BACKGROUND:
Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by progressive deterioration of language. Being rare, reports of PPA in multilingual individual are scarce, despite more than half of the world population being multilingual.
METHODS:
We describe clinical characteristics of 33 bilingual patients with PPA, including symptom presentation and language deficits pattern in their first (L1) and second language (L2), through a systematic literature review and new cases retrospectively identified in five countries.
RESULTS:
Fourteen patients presented with nonfluent/agrammatic variant, six with semantic variant, and 13 with logopenic variant, with a median symptom onset of two years. Word-finding difficulties was the first symptom in 65% of all cases, initially noticed in L2, and not always the dominant language. Our group had 22 different languages as L1, and nine as L2. At the whole-group level there is a tendency for parallel impairment in both languages, in line with the shared bilingual neural substrate hypothesis, but each PPA variant shows some heterogeneity.
DISCUSSION:
Each PPA variant shows heterogeneity, warranting the need for comprehensive language and cognitive assessment across languages, as well as further clarification on the role of language mediators.