairment. These two tests might represent valuable tools in PPA but they were not designed for degenerative conditions requiring the detection, classification and follow-up of PPA patients To address these open issues, we developed a rapid language test with a binary rating scale, designed for PPA (Progressive Aphasia RatIng Scale “PARIS”), while assessing its power for diagnosis/classification of PPA and its sensitivity to language decline, while comparing it with the two rapid and inter-rater consistent language tests: the “LAST” and “ART.” To evaluate its capacity to diagnose and differentiate PPA from other neurodegenerative conditions with aphasia features we applied the test both to PPA variants and typical amnesic AD. Primary progressive aphasias (PPA) have been investigated by clinical, therapeutic, and fundamental research but examiner-consistent language tests for reliable reproducible diagnosis and follow-up are lacking

Methods

Results

Conclusion