Primary progressive aphasia is a frontotemporal disorder marked by progressive losses in language ability, which includes speaking, comprehending, reading and writing. The word aphasia is a general term used to refer to deficits in language functions.
PPA is a neurological syndrome caused by shrinking of the frontal, temporal or parietal lobes in the brain, primarily on the left side. PPA affects each individual differently. It is not Alzheimer's disease.
The most common cause of dementia is Alzheimer’s disease, a chronically progressive brain disorder that impairs memory, intellect and behavior. Daily activities become compromised.
Those with PPA have difficulties finding words, abnormal speech patterns and major spelling difficulties. The ability to write may be less impaired than the ability to speak. Some with PPA develop agrammatism, using inappropriate word order, misusing word endings, prepositions, pronouns, conjunctions and word tenses.
Language issues may be the only symptoms of PPA, lasting a decade or longer. Although other cognitive impairments may emerge, the language deficits remain a primary feature throughout the illness and progresses more rapidly than deficits in other areas.
In contrast to Alzheimer’s disease, in which most affected individuals lose their creative skills or interest in them, many with PPA tend to maintain and even intensify their involvement and creativity in various hobbies such as painting, gardening or carpentry.
PPA is more common in adults under 65, though anyone can develop it.
Mental skills and memory become impaired, and some begin to have additional neurological conditions. As PPA worsens, the person will eventually need assistance with activities of daily living and day-to-day care. About 50% of people with PPA will eventually develop cognitive or behavioral problems consistent with a more invasive dementia syndrome, such as Alzheimer's disease or frontotemporal dementia, after an average of five years.
However, in some cases, aphasia may remain relatively isolated or even be the sole condition for as long as 15 years. In general, the longer the duration of aphasia as an isolated symptom, the less likely that other signs of dementia will develop.
About half of PPA-affected individuals have a family history of dementia, indicating the existence of a genetic component.
Once PPA is diagnosed, the primary goal of treatment is to improve the ability to communicate with interventions and speech therapies that can help the patient have a good quality of life.
There are no specific medications for this disorder. However, because of the 30% to 40% probability of Alzheimer's disease, doctors may prescribe familiar Alzheimer's medications such as Aricept, Namenda or Exelon, though none of these drugs has been shown to improve PPA.
As anxiety or depression occur later in the disorder, medications may be prescribed to manage those symptoms.