About Alzheimer's

Frontotemporal Dementia

Understanding Alzheimer's >> An Alzheimer's Overview | 03.18.15

What is Frontotemporal Dementia?

Frontotemporal Dementia (FTD) is the name of a group of rare neurodegenerative disorders caused by nerve cell damage in the frontal and temporal lobes of the brain. These are the areas of the brain known to affect personality, behavior and language.

Often misdiagnosed as Alzheimer’s Disease or a psychiatric condition, FTD tends to occur in younger people than those susceptible to Alzheimer’s, usually appearing between the ages of 40 and 60.  The symptoms grow progressively worse over time and life expectancy after onset is only 8 years.

The following subsets of Frontotemporal Dementia have been identified:

Frontal variant of FTD (fvFTD)

Once known as Pick’s disease, fvFTD is characterized by changes in personality and behavior.  Persons with this disorder can lack empathy with others and behave inappropriately or aggressively.  Obsessive behaviors with no meaningful purpose may be observed.  Those with this condition usually aren’t aware of their erratic behavior, so they typically refuse to seek medical intervention.

Primary progressive aphasia (PPA)

PPA is characterized by a person having difficulty in using and understanding language, both written and spoken.  The condition worsens over time.

Progressive nonfluent aphasia (PNFA)

People with PNFA speak slowly and have trouble using the right words when talking. They may find it difficult to name familiar objects and experience memory problems as well.

Semantic dementia (SD)

Those with semantic dementia can speak correctly, but the content of their speech has no relevance.  As with PPA and PNFA, these people may have difficulty understanding language and recalling the names of common objects.

FTD has also been linked genetically to the following diseases of the nervous system:

Progressive Supranuclear Palsy (PSP)

Corticobasal degeneration (CBD)

Amyotrophic Lateral Sclerosis (ALS)

Common signs and symptoms of FTD

Because FTD involves personality changes, it can be confused with other psychiatric conditions, such as mania, depression or obsessive-compulsive disorder.  Here are some of the symptoms which may signal the presence of FTD.

Cognitive symptoms

Diminished attention

Lack of focus

Decline in mental flexibility

Decreased judgment

Speech disorders

Disorientation (late stage)

Poor memory (late stage)

Psychiatric symptoms

Depression

Elevated mood or euphoria

Apathy

Loss of empathy/interpersonal skills

Lack of judgment and inhibition, with increasingly inappropriate actions

Repetitive compulsive behaviors

Changes in eating and drinking habits, predominantly overeating

Decline in personal hygiene

Neurological symptoms

Poor coordination

Rigidity

Tremors

Muscle spasms

Incontinence

Difficulty swallowing

Muscle weakness

How is FTD diagnosed?

Tests for Frontotemporal Dementia would involve the following: a detailed medical exam, including patient history; appropriate laboratory tests; neuropsychological tests to detect impaired problem-solving skills; and brain imaging to look for shrinkage in the frontal and temporal lobes.

What causes FTD?

Like other neurodegenerative dementias, researchers believe protein deposits in the brain are a contributing factor in the occurrence of Frontotemporal Dementia. For instance, in the Frontal variant of FTD, deposits of tau protein known as Pick bodies have been found in nerve cells.

Gene mutations have also been linked to specific types of FTD, suggesting a strong genetic influence.  Children of people with this mutation have a 50% risk of developing the disease.  However, more than half of the people who develop FTD have no family history of dementia.

How is FTD treated?

There is no cure for FTD and no way to slow its progression.  Management of symptoms is the primary treatment strategy.  In the case of agitation and aggressive behavior, antipsychotic drugs may be prescribed. Antidepressants can have value as well to help treat behavioral issues such as apathy and depression.

 

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