Frontotemporal dementia

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Frontotemporal dementia is an umbrella term for a group of rare disorders that primarily affect the areas of the brain associated with personality and behaviour.

Société Alzheimer Society

Overview

Frontotemporal dementia (sometimes referred to as FTD) is an umbrella term for a group of rare disorders that primarily affect the frontal and temporal regions of the brain – the areas generally associated with personality and behaviour.

Researchers estimate that approximately 5-10% of all dementia cases are frontotemporal dementia. However, when symptoms of dementia start before the age of 65 (known as young onset dementia), approximately 20% of cases are frontotemporal dementia.

Types

The most common frontotemporal disorder is behavioural variant frontotemporal dementia (bvFTD). Common symptoms of bvFTD include changes in personality, behaviour and judgement. A person with frontotemporal dementia may also experience language difficulties, known as primary progressive aphasia.

Other names often used for frontotemporal dementia include:

  • Semantic dementia,
  • Frontotemporal lobar degeneration,
  • Frontal lobe dementia,
  • Tau disease or taupathy and
  • Pick's disease and Pick's complex.

Frontotemporal dementia is also related to diseases that affect movement, such as atypical forms of parkinsonism (like corticobasal degeneration and progressive supranuclear palsy) and Amyotrophic Lateral Sclerosis (ALS).

Symptoms

Frontotemporal dementia can cause behaviour changes, difficulties with speech and movement, or memory loss.

Since the frontal (near the forehead) and temporal (near the ears) areas of the brain can be affected, early symptoms can be related to behaviour or speech.

In the early stage of frontotemporal dementia, behaviour changes or problems with speech can appear separately. However, as the disease progresses, these symptoms will increasingly overlap.

Unlike in Alzheimer’s disease, people with frontotemporal dementia often remain conscious of time (for example, what year it is) and memory is not of concern in the early stages.

In the later stages, general symptoms of dementia arise, including confusion and forgetfulness. Motor skills are lost and swallowing difficulties occur.

Changes to behaviour

When experiencing symptoms of frontotemporal dementia, people can display:

  • Withdrawn or disinhibited behaviour (e.g. losing the ability to restrain your actions),
  • Loss of interest in personal hygiene,
  • Tendency to become easily distracted, or to repeat the same activities continuously,
  • Overeating, or an unusual desire to put foreign objects in the mouth,
  • Incontinence, or inability to wait to go to the toilet.

Other possible behavioral changes can include:

  • Inappropriate social actions (making rude comments or seeming tactless),
  • Becoming easily distracted,
  • Drastic changes in food preference,
  • Blunted emotions,
  • Decrease in energy and motivation,
  • Changes in personality (for example, quieter people becoming more extroverted).

Changes to speech

Speech-related changes can include:

  • Speaking less,
  • Total loss of speech,
  • Difficulty finding the right words or describing yourself,
  • Repeating what has already been said by others,
  • Stuttering,
  • Difficulty sustaining a train of thought or maintaining a conversation.

Diagnosis

Although no single test can diagnose frontotemporal dementia, assessment is important to rule out other problems. For example, frontotemporal dementia is often misdiagnosed as depression because they share similar symptoms.

Doctors typically diagnose frontotemporal dementia by identifying a series of characteristic features, while ruling out other possible causes.

Measures that assist in diagnosis include:

  • Cognitive (thinking) tests: to assess which brain functions are affected.
  • Recent history may be documented: with insight from the person, their family and caregivers, to get a detailed picture of what has been happening.
  • Brain imaging: like an MRI for example, which can be helpful in making a diagnosis of frontotemporal dementia.

Risk factors

Little is known about the cause of frontotemporal dementia and risk factors have yet to be identified.

Most cases are not inherited. However, there is one extremely rare type of frontotemporal dementia which can be passed from generation to generation, known as familial frontotemporal dementia.

Treatment

At present, there is no known cure and no effective way to slow the progression of frontotemporal dementia.

Frontotemporal dementia affects different areas of the brain than Alzheimer's disease so available dementia medications (which are primarily used to treat Alzheimer's disease) do not work for the treatment of frontotemporal dementia.

It is important to learn as much as possible about the disease, to help understand and respond to the person’s new behaviours. This will help with symptom management.

If you’re a carer or a family member, you will need to seek support and learn coping strategies to work around the person’s behaviour, rather than trying to get the person to change. Speech pathologists may be helpful in teaching strategies to work around communication problems.

More useful links and resources

https://vimeo.com/40104824

Frontotemporal Dementia: Facing the Challenges for Patients, Family Members, and Clinicians. brainXchange, 2012. This presentation describes some of the challenges associated with diagnosing frontotemporal dementia, with a focus on the importance of interprofessional team collaboration. It also describes the value of unique tele-health support groups, and lessons learned from caregivers with experience of frontotemporal dementia.

https://vimeo.com/290352522

Behavioural variant frontotemporal dementia. brainXchange, 2018. In this presentation, you’ll learn about the criteria physicians use to diagnose behavioural variant frontotemporal dementia (bvFTD), the basics of its pathology, genetics, and treatment options.

https://www.theaftd.org/wp-content/uploads/2009/02/Final-FTD-Genetics-Brochure-with-Cover-8-2-2012.pdf

Understanding the Genetics of FTD: a guide for patients and their families. The Association for Frontotemporal Degeneration. This comprehensive booklet provides additional insight into pathology, treatments, types of FTD, the role of genetics, FAQs, and a detailed glossary of terms.

https://www.theaftd.org/wp-content/uploads/2009/03/What-About-the-Kids-web-version.pdf

What about the kids? Frontotemporal degeneration: Information for parents with young children and teens, The Association for Frontotemporal Degeneration. This resource offers information and guidance to assist parents in describing the concept of FTD to children and young adults.

https://www.theaftd.org/wp-content/uploads/2009/03/AFTD-40-pg-booklet-NewDiag_Website.pdf

The doctor thinks its FTD. Now what? A guide for managing a new diagnosis (FTD), The Association for Frontotemporal Degeneration. If you have recently received a diagnosis of FTD—or a loved one has received a diagnosis—this short booklet offers practical information to support you through the early stages of your experience.

https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/symptoms-causes/syc-20354737

Frontotemporal dementia, The Mayo Clinic. The Mayo Clinic provides an overview of risk factors and symptoms associated with frontotemporal dementia.

https://cchsa-ccssma.usask.ca/ruraldementiacare/

Improving the care of persons with dementia in rural and remote areas, Rural Dementia Action Research. This content offers high-level strategies for improved care for people with dementia in remote communities.

Contact your local Alzheimer Society to find out about more resources.