Diagnosis and management of frontotemporal dementia: a narrative review.
L’essentiel
Frontotemporal dementia (FTD) is an umbrella term that encompasses a group of clinically heterogeneous neurodegenerative disorders. It is biologically referred to as Frontotemporal lobar degeneration (FTLD) and is characterized by progressive degeneration of frontal and/or temporal lobes. FTD poses substantial diagnostic and therapeutic challenges due to its heterogeneous clinical presentations, limited biomarker specificity, and overlap with other neurodegenerative and psychiatric diseases. This review synthesizes updated knowledge on the clinical phenotypes of FTD, their prognostic elements, and the underlying genetic and molecular mechanisms. Advances in diagnostic strategies are discussed, including structural and functional neuroimaging, fluid biomarkers, and genetic testing, together with emerging digital and AI-assisted tools that may enhance diagnostic precision. Evidence-based management approaches are examined, alongside the role of multidisciplinary care and caregiver support. Promising therapeutic strategies, including gene-targeted interventions, antisense oligonucleotides, progranulin restoration strategies, immunotherapies, and neuromodulation techniques, are discussed considering recent clinical and preclinical developments. Despite the absence of approved disease-modifying therapies, rapid progress in biomarker development, patient stratification, and personalized approaches offers encouraging prospects for more effective interventions across the FTD spectrum. PubMed/MEDLINE was searched for peer-reviewed articles on FTD diagnosis and management; reference lists of key articles were screened to identify additional sources.
Synthèse détaillée
Résumé original
Frontotemporal dementia (FTD) is an umbrella term that encompasses a group of clinically heterogeneous neurodegenerative disorders. It is biologically referred to as Frontotemporal lobar degeneration (FTLD) and is characterized by progressive degeneration of frontal and/or temporal lobes. FTD poses substantial diagnostic and therapeutic challenges due to its heterogeneous clinical presentations, limited biomarker specificity, and overlap with other neurodegenerative and psychiatric diseases. This review synthesizes updated knowledge on the clinical phenotypes of FTD, their prognostic elements, and the underlying genetic and molecular mechanisms. Advances in diagnostic strategies are discussed, including structural and functional neuroimaging, fluid biomarkers, and genetic testing, together with emerging digital and AI-assisted tools that may enhance diagnostic precision. Evidence-based management approaches are examined, alongside the role of multidisciplinary care and caregiver support. Promising therapeutic strategies, including gene-targeted interventions, antisense oligonucleotides, progranulin restoration strategies, immunotherapies, and neuromodulation techniques, are discussed considering recent clinical and preclinical developments. Despite the absence of approved disease-modifying therapies, rapid progress in biomarker development, patient stratification, and personalized approaches offers encouraging prospects for more effective interventions across the FTD spectrum. PubMed/MEDLINE was searched for peer-reviewed articles on FTD diagnosis and management; reference lists of key articles were screened to identify additional sources.