Frontotemporal Dementia

These are the areas of the brain associated with personality, behaviour and language.

This disease is caused by the death of brain cells in the frontal and temporal lobes of the brain, causing portions of those areas to atrophy or shrink and is a significant cause of dementia in younger people.

The frontal lobes of the brain are located behind the forehead and are associated with personality and behaviour (on the right side) and language (on the left side). The temporal lobes of the brain are responsible for comprehension or the understanding of words (on the left side) and a person’s memory (more on the right side).

FTD tends to occur at a younger age than Alzheimer's disease (typically between 40 and 70 years of age). It’s more common when a person reaches their 60’s but has been known to occur as early as the 30’s. It is the third most common cause of dementia in this age group.

It is generally considered to be more rapidly progressive than Alzheimer’s disease. The average life span is six to eight years from the onset of symptoms. It can be as short as two years when it is associated with ALS (Amyotrophic lateral sclerosis, also referred to as motor neuron disease or Lou Gehrig's disease).

It is often assumed that Frontotemporal Dementia is synonymous with Pick’s disease. This is not true. Pick’s disease just one type of FTD.

FTD has been associated with multiple proteins- Tau, TDP43, FUS etc.

Is it genetic? Can it be passed on to family members?

• A combination of genetic, environmental and lifestyle factors have been suggested by researchers. While a variety of changes on multiple genes have been linked to specific subtypes of frontotemporal dementia, more than half of people who develop frontotemporal dementia have no family history of dementia.
• A person’s risk of developing frontotemporal dementia is higher if they have a family history of dementia. Roughly 10-15 per cent of persons living with the disease have a strong family history of the disease. Typically, in these cases, FTD is inherited from a parent as a defect (mutation) in one of three genes: MAPT, GRN or C9ORF72.
• Currently, there are no other known risk factors.

What changes can I expect?

Unlike Alzheimer’s disease (which commonly presents with memory loss), frontotemporal dementia often shows up as behaviour changes, poor judgment, or language difficulties. The signs and symptoms may vary greatly from one individual to the next. 

Researchers have identified several clusters of symptoms that tend to occur together, and 3 broader subtypes have been identified: Behavioural variant FTD, primary progressive aphasia and FTD with a movement disorder.

Behavioural variant FTD = Behavioural changes

• The most common signs and symptoms of frontotemporal dementia involve extreme changes in behaviour and personality. These include loss of inhibition; loss of empathy; inappropriate speech and behaviour and loss of other interpersonal skills; lack of judgment; repetitive compulsive behaviour; changes in eating habits (craving sweets or compulsive overeating); and an inability to detect and understand emotions in others. This is the most common presentation of FTD. This is also the most common inherited type of FTD.

Primary progressive aphasia = Speech and language problems

• Some subtypes of frontotemporal dementia are evidenced by a loss of speech and language difficulties. For example, this might include increasing difficulty in using and understanding written and spoken the language - known as progressive non-fluent aphasia. This type of language variant of FTD is characterized by effortful, halting speech with poor grammar. This is the most slowly progressing type of FTD. 

People with another subtype, semantic dementia, utter grammatically correct speech that has no meaning (empty speech) or may have difficulty with single-word comprehension.

FTD with movement disorders

• About 10-20 per cent of people with FTD can develop motor symptoms before or after they are diagnosed with dementia, characterized by problems with movement, like those associated with Parkinson's disease or amyotrophic lateral sclerosis (ALS).

The movement disorders usually associated with FTD include Motor neuron disease, progressive supranuclear palsy and corticobasal degeneration

Movement-related signs and symptoms may include tremors, rigidity, poor coordination, difficulty swallowing and muscle weakness.

How is it diagnosed?

