Within PPA three main patterns of language loss are recognised:
There is a support group specifically for those affected by Primary Progressive Aphasia. To find out more, please visit the Primary Progressive Aphasia Support Group page (opens new window).
The first symptoms of SD are usually problems with language. These may include:
Problems with language increase over time. There is a slow but progressive loss of vocabulary and the ability to understand what people are saying. Speech becomes increasingly vague and the quantity of speech tends to diminish.
With progression of the disease, changes in personality are more common in SD than the other types of PPA. These may include the development of a sweet tooth or other changes in eating, becoming obsessive, and disinhibited behaviour.
Later in the illness non-language functions become affected. In particular, the person may have increasing difficulty recognising familiar people or household items. There are also increasing problems carrying out normal daily living activities.
There is no single test that allows doctors to make a diagnosis of SD. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the temporal lobe, usually more on the left than the right.
The first symptoms of PNFA are usually difficulties producing speech. These may include:
As the disease progresses, people may develop other problems including:
As with SD, there is no single test that allows doctors to make a diagnosis of PNFA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the speech areas of the brain, particularly the frontal lobe on the left side.
In a small number of people PNFA is caused by a genetic problem. The genes that are known to cause problems are called progranulin (or GRN) and C9orf72.
The main symptoms of LPA are:
There is no single test that allows doctors to make a diagnosis of LPA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. Brain scanning shows loss of brain cells in areas further towards the back of the brain than the other PPA subtypes, particularly the area where the temporal and parietal lobes meet.
Like the other forms of PPA, why LPA develops in some people is unclear. Unlike the other types of PPA, in the majority of people where brain tissue has been examined under the microscope, the same pathology as Alzheimer’s disease has been found. LPA is therefore often thought to be an unusual form of early onset Alzheimer’s disease.
At most specialist clinics you will have a neurological assessment usually followed by a neuropsychological assessment by an experienced team of cognitive neurologists and neuropsychologists. Although these initial assessments can point towards a diagnosis of PPA, an MRI scan of the brain will also help with the diagnosis (see pictures above). In some circumstances a lumbar puncture (spinal tap) may be performed.
There is no cure for PPA at the moment despite research efforts around the world. However, speech therapy to help with communication strategies, particularly in PNFA, is useful. Co-existing depression or behavioural problems can be treated symptomatically.
The symptoms of PPA are caused by loss of brain cells in the frontal and temporal lobes of the brain. However, the processes that lead to this loss are not well understood. It is known that there is an abnormal accumulation in the brain cells of certain proteins (known as TDP-43, and tau). What makes these proteins deposit, why they occur in particular areas and how their deposition leads to brain cell damage is still being not known.
Some people with PNFA have a family history of the same condition or of frontotemporal dementia. Problems (mutations) in genes called progranulin and C9orf72 have been shown to be associated with PNFA. SD and LPA only rarely run in families.
This is a difficult question to answer as there has been very little research into it and it can be extremely variable from person to person. However, we know that from the onset of symptoms many people will live over 10 years and for SD this can be over 15 years.
The PPA support group holds several meetings a year. A newsletter is published and circulated to members between meetings and the group offers the opportunity for contact with other people who have experienced a diagnosis of Primary Progressive Aphasia.
For more information please visit the PPA Support Group website (opens link in a new window) or contact Chris Hardy :