Frontotemporal Dementia

A dementia that causes changes to language and behaviour

Also known as frontotemporal lobal degeneration, or frontal lobe disorder, and previously known as Pick’s disease, these terms refer to all dementias that affect the frontal and temporal lobes of the brain, which are responsible for language skills, emotional responses, and behaviour.

In frontotemporal dementia, damage to nerve cells in one or both lobes causes the lobe to shrink (atrophy). Depending on the lobe affected, this can cause significant changes to personality or cause the person to lose language.

This type of dementia differs from most other types of dementia in that the onset of symptoms usually starts between the ages of 40 and 75, rather than over 65 years of age. Frontotemporal dementia is rarer than the most common forms of the dementia; Alzheimer’s disease and vascular dementia.

Types of frontotemporal dementia

Depending on the area affected, people with frontotemporal dementia may have one of three types:

  • Behavioural variant
  • Semantic dementia
  • Progressive non-fluent aphasia

Behavioural variant
Most people with frontotemporal dementia are diagnosed with this type, which is characterised by major changes in personality and behaviour in the early stages.

Early symptoms:

  • Reduced initiative and increased apathy
  • Increasingly inappropriate and uninhibited behaviours
  • Repetitive or compulsive behaviours
  • Reduced personal hygiene
  • Loss of empathy and sympathy
  • Overeating
  • Lack of awareness of these changes

Semantic dementia
Symptoms for this type of frontotemporal dementia often come on slowly, sometimes over years. It is most noticeable in speech.

Early symptoms:

  • Trouble understanding words
  • Inability to recognise familiar people or objects
  • Not using joining words such as ‘to’,’ from’, or ‘the’
  • Problems finding the right words while speaking
  • Conversation that is grammatically correct but unrelated to the conversation topic

Progressive non-fluent aphasia
The least common of types of frontotemporal dementia, progressive non-fluent aphasia gradually affects a person’s ability to speak fluently.

Early symptoms:

  • Slow and difficult enunciation of words
  • Difficulties spelling
  • Tendency to use the wrong word
  • Slurred speech

Progression

Each person with frontotemporal dementia will have a different experience of the illness, as symptoms can vary from person to person, hour to hour.

As frontotemporal dementia progresses, the symptoms between the three types become harder to distinguish, as the disease moves to other parts of the brain. People with moderate or severe frontotemporal dementia may experience:

  • Profound behaviour problems including apathy, disinhibition, and loss of empathy
  • Severe language difficulty or loss
  • Memory loss
  • Difficulty recognising friends and family
  • Weight gain due to overeating
  • Loss of movement, requiring a wheelchair

At the end stage of frontotemporal dementia, 24 hour care is usually required because of the physical decline making eating, moving, using the toilet, showing, and dressing difficult. Life expectancy is around five to ten years, but will differ from person to person.

Some people move into a special dementia wing at a residential aged care facility.

Causes

Genetics plays a stronger role in frontotemporal dementia than other dementias, with around 50 per cent of people having a family history of the disease. This does not mean that if your parents have frontotemporal dementia that you will develop it.

A variety of mutations of specific genes have been found to cause different subtypes of frontotemporal dementia, however half of the people with this form of dementia have no family history of the disease.

Frontotemporal dementia can be caused by accumulation of one of two different proteins

  • Tau protein
  • TDP-43 protein

In some cases, the affected part of the brain contains abnormal tau protein filled structures that develop within brain cells. These are known as Pick bodies. While frontotemporal dementia used to be known as Pick’s disease, this term is now reserved for the subtype that actually features these microscopic abnormal structures.

Treatment

There are no current cures for frontotemporal dementia, however there are a range of therapies that can assist with symptoms, such as:

Support Services

A number of support services are available to Queenslanders living with frontotemporal dementia, their families, and carers. These include:


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