Frontotemporal dementia

Last reviewed: 5 Sep 2022

Last updated: 17 Sep 2021

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

More key diagnostic factors

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

Other diagnostic factors

Risk factors

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

More risk factors

Diagnostic investigations

1st investigations 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

More 1st investigations to order

Investigations to consider

brain fluorodeoxyglucose (FDG)-PET
brain single-photon emission computed tomography (SPECT)
brain biopsy
genetic testing
connective tissue panel
serum erythrocyte sedimentation rate

More investigations to consider

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.

Differentials
- Alzheimer disease (AD)
- Dementia with Lewy bodies (DLB)
- Vascular dementia
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- Use of antipsychotics to treat agitation or psychosis in patients with dementia
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Patient leaflets
Caring for someone with dementia
Dementia: frontotemporal dementia
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Frontotemporal dementia

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations 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

Differentials

Guidelines

Patient leaflets

Log in or subscribe to access all of BMJ Best Practice

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