Getting to Know Primary Progressive Aphasia

Primary Progressive Aphasia is a little known, often confused, and frequently missed diagnosis. What is it? What goes into the diagnosis? How can we harness the brain to compensate for it? Read on as we explain the symptoms, known causes, and how language tasks can help.

Primary Progressive Aphasia (PPA) presents similarly to typical Aphasia, however it progresses differently and is caused by different neurological processes.

PPA is actually a type of focal dementia, meaning that it affects a specific area of neurologic function, specifically language. It usually presents with word-finding issues first, and gradually affects grammar and understanding as it progresses in severity. It also differs from typical Aphasia in that it does not improve over time, but as is suggested in the name, progresses in severity. Initially, memory, visual processing, and personality are not affected outside of their association with language; however as the disease progresses, these cognitive functions can be affected as well. It can affect both written and oral language, and its progression and the ways that it affects language differ in each case of PPA.

It is sometimes difficult to diagnose primary progressive aphasia, as it is similar to more typical forms of dementia, such as Alzheimer’s and actually can be caused by Alzheimer’s and by another common form of dementia, Frontotemporal Lobar Degeneration (FTLD). Someone can, in fact, have both PPA and Alzheimer’s or FTLD.   The hallmark of PPA specifically, however, is that initially the only cognitive functions affected are linguistic. Frequently, PPA is hard to pick up on at first, because its progression is very gradual.

In order to be diagnosed with primary progressive aphasia, the cause of the language issues must be a neurodegenerative disorder, meaning that it is associated with a progressive issue causing death of or damage to neuro cells. For this reason, in addition to a thorough neurological evaluation, neuropsychological evaluation, and speech and language evaluation, neuroimaging is particularly important in the diagnosis of PPA. PPA can be caused by Alzheimer’s or FTLD, and is most commonly caused by one of these two types of neurodegenerative disorders. Other types of neurodegenerative disorders can cause it as well, though this is more rare.

PPA can present differently depending on what parts of the brain are being affected by the neurodegenerative disease. PPA is often divided into three types: PPA-G, PPA-S, and PPA-L. PPA-G usually causes difficulties with grammar (thus the “g”) and syntax, or how we put specific words together in a specific order to make meaning. Someone with PPA-G might misuse pronouns, articles, or prepositions, or might have scrambled sentence order. PPA-S affects how well someone understands and uses the meanings of words. Someone with PPA-S often seems to have “forgotten” the meaning of a word, and might be entirely confused when you refer to a table. Their speech is usually fairly fluid but empty of meaning. Finally, PPA-L presents with problems with word-finding. Someone with PPA-L will have a lot of “circumlocutions”, or talking around a word they are trying to think of, or will refer to something as a “thing” or might say “you know what I mean”.

Although there is no medical treatment for PPA, often people will take preventative medications for Alzheimer’s as it is commonly the underlying cause of PPA. One of the most practically useful things to do if you are diagnosed with primary progressive aphasia is to start speech therapy. Not only can the therapist give you language tasks and exercises to strengthen your current linguistic functioning, but they can also help you to create compensatory systems to assist in daily functioning.

A diagnosis of PPA is not the end of a productive and involved life.

Many persons with PPA are able to continue working, particularly if they have effective communication strategies in place. A clinician can help you to figure out if, when, and how to tell your co-workers or boss that you have PPA. They can help you learn how to explain PPA and how to teach your communication partners how to best assist you. They can help you set up data bases in cell phones or notebooks of important linguistic supports for your most key personal information.

Know that you are not alone!

With the advent of the Internet, there are many support groups. Check Facebook for groups, and see if any local academic or community institutions have any support groups for persons with PPA and their families. There are also many research studies going on where you can not only receive compensation in the form of money or therapy, but where you can also join a community of persons with PPA who want to help scientists develop more effective treatments for PPA.