FTD is subdivided into two types:

1. Behavioural variant FTD (bvFTD), which mainly affects behaviour and personality
2. Primary progressive aphasia (PPA), a group of dementias mainly causing a loss of speech and language abilities. These include progressive nonfluent aphasia (PNFA), and semantic dementia (SD).

The following information will mainly focus on behavioural variant FTD. For more detailed information on the forms of FTD that affect language please visit our Understanding PPA page.

FTD can affect both men and women and usually starts when people are in their 40s, 50s or 60s. However it can also affect older people and sometimes even younger people.

Causes

FTD is caused by the loss of brain cells in the front of the brain, but the processes that lead to the loss of these brain cells are varied and not well understood.

What we do know is that FTD is caused by an abnormal build-up of certain proteins in the brain cells, but we do not yet know why or how this happens.

In around 30-40% of cases a person with FTD may have a family history of the condition in which a parent or sibling has been affected. In these cases the cause of FTD is likely to be genetic. However, it is important to note that most cases of FTD do not have a genetic basis. Visit the familial frontotemporal dementia (fFTD) group page for information and support for families affected by this genetic form of FTD.

In a small number of people, FTD can overlap with one of a number of diseases that affect movement of the body such as motor neurone disease (MND), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). The symptoms of these diseases can occur alongside those of FTD.

Diagnosis and testing

Behavioural variant FTD (bvFTD)

When diagnosing bvFTD, a doctor will assess behaviour and cognitive functions (aspects of thinking) before, in some cases, a more detailed assessment. Brain scans can show the loss of brain cells caused by FTD, but there is no single test that can diagnose FTD with complete reliability.

In some people FTD can be confused with other conditions in which there are problems with behaviour (for example, some psychiatric disorders) and with other dementias. The doctor will often arrange blood tests, lumbar puncture and an MRI to help confirm the diagnosis and to rule out other conditions.

Semantic dementia (SD) and Progressive non-fluent aphasia (PNFA)

There are no single-tests available for SD and PNFA. Diagnosis is usually made using a combination of clinical assessment, psychological testing and a brain scan. For more information on diagnosing and testing SD and PNFA, please visit our Understanding PPA page.

As FTD is rare, many people describe long and frustrating journeys through different medical professionals before receiving a diagnosis.

It is therefore important that healthcare professionals are better informed about FTD and equipped to recognise the symptoms. Rare Dementia Support is working to improve awareness of FTD and other rare dementias and to train healthcare professionals. For more information and resources visit our Learning page.

By increasing awareness and understanding of FTD, we hope to ensure that healthcare professionals and communities are better equipped to support people affected by a diagnosis. Help us by sharing your stories and contacting us at contact@raredementiasupport.org.

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