Frontotemporal dementia

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Última revisão: 10 Dec 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

Declarações

RD declares that he has no competing interests.

Agradecimentos

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.

Declarações

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

Revisores

Daniel Kaufer, MD

Director

Memory Disorders Program

Department of Neurology

University of North Carolina at Chapel Hill

Declarações

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

Declarações

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

Declarações

PS declares that he has no competing interests.

Créditos aos pareceristas

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Declarações

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Referências

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Principais artigos

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.Texto completo Resumo

American College of Radiology. ACR appropriateness criteria: dementia. 2024 [internet publication].Texto completo

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. Resumo

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.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Alzheimer disease
- Dementia with Lewy bodies (DLB)
- Vascular dementia
Mais Diagnósticos diferenciais
Diretrizes
- European intersocietal recommendations for the biomarker-based diagnosis of neurocognitive disorders
- ACR appropriateness criteria: dementia
Mais Diretrizes
Folhetos informativos para os pacientes
Caring for someone with dementia
Dementia: frontotemporal dementia
Mais Folhetos informativos para os pacientes
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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

Declarações

Agradecimentos

Declarações

Revisores

Daniel Kaufer, MD

Declarações

Bruce L. Miller, MD

Declarações

Philip Scheltens, MD, PhD

Declarações

Créditos aos pareceristas

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Folhetos informativos para os pacientes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice