This includes the terms:

• Pick's Disease
• Frontotemporal Dementia
• Progressive Non-Fluent Aphasia
• Semantic Dementia
• Primary Progressive Aphasia

Frontotemporal lobar degeneration (FTLD) is a group of conditions caused by loss of cells in the frontal and temporal lobes of the brain. The main symptoms are a progressive change in personality or behaviour and/or progressive deterioration in language abilities.

It can affect both men and women and usually starts in the 40’s or 50’s. However it can also affect older people, and very rarely younger people.

It was originally described by Arnold Pick in 1892 after whom it was named. Most doctors now prefer to reserve the name ‘Pick’s disease’ for just one of the types of changes in the brain tissue that may be seen with the disease.

FTLD is classically subdivided into three types or variants which are described in more detail below:

• Frontotemporal dementia (FTD) or behavioural variant FTLD
• Progressive non-fluent aphasia (PNFA) or primary progressive aphasia (PPA)
• Semantic dementia (SD)

Confusingly, it should be noted that some doctors will use FTD to mean all three variants and also the term PPA is occasionally used to mean both PNFA and SD.

What causes FTLD?
FTLD is caused by loss of brain cells in the frontal and temporal lobes of the brain. However, the processes that lead to the loss of these brain cells are not well understood. There are a number of different underlying pathological changes that are recognised and several different pathological varieties of FTLD. It is known that there is an abnormal accumulation in the brain cells of certain proteins (these include the proteins known as ‘TDP-43’, ‘Ubiquitin’, and ‘Tau’) but we do not yet fully understand why this accumulation occurs, or how it leads to the loss of cells.

In somewhere between 30 and 50% of cases a person with the disease may have a family history of the disease in one of their parents, brothers or sisters. In these cases the cause may be genetic. Mutations (faults) in the tau gene or the progranulin gene on chromosome 17 are the cause in some of these cases, but not all abnormal genes have been discovered yet. Genetic testing is available in some centres. Two other genes known as VCP and CHMP2B are extremely rare causes of FTLD.

Links to other diseases
In a small number of patients FTLD can overlap with one of a number of diseases that affect movement of the body: motor neurone disease (MND), progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD). Symptoms of MND can include weakness of the limbs or problems with swallowing. PSP causes problems with movements of the eyes as well as problems with thinking and behaviour. There may also be other physical problems such as falls, difficulty walking and stiffness. See below for more details about CBD. Some patients with FTLD will later develop symptoms similar to Parkinson’s disease such as slowing of movements, tremor and stiffness of the limbs.

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