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Early functional MRI changes in a prodromal semantic variant of primary progressive aphasia: a longitudinal case report

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Abstract

Objective

To assess longitudinal patterns of brain functional MRI (fMRI) activity in a case of prodromal semantic variant of a primary progressive aphasia (svPPA).

Methods

Clinical, cognitive and neuroimaging data (T1-weighted and task-based fMRI during silent naming [SN] and object knowledge [OK]) were obtained at baseline, month 8 and month 16 from a 49-year-old lady presenting with anomias and evolving to overt svPPA in 8 months.

Results

At baseline, the patient showed isolated anomias and mild left anterior temporal pole atrophy. During SN–fMRI, she showed bilateral temporal and left inferior frontal gyri (iFG) activations. During OK–fMRI, we observed normal performance and the recruitment of bilateral posterior hippocampi, iFG and left middle orbitofrontal gyrus (mOFG). At month 8, the patient received a diagnosis of svPPA and showed isolated right iFG activity during SN–fMRI, and a borderline performance during OK–fMRI together with a disappearance of mOFG recruitment. At the last visit (after 7-month language therapy), the patient showed a stabilization of naming disturbances, and, compared to previous visits, an increased left iFG recruitment during SN–fMRI. During OK–fMRI, she performed abnormally and did not show the activity of mOFG and iFG. Across all visits, brain atrophy remained stable.

Conclusions

This case report showed longitudinal fMRI patterns during semantic-related tasks from prodromal to overt svPPA. Frontal brain recruitment may represent a compensatory mechanism in patients with early svPPA, which is likely to be reinforced by language-therapy. Brain fMRI is more sensitive compared with structural MRI to detect progressive brain changes associated with disease and treatment.

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Funding

This study has been supported by the Italian Ministry of Health (GR-2011-02351217).

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Correspondence to Federica Agosta.

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Conflicts of interest

Prof. Filippi is Editor-in-Chief of the Journal of Neurology; received compensation for consulting services and/or speaking activities from Biogen Idec, Merck-Serono, Novartis, Teva Pharmaceutical Industries; and receives research support from Biogen Idec, Merck-Serono, Novartis, Teva Pharmaceutical Industries, Roche, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla, and ARiSLA (Fondazione Italiana di Ricerca per la SLA. F. Agosta is Section Editor of NeuroImage: Clinical; has received speaker honoraria from Philips, Novartis and Biogen Idec; and receives or has received research supports from the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), and the European Research Council. E. Canu has received research supports from the Italian Ministry of Health. V. Bessi, S. Mazzeo, S. Padigioni, D. Simoni, B. Nacmias, S. Sorbi report no actual or potential conflicts of interest. D. Calderaro, V. Castelnovo, M. Leocadi, C. Cividini report no actual or potential conflicts of interest.

Ethical approval

This study has been approved by the local ethics committee and has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Consent to participate

The patient gave her informed consent prior to her inclusion in the study.

Availability of data and material

All data needed to evaluate the conclusions in the paper are present in the paper and/or in the Supplementary Materials. Additional data related to this paper may be requested from the corresponding author. Specifically, qualified academic investigators may request participant-level, de-identifed clinical data and supporting documents (statistical analysis plan and protocol) pertaining to this study.

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Canu, E., Bessi, V., Calderaro, D. et al. Early functional MRI changes in a prodromal semantic variant of primary progressive aphasia: a longitudinal case report. J Neurol 267, 3100–3104 (2020). https://doi.org/10.1007/s00415-020-10053-9

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  • DOI: https://doi.org/10.1007/s00415-020-10053-9

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