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 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
Risk factors
- mutations in MAPT gene
- mutations in GRN gene
- mutations in C9orf72 gene
- other mutations
- traumatic brain injury
- post-traumatic stress disorder (PTSD)
Diagnostic tests
1st tests to order
- 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/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)
Treatment algorithm
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 here.
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