Demência frontotemporal
Summary
Definition
History and exam
Key diagnostic factors
- alteração da personalidade e dos hábitos, com embrutecimento do comportamento social
- perda progressiva da fluência ou compreensão da linguagem
- desenvolvimento do comprometimento de memória, desorientação ou apraxias
- falta de cuidados pessoais progressiva e abandono de trabalho, atividades e contatos sociais
Other diagnostic factors
- idade de início precoce
- história familiar de demência frontotemporal (DFT)
- hábitos alimentares alterados
- desatenção, preocupações pueris, economia de esforço, resposta impulsiva e comportamentos compulsivos
- sinais de esclerose lateral amiotrófica (ELA)
- sintomas parkinsonianos
- fasciculações, atrofia, hiper-reflexia e outros sinais de doença do neurônio motor
- reflexos glabelares, orbicular da boca (snout), de sucção, de procura (rooting) ou de preensão
- perda da controle vesical e intestinal
Risk factors
- mutações do gene MAPT
- mutações no gene GRN
- mutações no gene C9orf72
- outras mutações
- lesão cerebral traumática
- transtorno do estresse pós-traumático (TEPT)
Diagnostic tests
1st tests to order
- testes cognitivos
- ressonância nuclear magnética (RNM) cranioencefálica
- tomografia computadorizada (TC) cerebral
- Hemograma completo
- proteína C-reativa sérica
- hormônio estimulante da tireoide (TSH) sérico
- tiroxina livre (T4)
- perfil metabólico
- ureia sérica
- creatinina sérica
- TFHs
- níveis séricos de vitamina B12
- níveis de folato sérico
- sorologia para sífilis
- teste de vírus da imunodeficiência humana (HIV)
- ensaio de imunoadsorção enzimática (ELISA) sérico
Tests to consider
- PET com fluordesoxiglucose (FDG)/CT cerebral
- tomografia computadorizada por emissão de fóton único (SPECT) de perfusão cerebral
- PET-CT de amiloide cranioencefálica
- biópsia do cérebro
- teste genético
- perfil do tecido conjuntivo
- velocidade de hemossedimentação sérica
- eletroencefalograma (EEG)
- eletromiografia (EMG)
Emerging tests
- biomarcadores em fluidos
Treatment algorithm
todos os pacientes
Contributors
Authors
Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), FRCPsych
Consultant Psycho-geriatrician
Waitemata District Health Board
Auckland
New Zealand
Disclosures
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
NC
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
CA
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
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
- Doença de Alzheimer
- Demência com corpos de Lewy (DCL)
- Demência vascular
More DifferentialsGuidelines
- European intersocietal recommendations for the biomarker-based diagnosis of neurocognitive disorders
- ACR appropriateness criteria: dementia
More GuidelinesPatient information
Tratamento de pessoas com demência
Demência: demência frontotemporal
More Patient information
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