Applicability and Correlates of a Symptom-Led Staging System for Primary Progressive Aphasia.

BACKGROUND

This study aimed at assessing the applicability of a symptom-led staging system for primary progressive aphasia (PPA) based on retrospective medical records, as well as at exploring their demographic and clinical correlates.

METHODS

75 PPA patients (10 semantic, 28 non-fluent, 22 logopenic, and 16 mixed variants) were retrospectively staged according to the PPA Progression Planning Aid (PPA-Squared) system, which stages the disease by accounting for clinical features along three axes: (1) Communication; (2) Non-Verbal Thinking and Personality; (3) Personal Care and Well-Being. The percentage of successfully staged patients was computed. The association between PPA-Squared scores and demographic and clinical data was tested via non-parametric tests. The predictive capability of PPA-Squared scores towards survival was explored via a Mantel-Cox test.

RESULTS

89.3% of patients were successfully staged based on retrospective medical records. The PPA-Squared was associated with the MMSE (p < 0.001), ADL (p = 0.021), and IADL scores (p < 0.001) and a set of second-level cognitive measures tapping on attention, executive functions, language, long-term memory, and visuo-spatial abilities (p ≤ 0.049). No association was found between the PPA-Squared and demographic features, symptom duration, PPA phenotype, the presence of motor involvement, and survival.

DISCUSSION: The PPA-Squared is a feasible and clinically valid tool for staging PPA patients based on their cognitive and functional status.