Resumo
Definição
História e exame físico
Principais fatores diagnósticos
- 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
Outros fatores diagnósticos
- idade de início precoce
- história familiar de 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
Fatores de risco
- mutações do gene MAPT
- mutações no gene GRN
- mutações no gene C9orf72
- lesão cerebral traumática
Investigações diagnósticas
Primeiras investigações a serem solicitadas
- testes cognitivos formais
- 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
Investigações a serem consideradas
- tomografia por emissão de pósitrons (PET) com fluordeoxiglicose (FDG) cerebral
- tomografia computadorizada por emissão de fóton único (SPECT) cerebral
- biópsia do cérebro
- teste genético
- perfil do tecido conjuntivo
- velocidade de hemossedimentação sérica
Novos exames
- análise do líquido cefalorraquidiano
Algoritmo de tratamento
todos os pacientes
Colaboradores
Autores
Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), MRCPsych
Consultant Psycho-geriatrician
Waitemata District Health Board
Auckland
New Zealand
Disclosures
RKR declares that he has no competing interests.
Sachin Jauhari, DMH, MRCPsych, FRANZCP
Consultant Psycho-geriatrician
Waitemata District Health Board
Honorary Senior Lecturer in Old Age Psychiatry
University of Auckland
Auckland
New Zealand
Disclosures
SJ declares that he has no competing interests.
Acknowledgements
Dr Raghava Kurup Radhakrishnan and Dr Sachin Jauhari would like to gratefully acknowledge 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
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.
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
Moore KM, Nicholas J, Grossman M, et al. Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study. Lancet Neurol. 2020 Feb;19(2):145-56.Full text Abstract
Mackenzie IR, Neumann M. Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies. J Neurochem. 2016 Aug;138(suppl 1):54-70.Full text Abstract
NHMRC Partnership Centre for Dealing with Cognitive and Related Functional Decline in Older People. Clinical practice guidelines and principles of care for people with dementia. Feb 2016 [internet publication].Full text
Dyer SM, Harrison SL, Laver K, et al. An overview of systematic reviews of pharmacological and non-pharmacological interventions for the treatment of behavioral and psychological symptoms of dementia. Int Psychogeriatr. 2018 Mar;30(3):295-309.Full text Abstract
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
Trieu C, Gossink F, Stek ML, et al. Effectiveness of pharmacological interventions for symptoms of behavioral variant frontotemporal dementia: a systematic review. Cogn Behav Neurol. 2020 Mar;33(1):1-15. 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 (DA)
- Demência com corpos de Lewy (DCL)
- Demência vascular
More DifferentialsGuidelines
- Clinical guidance in neuropalliative care: an AAN position statement
- Ethical considerations in dementia diagnosis and care: an AAN position statement
More GuidelinesPatient information
Tratamento de pessoas com demência
Demência: demência frontotemporal
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