Frontotemporal dementia

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

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Última revisão: 20 Aug 2025

Última atualização: 19 Sep 2025

Frontotemporal dementia manifests primarily as disruption in personality and social conduct, or as a primary language disorder.

Almost 50% of affected people display parkinsonism; a smaller subset may have motor neuron disease.

Clinical diagnosis derives primarily from examination and brain imaging.

Diagnostic confirmation is based on pathologic exam or identification of gene mutation.

Treatment is supportive, combining pharmacotherapy with nonpharmacologic and behavioral interventions, caregiver guidance, community services, and social work involvement.

Definição

Frontotemporal dementia (FTD) is the second most frequent primary neurodegenerative dementia (after Alzheimer disease) in adults <65 years of age. FTD typically appears in midlife, with average age of onset between 45 and 65 years, and peak prevalence in the seventh decade.[1][2][3][4]

The FTD spectrum comprises a heterogeneous group of conditions that are heritable in some cases. Prominent early symptoms include progressive coarsening of personality, social behavior, and self-regulation (of emotions, drives, and behavior), with language impairment. Gross changes in social behavior and language are often seen, including indifference to self-care and others' needs, loss of speech and comprehension, loss of empathy, distractibility, impulsiveness, disinhibition, stereotyped behaviors and rigid routines, and compulsions.[5][6]

História e exame físico

Principais fatores diagnósticos

coarsening of personality, social behavior, and habits
progressive loss of language fluency or comprehension
development of memory impairment, disorientation, or apraxias
progressive self-neglect and abandonment of work, activities, and social contacts

Detalhes completos

Outros fatores diagnósticos

early age at onset
family history of frontotemporal dementia (FTD)
altered eating habits
inattentiveness, puerile preoccupations, economy of effort, impulsive responding, and compulsive behaviors
signs of amyotrophic lateral sclerosis (ALS)
parkinsonian symptoms
fasciculations, atrophy, hyperreflexia, and other signs of motor neuron disease
glabellar, snout, sucking, rooting, or grasp reflex
loss of bladder and bowel control

Detalhes completos

Fatores de risco

mutations in MAPT gene
mutations in GRN gene
mutations in C9orf72 gene
other mutations
traumatic brain injury
post-traumatic stress disorder (PTSD)

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

cognitive testing
brain MRI
brain CT
CBC
serum CRP
serum thyroid-stimulating hormone (TSH)
free thyroxine (T4)
metabolic panel
serum BUN
serum creatinine
LFTs
serum vitamin B12 levels
serum folate levels
syphilis serology
HIV testing
serum enzyme-linked immunosorbent assay

Detalhes completos

Investigações a serem consideradas

brain fluorodeoxyglucose (FDG)-PET/CT
brain perfusion single-photon emission computed tomography (SPECT)
brain amyloid PET/CT
brain biopsy
genetic testing
connective tissue panel
serum erythrocyte sedimentation rate
electroencephalogram (EEG)
electromyogram (EMG)

Detalhes completos

Novos exames

fluid biomarkers

Algoritmo de tratamento

CONTÍNUA

all patients

Colaboradores

Autores

Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), FRCPsych

Consultant Psycho-geriatrician

Waitemata District Health Board

Auckland

New Zealand

Declarações

RKR serves as an advisory board member for the Australian and New Zealand Journal of Psychiatry.

Rommel Dawith, MBChB, FRANZCP, FFPOA

Psychiatrist of Old Age

Waitemata District Health Board

Auckland

New Zealand

Disclosures

RD declares that he has no competing interests.

Acknowledgements

Dr Raghava Kurup Radhakrishnan and Dr Rommel Dawith would like to gratefully acknowledge Dr Sachin Jauhari, Dr Lawrence Whalley, and Dr Chiadi Onyike, the previous contributors to this topic, and colleagues Dr Ann Boston (clinical psychologist), Dr Zara Godinovich (clinical psychologist), and Diane Ellis (clinical nurse specialist) for their suggestions.

Disclosures

SJ, LW, CO, AB, ZG, and DE declare that they have no competing interests.

Peer reviewers

Daniel Kaufer, MD

Director

Memory Disorders Program

Department of Neurology

University of North Carolina at Chapel Hill

Disclosures

DK has received research support, speaking honoraria, and consulting fees from Eisai, Forest Laboratories, Johnson & Johnson, Medivation, Novartis, Ortho-McNeil, and Pfizer.

Bruce L. Miller, MD

Professor of Neurology

University of California

San Francisco

Disclosures

BLM declares that he has no competing interests.

Philip Scheltens, MD, PhD

Professor of Neurology

Department of Neurology/Alzheimer Center

VU University Medical Center

Amsterdam

The Netherlands

Disclosures

PS declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011 Sep;134(Pt 9):2456-77.Full text Abstract

American College of Radiology. ACR appropriateness criteria: dementia. 2024 [internet publication].Full text

Buoli M, Serati M, Caldiroli A, et al. Pharmacological management of psychiatric symptoms in frontotemporal dementia: a systematic review. J Geriatr Psychiatry Neurol. 2017 May;30(3):162-9. Abstract

Reus VI, Fochtmann LJ, Eyler AE, et al. The American Psychiatric Association practice guideline on the use of antipsychotics to treat agitation or psychosis in patients with dementia. Am J Psychiatry. 2016 May 1;173(5):543-6.Full text Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials
- Alzheimer disease
- Dementia with Lewy bodies (DLB)
- Vascular dementia
More Differentials
Guidelines
- European intersocietal recommendations for the biomarker-based diagnosis of neurocognitive disorders
- ACR appropriateness criteria: dementia
More Guidelines
Patient information
Caring for someone with dementia
Dementia: frontotemporal dementia
More Patient information
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Frontotemporal dementia

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Novos exames

Algoritmo de tratamento

all patients

Colaboradores

Autores

Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), FRCPsych

Declarações

Rommel Dawith, MBChB, FRANZCP, FFPOA

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Daniel Kaufer, MD

Disclosures

Bruce L. Miller, MD

Disclosures

Philip Scheltens, MD, PhD

Disclosures

Peer reviewer acknowledgements

Disclosures

References

Key articles

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials

Guidelines

Patient information

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

Log in to access all of BMJ Best Practice