The Portuguese who Could no Longer Speak French: Primary Progressive Aphasia (PPA) in a Bilingual Man

Abstract

Introduction:

PPA is characterized by progressive language dissolution, with remarkable spare of other cognitive domains for at least two years, and should be considered in the differential diagnosis of dementia.

Clinical case:

A 56-year-old previously healthy man was seen for speech difficulties. He studied Portuguese for 4 years, and then went to live in France, where he learned French for 3 years. He returned definitely to Portugal when he was 42. Since early adolescence he was fluent on speaking, reading and writing in both languages. In the last 12 years he spoke French mainly when visiting his family. Three years ago he started showing disintegration of his second language preceding that of his native one. Upon examination he had laborious, effortful, nonfluent and agrammatic speech, with severe anomia and some repetition and complex-command comprehension difficulties. He could not name, understand or write any French word. The remaining neurological examination was near-normal to age and education level (MMSE - 25/30). Brain MRI showed left-side predominant frontotemporal cortical atrophy. PET scan revealed hypometabolism in the same area and the temporal lobe, anterior cynguli and dorsolateral frontal cortex.

Conclusion:

Our patient has nonfluent variant of PPA. Cases like this can give further insight into the neural network subserving language acquisition and dissolution. In proficient bilinguals, the main point on language dissolution in PPA seems to be the recency of use. The best way to confirm this would be to systematically characterize PPA patients on secondary language usage, attained proficiency and disintegration sequence.