FIGURE 1.

Development of the Primary Progressive Aphasia Progression Planning Aid (PPA−Squared). The figure illustrates the different sources of information incorporated in PPA‐Squared. Data were synthesized from “top‐down” sources to characterize what was known from a clinician/researcher perspective (i.e., clinician‐led interpretation of patient records, histories, and neuropsychological test scores) and from “bottom‐up” sources to capture crucial information from those with lived experience of the conditions (i.e., patient/caregiver‐derived symptoms collected on surveys, and organized, prioritized, and amended to reflect their lived experience of disease progression). We present two levels of illness description that clinicians and people with lived experience of PPA may find useful: the initial six stage ordering with a granular overview of specific symptoms (see Figures 2 and 3); and the PPA‐Squared (Table 3), a distillation of the survey data to create a clinically applicable staging scheme for clinician scoring of illness progression and severity, under the same broad functional domains presented in the figures.