What is primary progressive aphasia?

Primary progressive aphasia, or PPA, is a frontotemporal disorder marked by progressive losses in language ability, which includes speaking, comprehending, reading and writing.

Aphasia is a general term used to refer to deficits in language functions. PPA is a neurological syndrome caused by a 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 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 experiences difficulty thinking of common words while speaking or writing.

Memory, reasoning and visual perception are commonly not affected, so in that respect, the individual with the condition can function rather normally in many daily routine activities. As PPA progresses, verbal communication is difficult, and other mental and cognitive abilities eventually decline.

The individual might hesitate as he is searching for the right word or verbalize thoughts in a noncohesive manner, such as using a sentence with abnormal word orders. Substitution of words or names of objects is common in PPA, as is using words that are mispronounced or incomprehensible. There is a sudden lapse in understanding simple words, and though they can recognize other individuals, an individual with PPA is unable to think of their names.

PPA also produces difficulty in writing, such as checks or correspondence, and also difficulty in reading and following simple written instructions. People with PPA also struggle with calculations and arithmetic, such as making change, adding up money or leaving a tip at a restaurant.

PPA is more common in adults under the age of 65, though anyone can develop it. Mental skills and memory become impaired, and some begin to have additional neurological conditions. As the condition worsens, the affected person eventually needs assistance with activities of daily living and day-to-day care.

About 50 percent of people with PPA will 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 other signs of dementia will develop. Additionally, about half of PPA-affected people 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. The individual may undergo interventions and speech therapies that can help him have a quality of life. There are no specific medications for the disorder. However, because of the 30 to 40 percent probability of Alzheimer's disease, doctors may prescribe familiar Alzheimer's medications such as Aricept, Namenda or Exelon, though none of these drugs have been shown to improve PPA. As anxiety and/or depression occur later in the disorder, medications may be prescribed to manage those symptoms.

Questions about Alzheimer's disease or a related disorder can be sent to Dana Territo, the Memory Whisperer, director of services at Alzheimer's Services of the Capital Area at advice@alzbr.org or visit the organization at 3772 North Blvd., Baton Rouge.