ftd

Frontotemporal dementia (FTD) is a group of neurodegenerative disorders characterized by progressive atrophy of the frontal and/or temporal lobes of the brain. It is a common cause of early-onset dementia and typically emerges in midlife, though onset can occur earlier or later.

FTD includes several clinical syndromes, most prominently behavioral variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA), and nonfluent/agrammatic variant PPA (nfvPPA). bvFTD presents with changes in personality, social conduct, apathy, or disinhibition; svPPA and nfvPPA affect language, with svPPA impairing word meaning and nfvPPA impairing speech production.

Most cases are sporadic, but a subset runs in families. Genetic causes include mutations in MAPT, GRN, and C9orf72, among others, with autosomal dominant inheritance in some families. Pathology often involves abnormal accumulations of tau or TDP-43 protein, with subtype-specific patterns that correlate with clinical presentation.

Diagnosis relies on clinical features supported by neuropsychological testing and neuroimaging. MRI commonly shows atrophy in frontal and/or temporal regions; FDG-PET can reveal hypometabolism in these areas. Genetic testing and biomarker evaluation may be used in selected cases to exclude other disorders.

Management is supportive and multidisciplinary. There is no cure or proven disease-modifying therapy. Treatment focuses on managing symptoms, providing behavioral strategies, speech and language therapy, and caregiver support. Some medications may address mood or behavioral symptoms, with emphasis on safety and quality of life. The course is progressive with variable tempo, and mean survival after onset is measured in years. FTD is a leading cause of early-onset dementia, typically presenting in the 40s to 60s.