What is primary progressive aphasia?
Primary progressive aphasia, or PPA, is a frontotemporal disorder characterized by a progressive loss of language abilities that includes speaking, understanding, 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 shrinkage of the frontal, temporal or parietal lobes of the brain, predominantly on the left side. PPA affects each individual differently. PPA is not Alzheimer’s disease.
The highest risk factors for PPA include having a history of learning disabilities and having certain gene mutations. The condition begins gradually and the individual initially begins to have difficulty thinking of common words while speaking or writing. Memory, reasoning, and visual perception are usually not affected in individuals first diagnosed with PPA, so in this respect the individual with the disorder can function quite normally in many daily routine activities. As PPA progresses, verbal communication by any means is difficult and other mental and cognitive abilities eventually decline.
Symptoms of language deficits are first noticed in PPA; however, there are other symptoms to note. The individual may often hesitate while searching for the correct word or if the individual speaks in a disjointed manner such as a sentence with unusual word order. Substitution of words or object names is common in PPA, as is the use of words that are mispronounced or incomprehensible. There is a sudden failure to understand simple words, and although they can recognize other people, the individual with PPA is unable to think of their names. Difficulty with writing is prevalent in PPA, such as checks or correspondence, as well as difficulty reading and following simple written instructions. Individuals with PPA also struggle with calculations and arithmetic, such as making change, adding money, or leaving a tip at a restaurant.
PPA is more common in adults under the age of 65, although anyone can develop it. Mental skills and memory deteriorate, and some people develop additional neurological conditions. As the condition worsens, the individual eventually needs help with activities of daily living and daily 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.
Once PPA is diagnosed, the main goal of treatment is to improve the ability to communicate; therefore, the individual can undergo interventions and speech therapies that can help them have a quality of life. There are no specific medications for this disorder; however, because of the 30%-40% probability of Alzheimer’s disease, doctors may prescribe known AD drugs such as Aricept, Namenda, or Exelon, even though none of these drugs have been shown to improve PPA. Because anxiety and/or depression appear later in the disorder, medications may be prescribed to manage these symptoms.
Symptoms begin gradually, often before age 65, and worsen over time. People with primary progressive aphasia may lose the ability to speak and write and eventually understand written or spoken language.
Questions about Alzheimer’s or related disorders can be sent to Dana Territo, author of What My Grandchildren Taught Me About Alzheimer’s, at [email protected]