Get to Know Frontotemporal Dementia and One Type of Dementia

Bumisuka.com – Get to Know Frontotemporal Dementia and One Type of Dementia. Frontotemporal dementia is an umbrella term for a group of brain disorders that primarily affect the frontal and temporal lobes of the brain. This area of the brain is generally associated with personality, movement, behavior, and language.

Dementia mostly affects people over the age of 65, but frontotemporal dementia tends to start at a younger age. Most cases are diagnosed in people aged 45–65 years, although it can affect younger or older people as well.

Like other types of dementia, frontotemporal dementia tends to develop slowly and get worse gradually over several years.

1. Type

Described on the Alzheimer’s Society website, there are two types of frontotemporal dementia, namely:

• Behavioral variant frontotemporal dementia: Damage to the frontal lobes of the brain primarily causes problems with behavior and personality. This lobe is found behind the forehead and processes information that influences how we behave and control our emotions. They also help us plan, solve problems, and focus long enough to complete tasks.
• Primary progressive aphasia: Occurs when damage to the temporal lobes—on both sides of the head closest to the ears—causes language problems. This part of the brain has many roles. The key function of the left temporal lobe is to store the meaning of words and names of objects. The right temporal lobe helps most people recognize familiar faces and objects.

2. Cause

Reported by the National Institute of Aging, scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to frontotemporal dementia brain scans.

Scientists describe frontotemporal dementia using patterns of changes in the brain seen in postmortem autopsies. These changes include loss of neurons and abnormal numbers, or forms of proteins called tau and TDP-43. These proteins occur naturally in the body and help cells function properly. When the protein doesn’t work properly, for reasons not yet fully understood, neurons in certain brain areas are damaged.

In most cases, the cause of frontotemporal dementia is unknown. Individuals with a family history of frontotemporal dementia are more likely to develop such a disorder. About 10 to 30 percent of behavioral variant frontotemporal dementia is due to some genetic cause.

Frontotemporal dementia that runs in families is often associated with mutations in certain genes. Genes are the basic units of heredity that tell cells how to make the proteins the body needs to function. Even small changes in genes can produce abnormal proteins, which can lead to changes in the brain and, eventually, disease.

Scientists have discovered several different genes that, when mutated, can cause frontotemporal dementia:

• Tau gene (also called MAPT gene): Mutations in this gene cause abnormalities in a protein called tau, which then causes kinks in neurons and eventually damages brain cells. Inheriting a mutation in this gene means that a person will almost certainly develop a frontotemporal disorder, usually a behavioral variant of frontotemporal dementia, but the age of onset and the exact symptoms are unpredictable.
• GRN gene: Mutations in this gene can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to cause errors in brain cells. Many frontotemporal disorders can occur, although the behavioral variant of frontotemporal dementia is the most common. The GRN gene can cause different symptoms in different family members and cause the disease to start at different ages.
• C9ORF72 gene: An unusual mutation in this gene appears to be the most common genetic disorder in familial frontotemporal disorder and familial amyotrophic lateral sclerosis (ALS). These mutations can cause frontotemporal disorder, ALS, or both.

In recent years researchers have found several other genetic mutations in the gene that cause a rare type of familial frontotemporal disorder. These other mutations account for less than 5 percent of all cases of frontotemporal dementia.

3. Symptoms

The first symptoms seen in people with frontotemporal dementia are changes in personality and behavior and/or language difficulties. This is very different from the early symptoms of more common types of dementia. For example, in Alzheimer’s disease, early changes are often problems with everyday memories. In the early stages of frontotemporal dementia, many people can still remember current events.

The signs and symptoms of frontotemporal dementia vary from person to person and the order in which they appear may also vary. Quoting Alzheimers.gov, changes in the frontal lobes of the brain are generally associated with behavioral symptoms and can also cause movement symptoms. Changes in the temporal lobe commonly cause language and emotional disturbances.

Symptoms of frontotemporal dementia and related disorders can include:

• Decreased energy and motivation.
• Lack of interest in other people.
• Inappropriate and impulsive behavior.
• Not acting with consideration for others.
• Repeating activities or words over and over again.
• Changes in food preferences and compulsive eating.
• Increased interest in sex.
• Ignoring personal hygiene.
• Flat emotions or exaggerated emotions.
• Difficulty making or understanding speech.
• Inability to perform common movements, such as using a fork.
• Problems with balance and walking.
• Increasing awkwardness.
• Slow motion, falling, stiff body.
• Limited eye movement.
• Hands shaking.
• Muscle weakness and loss, subtle jerks, swaying muscles.

4. Diagnosis

Frontotemporal dementia because its symptoms mimic other conditions. For example, frontotemporal dementia is sometimes misdiagnosed as a mood disorder, such as depression. Even more confusing, a person can have frontotemporal dementia and other types of dementia, such as Alzheimer’s disease. Also, because this disorder is rare, doctors may not be familiar with its signs and symptoms.

To help diagnose frontotemporal dementia, doctors may:

• Do an examination and ask about symptoms.
• Evaluate personal and family medical history.
• Using laboratory tests to help rule out other conditions.
• Order genetic testing.
• Take tests to assess memory, thinking, language skills, and physical functioning.
• Order brain imaging.

A psychiatric evaluation can help determine whether depression or another mental health condition is causing or contributing to the condition. Only genetic testing in familial cases or a brain autopsy after a person has died can confirm a diagnosis of frontotemporal dementia.

Several researchers are studying ways to diagnose frontotemporal dementia earlier and more accurately, and differentiate it from other types of dementia. One area of research involves biomarkers, such as proteins or other substances in blood or cerebrospinal fluid that can be used to measure disease progression or the effect of treatment. Researchers are also exploring ways to improve brain imaging and neuropsychological testing.

5. Treatment

According to the National Health Service, there is currently no cure for frontotemporal dementia or treatments that help slow the progression of the disease. However, there are treatments that can help control some of the symptoms, possibly for several years.

Treatment may include:

• Medications: To control some behavioral problems.
• Therapy: For example physiotherapy, occupational therapy, speech therapy, language therapy for problems with movement, daily tasks and communication.
• Dementia activities: For example memory cafes, which are informal local meeting sessions for people with memory problems and their caregivers for support and advice.
• Support groups: These can provide and share tips about managing symptoms from dementia professionals and people living with frontotemporal dementia, and their families.

Frontotemporal dementia is a long-term condition that ultimately affects the ability to control one’s own behavior, or to speak and understand other people who speak to them.

Over time, this condition will interfere with the ability to think, care for yourself, and live independently. Because the effects can be severe, people living with frontotemporal dementia may want to consider getting their wishes or treatment instructions in writing as soon as possible.

Unfortunately, frontotemporal dementia has no cure, and there is no standard treatment. Sometimes it is possible to treat the symptoms, but this varies from person to person. Finally, most people with this condition need 24/7 care from a healthcare professional.