Frontotemporal dementia

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 9 Apr 2025

Last updated: 08 Nov 2022

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 medications with nonpharmacologic and behavioral interventions, caregiver guidance, community services, and social work involvement.

Definition

Frontotemporal dementias (FTDs) are the second most frequent primary neurodegenerative brain diseases (after Alzheimer disease) in adults <65 years of age. FTDs typically appear 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, self-regulation (of emotions, drives, and behavior), and language. 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]

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

Full details

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

Full details

Risk factors

mutations in MAPT gene
mutations in GRN gene
mutations in C9orf72 gene
traumatic brain injury

Full details

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

Full details

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

Full details

Emerging tests

cerebrospinal fluid analysis

Treatment algorithm

ONGOING

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

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

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

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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.

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Frontotemporal dementia

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Emerging tests

Treatment algorithm

all patients

Contributors

Authors

Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), MRCPsych

Disclosures

Sachin Jauhari, DMH, MRCPsych, FRANZCP

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Daniel Kaufer, MD

Disclosures

Bruce L. Miller, MD

Disclosures

Philip Scheltens, MD, PhD

Disclosures

References

Key articles

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials

Guidelines

Patient information

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

Log in to access all of BMJ Best Practice