Very Early‐Onset Behavioral Variant Frontotemporal Dementia: A Case Report

Very early onset behavioral variant frontotemporal dementia (bvFTD) is an uncommon clinical presentation of frontotemporal dementia (FTD). A review of the literature identified 18 patients with an onset age of 25 years or younger, with the youngest patient reported to be 14 years old. Due to its early onset and primarily neuropsychiatric symptoms, bvFTD is often misdiagnosed as a psychiatric condition. In these cases, the most commonly reported pathology is tau protein, while mutations are frequently found in the MAPT gene. This differs from typical FTD, where the most commonly reported pathology is TDP-43 protein. Although rare, it is important to consider bvFTD even in very young patients presenting with new, significant behavioral disturbances.

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Publication Details

Journal
Alzheimer s & Dementia
Published
2025-12-01
DOI
https://doi.org/10.1002/alz70857_104327
Primary Topic
Amyotrophic Lateral Sclerosis Research
Type
article
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article

Very Early‐Onset Behavioral Variant Frontotemporal Dementia: A Case Report

Pablo Andrés Lopera Toro, Verónica Pérez, Rodrigo Santibáñez, Marcelo Jobet
Alzheimer s & Dementia
Amyotrophic Lateral Sclerosis Research
article

Very Early‐Onset Behavioral Variant Frontotemporal Dementia: A Case Report

Pablo Andrés Lopera Toro, Verónica Pérez, Rodrigo Santibáñez, Marcelo Jobet
article en

Abstract

Very early onset behavioral variant frontotemporal dementia (bvFTD) is an uncommon clinical presentation of frontotemporal dementia (FTD). A review of the literature identified 18 patients with an onset age of 25 years or younger, with the youngest patient reported to be 14 years old. Due to its early onset and primarily neuropsychiatric symptoms, bvFTD is often misdiagnosed as a psychiatric condition. In these cases, the most commonly reported pathology is tau protein, while mutations are frequently found in the MAPT gene. This differs from typical FTD, where the most commonly reported pathology is TDP-43 protein. Although rare, it is important to consider bvFTD even in very young patients presenting with new, significant behavioral disturbances.

Alzheimer s & DementiaVol. 21(S3)
Pontificia Universidad Católica de Chile (CL), Complejo Asistencial Sótero del Río (CL)
Reduced inequalities
Openalex Percentile: Top 31%
Amyotrophic Lateral Sclerosis Research
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