Frontotemporal dementia

Información en español Title

Other Names:

Dementia, frontotemporal, with parkinsonism; Frontotemporal dementia with parkinsonism; Frontotemporal lobe dementia (FLDEM); Dementia, frontotemporal, with parkinsonism; Frontotemporal dementia with parkinsonism; Frontotemporal lobe dementia (FLDEM); MSTD; Multiple system tauopathy with presenile dementia; FTD See More

Categories:

Nervous System Diseases

Subtypes:

Behavioral variant of frontotemporal dementia; Primary progressive aphasia; Semantic dementia

Summary Summary

Frontotemporal dementias (FTDs) are a group of neurodegenerative disorders associated with shrinking of the frontal and temporal anterior lobes of the brain.^[1] Symptoms include marked changes in social behavior and personality, and/or problems with language. People with behavior changes may have disinhibition (with socially inappropriate behavior), apathy and loss of empathy, hyperorality (eating excessive amounts of food or attempting to consume inedible things), agitation, compulsive behavior, and various other changes.^[1]^[2] Examples of problems with language include difficulty speaking or understanding speech.^[1] Some people with FTD also develop a motor syndrome such as parkinsonism or motor neuron disease (which may be associated with various additional symptoms).^[2]

There is a strong genetic component to FTDs.^[1]^[2] It sometimes follows an autosomal dominant inheritance pattern, or sometimes there is a general family history of dementia or psychiatric disorders.^[2] The three main genes responsible for familial FTD are MAPT, GRN, and C9orf72. However, the genetic cause of familial FTD cannot always be identified.^[2]

While there are currently no treatments to slow or stop the progression of the disease, some of the symptoms can be managed.^[3] Treatment of symptoms may involve behavior modification, or medications for symptoms such as aggressiveness, agitation, or dangerous behaviors. Anti-depressants have been shown to improve some symptoms.^[1] Involving a team of specialists can help ensure that the challenges of the disease are properly addressed.^[3] Unfortunately, the outlook for people with FTD is poor, as the disease often progresses rapidly. However, the outlook does vary, with the disease course ranging from less than 2 years in some people, to more than 10 years in others.^[1]

Although the name and classification of FTD has been a topic of discussion for over a century, the current classification considers Pick’s disease, primary progressive aphasia, and semantic dementia as sub-types of FTD.^[1]

Last updated: 8/6/2018

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
Percent of people who have these symptoms is not available through HPO
Amyotrophic lateral sclerosis		0007354
Apathy	Lack of feeling, emotion, interest	0000741
Autosomal dominant inheritance		0000006
Disinhibition		0000734
Frontal lobe dementia		0000727
Frontotemporal dementia		0002145
Hyperorality		0000710
Inappropriate laughter		0000748
Inappropriate sexual behavior		0008768
Irritability	Irritable	0000737
Language impairment		0002463
Neuronal loss in central nervous system	Loss of brain cells	0002529
Parkinsonism		0001300
Personality changes	Personality change	0000751
Polyphagia	Voracious appetite	0002591
Primitive reflex		0002476

Showing of 16 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 12/1/2020

Do you have updated information on this disease? We want to hear from you.

Diagnosis Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.
Orphanet lists international laboratories offering diagnostic testing for this condition.

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.
You can view a list of frontotemporal dementia research centers on the Frontotemporal Dementia Caregiver Support Center Web site by clicking on "research centers" above.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Frontotemporal dementia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition.

Patient Registry

The Clinical Research in Amyotrophic Lateral Sclerosis and Related Disorders for Therapeutic Development (CREATE) Consortium is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research involving sporadic and familial forms of amyotrophic lateral sclerosis, frontotemporal dementia (FTD), primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), and progressive muscular atrophy (PMA). The CREATE Consortium has a contact registry for patients who wish to be contacted about clinical research opportunities and updates on the progress of the research projects.

For more information on the registry see: https://www.rarediseasesnetwork.org/registry/index.htm
The Advancing Research and Treatment for Frontotemporal Lobar Degeneration Consortium (ARTFL) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research on conditions that occur in association with frontotemporal lobar degeneration. The ARTFL has a contact registry for patients who wish to be contacted about clinical research opportunities and updates on the progress of the ARTFL research projects.

For more information on the registry see: http://www.rarediseasesnetwork.org/cms/artfl/Get-Involved/Contact-Registry

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Association for Frontotemporal Degeneration (AFTD)
2700 Horizon Drive, Suite 120
King of Prussia, PA 19406
Toll-free: 1-866-507-7222
Telephone: +1-267-514-7221
E-mail: info@theaftd.org
Website: https://www.theaftd.org/
Cure VCP Disease, Inc.
PO Box 6533
Americus, GA 31709
E-mail: curevcpdisease@gmail.com
Website: https://www.curevcp.org/
Frontotemporal Dementia Caregiver Support Center
E-mail: jeffbrauer@cableone.net
Website: http://www.ftdsupport.com

Social Networking Websites

The FTD Support Forum is a online community for people with frontotemporal dementia and their families. Click on FTD support Forum to learn more.

Do you know of an organization? We want to hear from you.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

The Social Security Administration has included this condition in their Compassionate Allowances Initiative. This initiative speeds up the processing of disability claims for applicants with certain medical conditions that cause severe disability. More information about Compassionate Allowances and applying for Social Security disability is available online.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.

In-Depth Information

GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the following links to view articles on the CHMP2B-related, GRN-related, and MAPT-related, types of this condition.
Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Frontotemporal dementia. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NORD RareLaunch® Workshops
December 1, 2020
Rare Diseases Are Not Rare - Gallery of Creative Work Raises Awareness of Rare Diseases
September 1, 2020
NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community
May 22, 2020
NCATS Translational Approach Addresses COVID-19
May 21, 2020

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Frontotemporal Dementia Information Page. National Institute of Neurological Disorders and Stroke (NINDS). June 21, 2018; https://www.ninds.nih.gov/Disorders/All-Disorders/Frontotemporal-Dementia-Information-Page.
Lee SE, Miller BL. Frontotemporal dementia: Clinical features and diagnosis. UpToDate. Waltham, MA: UpToDate; December 15, 2017; https://www.uptodate.com/contents/frontotemporal-dementia-clinical-features-and-diagnosis.
Understanding FTD: Disease Overview. The Association for Frontotemporal Degeneration (AFTD). 2016; http://www.theaftd.org/understandingftd/ftd-overview.

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