Frontotemporal dementia is an umbrella term for a group of uncommon brain disorders that primarily affect the frontal and temporal lobes of the brain. These areas of the brain are generally associated with personality, behavior and language.

In frontotemporal dementia, portions of these lobes shrink (atrophy). Signs and symptoms vary, depending on which part of the brain is affected. Some people with frontotemporal dementia have dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use language properly.

Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease. But frontotemporal dementia tends to occur at a younger age than does Alzheimer's disease. Frontotemporal dementia often begins between the ages of 40 and 65.

Signs and symptoms of frontotemporal dementia can be different from one individual to the next. Signs and symptoms get progressively worse over time, usually over years.

Clusters of symptom types tend to occur together, and people may have more than one cluster of symptom types.

Behavioral changes

The most common signs of frontotemporal dementia involve extreme changes in behavior and personality. These include:

• Increasingly inappropriate social behavior
• Loss of empathy and other interpersonal skills, such as having sensitivity to another's feelings
• Lack of judgment
• Loss of inhibition
• Lack of interest (apathy), which can be mistaken for depression
• Repetitive compulsive behavior, such as tapping, clapping or smacking lips
• A decline in personal hygiene
• Changes in eating habits, usually overeating or developing a preference for sweets and carbohydrates
• Eating inedible objects
• Compulsively wanting to put things in the mouth

Speech and language problems

Some subtypes of frontotemporal dementia lead to language problems or impairment or loss of speech. Primary progressive aphasia, semantic dementia and progressive agrammatic (nonfluent) aphasia are all considered to be frontotemporal dementia.

Problems caused by these conditions include:

• Increasing difficulty in using and understanding written and spoken language, such as having trouble finding the right word to use in speech or naming objects
• Trouble naming things, possibly replacing a specific word with a more general word such as "it" for pen
• No longer knowing word meanings
• Having hesitant speech that may sound telegraphic
• Making mistakes in sentence construction

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).

Movement-related problems may include:

• Tremor
• Rigidity
• Muscle spasms
• Poor coordination
• Difficulty swallowing
• Muscle weakness
• Inappropriate laughing or crying

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink. In addition, certain substances accumulate in the brain. What causes these changes is usually unknown.

There are genetic mutations that have been linked to frontotemporal dementia. But more than half of the people who develop frontotemporal dementia have no family history of dementia.

Recently, researchers have confirmed shared genetics and molecular pathways between frontotemporal dementia and amyotrophic lateral sclerosis (ALS). More research needs to be done to understand the connection between these conditions, however.

Your risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.

There's no single test for frontotemporal dementia. Doctors look for signs and symptoms of the disease and try to exclude other possible causes. The disorder can be especially challenging to diagnose early because symptoms of frontotemporal dementia often overlap with those of other conditions.

Blood tests

To help rule out other conditions, such as liver or kidney disease, your doctor may order blood tests.

Sleep study

Some symptoms of obstructive sleep apnea (memory and thinking problems and behavioral changes) can be similar to those of frontotemporal dementia. If you also have symptoms of sleep apnea (loud snoring and pauses in breathing while sleeping), your doctor may have you undergo a sleep study to rule out obstructive sleep apnea as a cause of your symptoms.

Neuropsychological testing

Sometimes doctors extensively test your reasoning and memory skills. This type of testing is especially helpful in determining the type 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.
• Fluorodeoxyglucose positron emission tracer (FDG-PET) scan. This test uses a low-level radioactive tracer that's injected into the blood. The tracer can help show areas of the brain where nutrients are poorly metabolized. Areas of low metabolism can show where degeneration has occurred in the brain, which can help doctors diagnose the type of dementia.

There's currently no cure or specific treatment for frontotemporal dementia. Drugs used to treat or slow Alzheimer's disease don't seem to be helpful for people with frontotemporal dementia, and some may worsen the symptoms of frontotemporal dementia. But certain medications and speech therapy can help manage symptoms of frontotemporal dementia.

Medications

• Antidepressants. Some types of antidepressants, such as trazodone, may reduce the behavioral problems associated with frontotemporal dementia. Selective serotonin reuptake inhibitors (SSRIs) — such as citalopram (Celexa), paroxetine (Paxil) or sertraline (Zoloft) — also have been effective in some people.
• Antipsychotics. Antipsychotic medications, such as olanzapine (Zyprexa) or quetiapine (Seroquel), are sometimes used to combat the behavioral problems of frontotemporal dementia. However, these medications must be used with caution in people with dementia due to the risk of serious side effects, including an increased risk of death.

Therapy

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

You'll need to have caregivers, as your condition progresses, to assist with daily life activities, maintain your safety, provide transportation and help with finances. Your doctor will discuss lifestyle changes with you, such as when you may need to stop driving a car or let someone you trust take over your finances.

Regular cardiovascular exercise may help improve your mood and thinking skills.

It may be helpful to make some adjustments in your home to make daily living tasks easier and reduce your chance of injuries, such as removing rugs or raising toilets.

In some cases, caregivers can reduce behavioral problems by changing the way they interact with people with dementia. Ask your loved one's doctor about any available resources that provide training in caring for someone with dementia. Possible changes in interaction include:

• Avoiding events or activities that trigger the undesirable behavior
• Removing negative environmental cues, such as the car keys
• Maintaining a calm environment
• Providing structured routines
• Simplifying daily tasks
• Distracting and redirecting attention from problem behaviors

If you've been diagnosed with frontotemporal dementia, receiving support, care and compassion from people you trust can be invaluable.

Through your doctor or the internet, find a support group for people with frontotemporal dementia. A support group can provide valuable information tailored for your needs as well as a forum that gives you the opportunity to share your experiences and feelings.

For caregivers

Caring for someone with frontotemporal dementia can be challenging and stressful because of the extreme personality changes and behavioral problems that often develop. It may be helpful to educate others about behavioral symptoms and what they can expect when spending time with your loved one.

Caregivers need assistance from family members, friends and support groups, or respite care provided by adult care centers or home health care agencies.

Caregivers should remember to take care of their health, exercise, eat a healthy diet and manage their stress. Participating in hobbies outside the home may help ease some stress.

When a person with frontotemporal dementia requires 24-hour care, most families turn to nursing homes. Plans made ahead of time will make this transition easier and may allow the person to be involved in the decision-making process.

People with 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 doctor may refer you to a doctor trained in nervous system conditions (neurologist) or mental health conditions (psychologist) for further evaluation.

What you can do

Because you may not be aware of all your signs and symptoms, it's a good idea to take a family member or close friend along with you to your doctor's appointment. You may also want to take a written list that includes:

• Detailed descriptions of your symptoms
• Information about medical conditions you've had in the past
• Information about medical conditions of your parents or siblings
• All the medications and dietary supplements you take
• Questions you want to ask the doctor

What to expect from your doctor

In addition to a physical exam, your doctor may check your neurological health by testing things such as your balance, muscle tone and strength. Your doctor might also conduct a brief mental status evaluation to check your memory and thinking skills.