Summary
Definition
History and exam
Key diagnostic factors
- 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
Other diagnostic factors
- early age at onset
- family history of 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
Risk factors
- mutations in MAPT gene
- mutations in GRN gene
- mutations in C9orf72 gene
- traumatic brain injury
Diagnostic tests
1st tests to order
- formal 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
Tests to consider
- brain fluorodeoxyglucose (FDG)-PET
- brain single-photon emission computed tomography (SPECT)
- brain biopsy
- genetic testing
- connective tissue panel
- serum erythrocyte sedimentation rate
Emerging tests
- cerebrospinal fluid analysis
Treatment algorithm
all patients
Contributors
Authors
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.
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
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
- Alzheimer disease (AD)
- Dementia with Lewy bodies (DLB)
- Vascular dementia
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
Caring for someone with dementia
Dementia: frontotemporal dementia
More Patient information
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