Frontotemporal dementia (Pick's Disease) by
Dr Jonathan Rohrer & Dr. Jason Warren, Dementia Research Centre
Frontotemporal dementia (FTD) is a group of conditions caused by loss of cells mainly in the frontal and temporal lobes of the brain. The main symptoms are a progressive change in personality and behaviour or progressive deterioration in language abilities.
It can affect both men and women and usually starts in the 40’s, 50’s or 60’s. However it can also affect older people, and rarely, even 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.
FTD is classically subdivided into two types:
Behavioural variant FTD (bvFTD)
Primary progressive aphasia (PPA), which includes progressive nonfluent aphasia (PNFA), semantic dementia (SD) and logopenic aphasia (LPA)
What causes FTD?
FTD 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 FTD. 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’, ‘FUS’ 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 is more likely to be genetic. Problems in the tau (or MAPT) gene, progranulin (or GRN) gene or a gene called C9ORF72 are the cause in some of these cases, but not all abnormal genes have been discovered yet. Genetic testing is available in some centres. Other genes known as VCP, TARDP (or TDP-43), FUS and CHMP2B are extremely rare causes of FTD. Testing for these is not generally clinically available.
Links to other diseases
In a small number of patients FTD can overlap with one of a number of diseases that affect movement of the body: motor neurone disease (MND, sometimes called amyotrophic lateral sclerosis, ALS), 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 are often other physical problems such as falls, difficulty walking and stiffness.Even when the diagnosis is not clearly MND, PSP or CBD, some patients with FTD will later develop symptoms similar to Parkinson’s disease such as slowing of movements, tremor and stiffness of the limbs.
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