Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; Pick disease; 3R tauopathy; Frontotemporal lobar degeneration (FTLD) DefinitionFrontotemporal dementia (FTD) is a rare form of dementia that is similar to Alzheimer disease, except that it tends to affect only certain areas of the brain. Dementia may also be referred to as major neurocognitive disorder. CausesPeople with FTD have abnormal substances (called tangles, Pick bodies, Pick cells, and tau proteins) inside nerve cells in the damaged areas of the brain. The exact cause of the abnormal substances is unknown. Many different abnormal genes have been found that can cause FTD. Some cases of FTD are passed down through families. FTD is rare. It can occur in people as young as 20. But it usually begins between ages 40 and 60. The average age at which it begins is 54. SymptomsThe disease gets worse slowly. Tissues in parts of the brain shrink over time. Symptoms such as behavior changes, speech difficulty, and problems with thinking occur slowly and get worse. Early personality changes can help health care providers tell FTD apart from Alzheimer disease. (Memory loss is often the main, and earliest, symptom of Alzheimer disease.) People with FTD tend to behave the wrong way in different social settings. The changes in behavior continue to get worse and are often one of the most disturbing symptoms of the disease. Some persons have more difficulty with decision-making, complex tasks, or language (trouble finding or understanding words or writing). There are variations of FTD seen with other nervous system problems such as:
General symptoms include: BEHAVIORAL CHANGES:
EMOTIONAL CHANGES
LANGUAGE CHANGES
NERVOUS SYSTEM PROBLEMS
OTHER PROBLEMS Exams and TestsYour provider will ask about the medical history and symptoms. Tests may be ordered to check for other causes of dementia, including dementia due to metabolic causes. FTD is diagnosed based on symptoms and results of tests, including:
Genetic tests can find mutations known to cause FTD and can support the diagnosis. A brain biopsy can also confirm the diagnosis, although it's usually never recommended. TreatmentThere is no specific treatment for FTD. Medicines may help manage mood swings. Sometimes, people with FTD take the same medicines used to treat other types of dementia. In some cases, stopping or changing medicines that worsen confusion or that are not needed can improve thinking and other mental functions. Such medicines include:
It is important to treat any disorders that can cause confusion. These include:
Medicines may be needed to control aggressive, dangerous, or agitated behaviors. Behavior modification can help some people control unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (when it is safe to do so). Talk therapy (psychotherapy) does not always work. This is because it can cause further confusion or disorientation. Reality orientation, which reinforces environmental and other cues, may help reduce disorientation. Depending on the symptoms and severity of the disease, monitoring and help with personal hygiene and self-care may be needed. Eventually, there may be a need for 24-hour care and monitoring at home or in a special facility. Family counseling can help the person cope with the changes needed for home care. Care may include:
People with FTD and their family may need to seek legal advice early in the course of the disorder. Advance care directive, power of attorney, and other legal actions can make it easier to make decisions regarding the care of the person with FTD. Support GroupsYou can ease the stress of FTD by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone. More information and support for people with FTD and their families can be found at: The Association for Frontotemporal Degeneration -- www.theaftd.org/get-involved/in-your-region/ Outlook (Prognosis)The disorder steadily becomes worse. The person becomes totally disabled early in the course of the disease. FTD commonly causes death within 8 to 10 years, usually from infection, or sometimes because other body systems fail. When to Contact a Medical ProfessionalContact your provider or go to the emergency room if mental function gets worse. PreventionThere is no known prevention. ReferencesBudson AE, Solomon PR. Other disorders that cause memory loss or dementia. In: Budson AE, Solomon PR, eds. Memory Loss, Alzheimer's Disease, and Dementia. 3rd ed. Philadelphia, PA: Elsevier; 2022:chap 17. Knopman DS. Cognitive impairment and dementia. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 371. Paulsen JS, Gehl C. Neuropsychology. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 44. Peterson R, Graff-Radford J. Alzheimer disease and other dementias. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 95. | |
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Review Date: 3/31/2024 Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | |
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