While primary progressive aphasia (PPA) may be the rarest form of aphasia, we frequently get questions because there are fewer resources out there for this neurodegenerative disease. Treating PPA differs from treating aphasia caused by a stroke or traumatic brain injury, highlighting the fact that aphasia information is rarely one-size-fits-all. The NAA is committed to increasing our resources and outreach for PPA, and we want to start by pulling together some facts about this lesser-known form of aphasia.
What Is PPA?
The gradual degeneration of brain tissue in the parts of the brain that control speech and language causes PPA. That tissue loss slowly impacts communication skills, though the speed and way it progresses vary from person to person (and from one subtype of PPA to another). While strokes cause a sudden, immediate change in communication abilities, PPA is gradual. This means people with PPA may lose the ability to speak, write, read, or understand language over time. Once words and communication begin to go, people with PPA may find other changes occur including memory loss.
What Are the Types of PPA?
There are three types of PPA. People with agrammatic PPA have trouble producing words. They “remember words but they struggle to speak and to string grammatical sentences together. They can understand single words but find sentences harder.” They may have difficulty getting the word out of their mouth or have halting speech filled with pauses. At the same time, this type of PPA affects listening skills, and people with agrammatic PPA may not understand what is being said as the disease progresses.
People with logopenic PPA have trouble plucking the correct word from their brain. They may say a word that is closely related or say the wrong word that sounds similar to the one they want. Similar to people with agrammatic PPA, their speech may be filled with pauses, and they may also have difficulty repeating back a sentence.
Finally, people with semantic PPA have difficulty understanding words. They “can speak fluently, but their words make no sense together (even though they’re in the right grammatical order). They also have severe comprehension problems. They can understand casual conversation through contextual cues, but they struggle with isolated words.” They may have trouble naming objects or reading because they are having problems with word meaning.
Is PPA the Same as Alzheimer’s Disease?
No, though both are neurodegenerative diseases. PPA affects language first, while Alzheimer’s disease affects memories early on. PPA begins in the frontotemporal area of the brain, which is responsible for language. As the two diseases progress, they begin to resemble each other in the sense that people with PPA may lose memories as well as the ability to care for themselves, much like Alzheimer’s disease. But the first signs of PPA are language-based, and the first signs of Alzheimer’s disease are memory-based.
How Do You Get Diagnosed with PPA?
You can’t diagnose PPA on your own. The best way to receive a detailed evaluation and prognosis about PPA is a referral to a neurologist or neuropsychologist who has experience with PPA. Make sure you ask your neurologist to refer you to a speech-language pathologist (SLP) for speech therapy (see below). If you can’t find a neurologist who has worked with other patients with PPA, you can make an appointment with a speech-language pathologist (SLP) who has PPA experience and who can refer you to a neurologist that is familiar with this condition.
What Are the Early Stages of PPA?
PPA differs from person to person, so it is impossible to predict how long each stage will last. Initially, for a period that can last many months to a couple of years, a person diagnosed with PPA will exhibit a progressive loss of speech and language capabilities. At this stage, the rest of life may continue as usual, including work and daily activities.
Symptoms in this early stage depend on the type of PPA (see above) but may include slowed or halting speech, decreased use of language, word-finding difficulty, written or spoken sentences with abnormal word order, substituting words, mispronouncing words, talking around a word, difficulty understanding conversation, sudden difficulty understanding simple words, problems writing, problems reading, difficulty with spelling, and difficulty with arithmetic.
Beyond language and communication changes, people with PPA and their caregivers experience the emotional side of a PPA diagnosis. In addition to working with a speech therapist to maintain language skills, people with PPA and their caregivers may also want to see a therapist to work through the emotional landscape.
What Are the Later Stages of PPA?
Due to the progressive nature of PPA, language abilities will continue to deteriorate over many years. PPA may also affect other cognitive abilities such as memory, reasoning, and visual perceptual skills. The rate of progression of PPA varies, and it is unknown why some people progress more rapidly than others. In the later stages, people may need an assisted living facility.
Can Speech Therapy Help PPA?
Many resources about aphasia suggest working with a speech therapist because SLPs help people regain lost communication skills. The idea of gaining lost language is more applicable to aphasia caused by a stroke or a traumatic brain injury.
But SLPs can still be helpful when it comes to PPA, working with people to maintain language skills. They can work with the family to find new ways to communicate or use tools that aid communication. While they cannot cure PPA or slow down its progression, they can help prepare the person and their caregivers for future stages of the disease.
Tactus Therapy gives plenty of useful tips for directing speech therapy when it comes to PPA (vs. Broca’s aphasia), including focusing on priority vocabulary. Rather than trying to hold onto all speech, focus on the most important, useful words for the individual. “Practice only what’s important to your client. For example, if the client loves pineapple, then use the word in your program. If they don’t care for or need to talk about pineapple, then leave the word out of treatment.”
Also, it can be helpful to create “PPA cards,” a card the person can carry in their pocket with an explanation to hand others as well as common phrases they may need to remember while interacting with other people. It’s also helpful to start working early with non-verbal cues and gestures.
A Starting Point
This is just the starting point—a general overview of the most common questions we get about PPA. It doesn’t begin to touch upon the emotional side of the diagnosis or deeper questions about communication in later stages. We would love to hear YOUR PPA story as we build out these resources—how you and your family processed the diagnosis or what has worked for you. Please leave your story in the comment section below.
En Español, Afasia primaria progresiva
Read more about PPA: