Frontotemporal dementia
Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; Pick disease; 3R tauopathy; Frontotemporal lobar degeneration (FTLD)Frontotemporal 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
Dementia is a loss of brain function that occurs with certain diseases. It affects one or more brain functions such as memory, thinking, language, j...
Read Article Now Book Mark ArticleAlzheimer disease
Dementia is a loss of brain function that occurs with certain diseases. Alzheimer disease (AD) is the most common form of dementia. It affects memo...
Read Article Now Book Mark ArticleDementia may also be referred to as major neurocognitive disorder.
Neurocognitive disorder
Neurocognitive disorder is a general term that describes decreased mental function due to a medical disease other than a psychiatric illness. Neuroco...
Read Article Now Book Mark ArticleCauses
People 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.
Symptoms
The 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:
- Amyotrophic lateral sclerosis (ALS/Lou Gehrig disease)
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain, brain stem and spinal cord that control voluntary muscle movemen...
Read Article Now Book Mark Article - Primary progressive aphasia
Aphasia
Speech and language impairment may be any of several problems that make it difficult to communicate.
Read Article Now Book Mark Article - Progressive supranuclear palsy
Progressive supranuclear palsy
Progressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain.
Read Article Now Book Mark Article
General symptoms include:
BEHAVIORAL CHANGES:
- Not able to keep a job
- Compulsive behaviors
- Impulsive or inappropriate behavior
- Inability to function or interact in social or personal situations
- Problems with personal hygiene
- Repetitive behavior
- Withdrawal from social interaction
EMOTIONAL CHANGES
- Abrupt mood changes
- Decreased interest in daily living activities
- Failure to recognize changes in behavior
- Failure to show emotional warmth, concern, empathy, sympathy
- Inappropriate mood
- Not caring about events or environment
LANGUAGE CHANGES
- Cannot speak (mutism)
- Decreased ability to read or write
- Difficulty finding a word
- Difficulty speaking or understanding speech (aphasia)
- Repeating anything spoken to them (echolalia)
- Shrinking vocabulary
- Weak, uncoordinated speech sounds
NERVOUS SYSTEM PROBLEMS
- Increased muscle tone (rigidity)
Increased muscle tone
Spasticity is stiff or rigid muscles. It may also be called unusual tightness or increased muscle tone. Reflexes (for example, a knee-jerk reflex) ...
Read Article Now Book Mark Article - Memory loss that gets worse
- Movement/coordination difficulties (apraxia)
- Weakness
OTHER PROBLEMS
- Urinary incontinence
Urinary incontinence
Urinary (or bladder) incontinence occurs when you are not able to keep urine from leaking out of your urethra. The urethra is the tube that carries ...
Read Article Now Book Mark Article
Exams and Tests
Your 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:
Dementia due to metabolic causes
Dementia is loss of brain function that occurs with certain diseases. Dementia may also be referred to as major neurocognitive disorder. Dementia du...
Read Article Now Book Mark Article- Assessment of the mind and behavior (neuropsychological assessment)
- Brain MRI
MRI
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
Read Article Now Book Mark Article - Electroencephalogram (EEG)
Electroencephalogram
An electroencephalogram (EEG) is a test to measure the electrical activity of the brain.
Read Article Now Book Mark Article - Examination of the brain and nervous system (neurological exam)
- Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture
Lumbar puncture
Cerebrospinal fluid (CSF) collection is a test to look at the fluid that surrounds the brain and spinal cord. CSF acts as a cushion, protecting the b...
Read Article Now Book Mark Article - Head CT scan
Head CT scan
A head computed tomography (CT) scan uses many x-rays to create pictures of the head, including the skull, brain, eye sockets, and sinuses.
Read Article Now Book Mark Article - Tests of sensation, thinking and reasoning (cognitive function), and motor function
- Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future
- Positron emission tomography (PET) scan of brain
Positron emission tomography (PET) scan...
A brain positron emission tomography (PET) scan is an imaging test of the brain. It uses a radioactive substance called a tracer to look for disease...
Read Article Now Book Mark Article
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.
Treatment
There 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:
Confusion
Confusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...
Read Article Now Book Mark Article- Analgesics
Analgesics
Over-the-counter (OTC) pain relievers can help relieve pain or lower a fever. Over-the-counter means you can buy these medicines without a prescript...
Read Article Now Book Mark Article - Anticholinergics
- Central nervous system depressants
- Cimetidine
- Lidocaine
It is important to treat any disorders that can cause confusion. These include:
- Anemia
Anemia
Anemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. Different type...
Read Article Now Book Mark Article - Decreased blood oxygen (hypoxia) level
Hypoxia
Cerebral hypoxia occurs when there is not enough oxygen getting to the brain. The brain needs a constant supply of oxygen and nutrients to function....
Read Article Now Book Mark Article - Heart failure
Heart failure
Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...
Read Article Now Book Mark Article - High blood carbon dioxide level
- Infections
- Kidney failure
- Liver failure
- Nutritional disorders
- Thyroid disorders
- Mood disorders, such as depression
Depression
Depression may be described as feeling sad, blue, unhappy, miserable, or down in the dumps. Most of us feel this way at one time or another for shor...
Read Article Now Book Mark Article
Medicines may be needed to control aggressive, dangerous, or agitated behaviors.
Agitated
Agitation is an unpleasant state of extreme arousal. An agitated person may feel stirred up, excited, tense, confused, or irritable.
Read Article Now Book Mark ArticleBehavior 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.
Monitoring and help
Dementia is a loss of cognitive function that occurs with certain diseases. It affects memory, thinking, and behavior.
Read Article Now Book Mark ArticleCare may include:
- Adult protective services
- Community resources
- Homemakers
- Visiting nurses or aides
- Volunteer services
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.
Advance care directive, power of attorn...
When you are very ill or injured, you may not be able to make health care choices for yourself. If you are unable to speak for yourself, your health...
Read Article Now Book Mark ArticleSupport Groups
You 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 Professional
Contact your provider or go to the emergency room if mental function gets worse.
Prevention
There is no known prevention.
References
Budson 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.
Brain - illustration
The major areas of the brain have one or more specific functions.
Brain
illustration
Brain and nervous system - illustration
The nervous system controls the many complicated and interconnected functions of the body and mind. Motor, sensory cognitive and autonomic function are all coordinated and driven by the brain and nerves. As people age, nerve cells deteriorated in number and facility, causing some lessening in function.
Brain and nervous system
illustration
Brain - illustration
The major areas of the brain have one or more specific functions.
Brain
illustration
Brain and nervous system - illustration
The nervous system controls the many complicated and interconnected functions of the body and mind. Motor, sensory cognitive and autonomic function are all coordinated and driven by the brain and nerves. As people age, nerve cells deteriorated in number and facility, causing some lessening in function.
Brain and nervous system
illustration
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.