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Bilingualism in Primary Progressive Aphasia

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Background:

Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by progressive deterioration of language. Being rare, reports of PPA in multilingual individuals 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 5 countries.

Results:

In total, 14 patients presented with nonfluent/agrammatic variant, 6 with semantic variant, and 13 with logopenic variant, with a median symptom onset of 2 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 9 as L2. At the whole-group level there was a tendency for parallel impairment in both languages, in line with the shared bilingual neural substrate hypothesis, but each PPA variant showed some heterogeneity.

Discussion:

Each PPA variant showed heterogeneity, showing the need for comprehensive language and cognitive assessment across languages, as well as further clarification on the role of language mediators.
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Keywords: bilingualism; dementia; language; primary progressive aphasia; retrospective study; systematic review

Document Type: Research Article

Affiliations: 1: Neurocognition Unit, Department of Neurology, Hospital de Braga, Sete Fontes-São Victor, Braga, Portugal, Department of Neurology, RWTH Aachen University, Pauwelsstr, Aachen, JARA-BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH, Jülich and RWTH Aachen University, Aachen, Germany 2: Rotman Research Institute, 3560 Bathurst Street, North York, Ontario, University of Toronto, 500 University Avenue, Toronto, Canada 3: Department of Neurology, Hospital 12 de Octubre, Imas12. CIBERNED. Universidad Complutense de Madrid, Avda. de Córdoba, Madrid, Spain 4: Department of Neurology, University of Washington, 325 Ninth Ave Box 359791, Seattle, WA 5: Department of Neurology, RWTH Aachen University, Pauwelsstr, Aachen 6: Department of Neurology, RWTH Aachen University, Pauwelsstr, Aachen, JARA-BRAIN Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH, Jülich and RWTH Aachen University, Aachen, Germany 7: Neurocognition Unit, Department of Neurology, Hospital de Braga, Sete Fontes-São Victor, Braga, Portugal

Publication date: January 1, 2019

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