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What is Frontotemporal Dementia?

Posted Oct 05 2009 10:01pm


Frontotemporal dementia (FTD) is a cluster of progressive diseases that affect the regions of the brain that control personality, behavior, language, and decision-making.

Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease. Frontotemporal dementia tends to occur at a younger age than does Alzheimer's disease, typically between the ages of 40 and 70.

More than half the people who develop frontotemporal dementia have no family history of dementia.

Frontotemporal dementia was once known as Pick's disease.

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FTD is a disease of behavior and language dysfunction, while the hallmark of Alzheimer’s disease is loss of memory.

Some people with frontotemporal dementia undergo dramatic changes in their personality and become socially inappropriate, impulsive or emotionally blunted, while others lose the ability to use and understand language.

Often, FTD patients exhibit behavioral and personality changes like lack of concern for social norms or other people. FTD patients often lack insight into their own behaviors; but at the same time, retain features of memory like keeping track of day-to-day events and orientation to space and time. This is the different than patients with Alzheimer's disease who suffer from a persistent loss of memory, but usually maintain their social skills. Memory loss is often the main, and earliest, symptom of Alzheimer's. Symptoms such as behavior changes, speech difficulty, and impaired thinking are the hallmarks of FTD.

Symptoms

Frontotemporal dementia is extremely difficult to diagnose. The signs and symptoms may vary greatly from one individual to the next.

Researchers have identified clusters of symptoms that tend to occur together and be dominant in in most subgroups of people with the disorder. More than one symptom cluster may be apparent in the same person.

Behavioral changes

The most common signs and symptoms of frontotemporal dementia involve extreme changes in behavior and personality. These include:
  • Increasingly inappropriate actions
  • Euphoria
  • Lack of judgment and inhibition
  • Apathy
  • Repetitive compulsive behavior
  • A decline in personal hygiene
  • Lack of awareness of thinking or behavioral changes

Speech and language problems

Some subtypes of frontotemporal dementia are marked by the impairment or loss of speech and linguistic abilities. For example, primary progressive aphasia is characterized by an increasing difficulty in using and understanding written and spoken language. People with another subtype, semantic dementia, utter grammatically correct speech that has no relevance to the conversation at hand.

Movement disorders

Rarer subtypes of frontotemporal dementia are characterized by problems with movement, similar to those associated with Parkinson's disease or amyotrophic lateral sclerosis (ALS) — which is also often called Lou Gehrig's disease.

Movement-related signs and symptoms may include:
  • Tremor
  • Rigidity
  • Muscle spasms
  • Poor coordination
  • Difficulty swallowing
  • Muscle weakness
Patient Awareness

People who have frontotemporal dementia often don't recognize that they have a problem. In many cases, family members are the ones who notice the symptoms and arrange for a doctor's appointment. Your family physician should refer you to a neurologist or psychologist for further evaluation if he suspects FTD.

What to expect from your doctor

In addition to a physical exam, your doctor may also check neurological health by testing :
  • Reflexes
  • Muscle strength
  • Muscle tone
  • Senses of touch and sight
  • Coordination
  • Balance
During the appointment, your doctor might also conduct a brief mental status evaluation, which may assess:
  • Memory
  • Problem-solving abilities
  • Attention span
  • Counting skills
  • Language usage
Tests and diagnosis

No single test can identify frontotemporal dementia, so doctors need to identify certain characteristic features while ruling out other possible causes.

Blood tests
To see if your symptoms are being caused by some systemic illness, your doctor should order blood work to check your:
  • Complete blood count
  • Electrolytes, such as sodium and potassium levels
  • Liver function
  • Kidney function
  • Thyroid function
  • Neuropsychological testing

Sometimes doctors undertake a more extensive assessment of reasoning and memory skills. This type of testing, which can take several hours to complete, is especially helpful in trying to differentiate between the different types of dementia at an early stage.

Brain scans

By looking at images of the brain, doctors may be able to pinpoint any visible abnormalities — such as clots, bleeding or tumors — that may be causing signs and symptoms.

Magnetic resonance imaging (MRI)

An MRI machine uses radio waves and a strong magnetic field to produce detailed images of your brain.  The entire procedure can take an hour or more.

Computerized tomography (CT)

X-rays pass through your head from various angles, and a computer uses this information to create cross-sectional images, or slices, of your brain. The test is painless and takes about 20 minutes.

PET (Positron Emission Tomography), SPECT (Single Photon Emission Computed Tomography), and Functional MRI (fMRI) scans are might also be used.

Treatments and Drugs

There is no cure for frontotemporal dementia and no effective way to slow its progression. Treatment relies on managing the symptoms.

Medications
  • Antidepressants. Some types of antidepressants, such as trazodone, may reduce the behavioral problems associated with frontotemporal dementia. Selective serotonin reuptake inhibitors (SSRIs) have also been effective in some cases, although study results have been mixed.
  • Antipsychotics. Although antipsychotic drugs are sometimes used to combat the behavioral problems of frontotemporal dementia, side effects can include an increased risk of mortality in older people.

Therapy

People experiencing language difficulties may benefit from speech therapy, to learn alternate strategies for communication.

Also see Dementia and the Right Types of Dementia


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Bob DeMarco is the editor of the Alzheimer's Reading Room and an Alzheimer's caregiver. The Alzheimer's Reading Room is the number one website on the Internet for news, advice, and insight into Alzheimer's disease. Bob has written more than 800 articles with more than 18,000 links on the Internet. Bob resides in Delray Beach, FL.

Original content Bob DeMarco, Alzheimer's Reading Room
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