• FTD is often misdiagnosed as a psychiatric condition or as Alzheimer's disease. There is no single test for diagnosing FTD with certainty. Doctors attempt to identify certain characteristic features while excluding other possible causes.
• The diagnosis is often difficult because of the absence of memory problems, lack of insight of the person about their personality and behaviour changes and the relatively younger age of the person.
• The diagnostic process includes detailed history from the person and a family member, blood tests, neurological testing, cognitive assessment and brain scans (such as MRI, CT and PET scans). CT & MRI scans help to assess any patterns of damage in the brain. They can also rule out other conditions such as strokes or tumours. Specialized brain scans such as PET (Positron Emission Tomography) and SPECT (Single Photon Emission Computerized Tomography) can be conducted to measure glucose metabolism in the brain and brain activity. These scans may detect reduced glucose metabolism and reduced activity in the frontal and/or temporal lobes at an earlier stage, even before CT or MRI scans might pick up any structural changes.
• When there is a strong family history of FTD, genetic testing may help in confirming the diagnosis and enabling family members to find out their risk of developing the disease.

Is there a cure? What kind of treatment is available?

Unfortunately, there is currently no known cure for frontotemporal dementia and no effective way to slow its progression. Treatment relies on managing the symptoms. Antidepressants form the main approach to treatment. 

Overeating and compulsive behaviour symptoms may respond to the SSRI group of antidepressants such as Citalopram or Escitalopram. Apathy-related symptoms may respond to Venlafaxine, given its activating properties. 

Social disinhibition (inappropriate behaviour) is notoriously difficult to treat with medications. Extremely agitated behaviour that threatens caregiver safety is sometimes treated with atypical antipsychotic drugs such as Quetiapine or Risperidone in low incremental doses. These drugs must be used with extreme caution given the risk of cardiac complications, including sudden death. 

Caring for a person with FTD

Caring for a person with frontotemporal dementia can be challenging and stressful at times because of the personality and behaviour changes experienced. 

A major treatment imperative is to provide the spouse and other caregivers with key information, emotional support, strategies for managing behavioural changes and access to community resources. Without these tools, caregiver burnout is a significant risk and can have major adverse consequences, including poor health outcomes, for both the person with FTD and the caregiver. 

Important Note: Alzheimer Society of Calgary is here to help you.

Workshops: Caregiver Strategies for Dementia (offered every month)

Free support line: Talk to our support team at 403-290-0110

Club 36 Adult Day Program

Caregivers need assistance from family members and friends; support groups and educators; or respite care provided by Adult Day Programs or home health care agencies. 

For persons with language difficulties, a speech and language therapist can help maximize existing skills and help the individual learn alternate ways of communicating. This can also assist the caregiver in developing new ways of connecting. 

Caregivers should engage in exercise, or other stress-reducing activities, prioritize their own sleep and maintain social contacts and personal hobbies to foster a sense of wellbeing.

Day-to-day interactions between the caregiver and person with FTD should focus on redirecting attention away from unwanted preoccupations and activities in a non-confrontational manner. Harmless compulsions within the home need not be discouraged. Unwelcome compulsive behaviours can be replaced by harmless alternatives such as a squeeze ball (to replace touching strangers) or a lollipop (to reduce repetitive, stereotyped vocalizations).

Dietary or other meaningful rewards for desired behaviours such as showering, grooming etc. work with some people. 

The person’s access to financial resources should be controlled and monitored or removed based on the context. 

There are other tools to help.

“The Person I am with has Dementia” card: 

If the person tends to exhibit problematic behaviour in public, carrying a business card-sized explanation that the person has a brain disease that affects behaviour might help defuse socially embarrassing or difficult situations. Check out this and other helpful tools on the Dementia Network Calgary site. http://www.dementianetworkcalgary.ca/print-at-home-tools

Source: www.alzheimers.org.uk

This content is provided for informational purposes only and is not to be used for diagnosis or treatment of any type of dementia or its symptoms. Any mention of pharmaceutical interventions or treatments is not an endorsement by the Alzheimer Society of Calgary. This information is apt to change at any time without notice. For medical advice, please contact your physician.