This review was updated from the original version on July 31, 2023. Two references were added and the list of assessments was expanded. The text version of the review reflects the changes. 

Primary Progressive Aphasia (PPA). What a tangled web of confusion this condition is, right? Is it aphasia? Is it dementia? Well, for starters, it’s both. Before we dive deep into this tangled web, let’s start with some very basic reminders to help us, you know, not have web all over our faces and in our hair:

Aphasia = loss of receptive and expressive language

Dementia = loss of memory, language, problem-solving and other thinking abilities that are severe enough to make living independently impossible or dangerous.

With PPA, we eventually have both aphasia and dementia. 

The sheer amount of research that has come out in the last 10 years about PPA is staggering, and SLPs who previously knew little about PPA now report seeing more of these patients on their caseloads. Thanks to Bruce Willis and his family bravely sharing his journey, the public has a new familiarity and respect for this condition. After all, if toughest-guy-in-the-world John McClane can be diagnosed with PPA, anyone can, right? 

We need to untangle all the information, old and new, so that we can better understand PPA and its progression. This is where we need a primer. A Primary Primer, if you will. A primer on the progression of Primary Progre… OK, you get it. 

Your Primary Progressive Aphasia Primer

PPA is a progressive, neurodegenerative condition in which language deficits are the initial symptoms, and actually, the predominant feature for much of the disease's course. Oh, and that term? Disease? Some experts posit that it’s not even a disease in and of itself; rather, a “range of clinical syndromes” without one underlying cause. Focal, progressive loss of language was first identified in the late 1800s and was associated with frontotemporal atrophy of the brain (frontotemporal dementia). It was later called Primary Progressive Aphasia, and to account for the progressive evolution to more global impairments, some even took to calling it “PPA+”. 

PPA doesn’t improve or plateau like in stroke or TBI-based aphasia, and this is where things get… a little tangled. Foremost, it’s a progressive condition that eventually always evolves into dementia, (more on that later). The onset isn’t acute and diagnosis typically happens before the age of 65. The prognosis is widely variable, with some cases evolving into dementia in just months, while other cases maintain only mild language deficits for many years. Life expectancy is also variable, but the average is around 7–10 years after diagnosis. 

Understandably, PPA wreaks havoc on people’s lives. Because of that fairly young age of onset, the progressive loss of the ability to communicate can have a ”devastating and all-consuming” impact, including loss of participation in everyday events, loss of income, far-reaching implications on mood and mental health, poor quality of life, and of course, care partner burden. Actually, the U.S. Social Security Administration recently added PPA to the ”most disabling conditions” list, allowing for approval of expedited benefits to those diagnosed. 

Perhaps the most difficult is that because language deficits are the only major symptom and independent function for ADLs usually stays intact for much longer, patients with PPA retain insight into their own progressive loss of communication. So, they’re often all too aware that this condition will evolve into dementia. That progression, although variable, is characterized by many of the deficits we see in dementia—changes in motor function, episodic memory, behavior, and personality, all resulting in the inability to complete activities of daily living independently. 

Variants of PPA

Within PPA, there is a pretty wide spectrum of presentations.

There are three main variants: 

• semantic variant, which often progresses to Frontotemporal Dementia
• nonfluent/agrammatic variant, which can progress to Frontotemporal Dementia, Corticobasal syndrome, or Progressive Supranuclear Palsy
• logopenic variant, which often progresses to Alzheimer's Dementia.

Sorting through the tangles here can be particularly challenging because while they’re very different from one another, discerning the differences while the language deficits are still mild is tricky. Why is classification so important? Well, it helps to inform the nature and suspected progression of PPA and can help providers and caregivers build on the patient’s strengths—an important consideration. 
 

There is an online calculator from the cognitive screener ACE-III that can help us with that if we’re in a hurry. And although 1 in 5 PPA diagnoses will be unclassifiable, you’ll want to still try to identify relative areas of strength and impairment. You know what? When I have something that’s tangled, I lay it out on a table to unravel. Cue the table! 

Get a printable version of this chart here.

Now that we’ve sorted through that, where do we start our assessments? 

Diagnosis and Assessment

Of course, we don't give a PPA diagnosis on our own. But certainly, our involvement in speech, language, and cognitive testing provides insight to the medical team. One purpose of assessment is to help establish the variant and severity and to get baseline measurements to track progression. The other purpose, of course, is to plan treatment that emphasizes independence, quality of life, and optimal communication. 

Purpose 1:  Establishing PPA variant, severity, and baseline

While there’s no single test for PPA, you can use subtests of language tests you’re already familiar with, but you won’t be administering these in the standardized way (e.g., giving the whole thing). Considering where your patient is in the disease progression is important in assessment selection too. A mildly impaired patient may present as one variant, but as the impairments get more severe, a different variant may come to light. It is important to capture some language features, like lexical retrieval, object knowledge, syntax, and repetition with attention to disproportionate performance, so think about the must-haves and probably-haves in the table above. A couple more tests may be more useful here than others for establishing baselines, determining severity, and differentiating variants:
 

• ACE-III with online calculator (free)
• Boston Diagnostic Aphasia Examination (specifically the syntax subtests and BNT, where a score of 20+ → likely non-fluent variant; 11 or less → likely semantic)
• Hopkins Action Naming Assessment (free; score of 20+ → likely non-fluent; score of less than 12 → likely semantic)
• Kissing and Dancing Short Form (free)
• Northwest Anagram Test
• Portions of the FTLD module from the National Alzheimer’s Coordinating Center (all free)
  • Benson Figure Copy Test
  • Verbal Fluency Phonemic Test 
  • Word Reading Test—Regular and Irregular Words 
  • Noun and Verb Naming
  • Sentence Repetition and Reading Test (when including the long items, a score of 20+ → likely logopenic)
• Pyramids and Palm Trees Short Form 
• Repeat and Point Test (free)
• Sydney Language Battery (free)
• Western Aphasia Battery

If you’re tracking progression or considering functional outcomes:

• Progressive Aphasia Severity Scale

If you’re assessing language function for participation in ADLs:

• Communication Activities of Daily Living

Purpose 2: Planning treatment that emphasizes independence, quality of life, and optimal communication

Traditional aphasia assessment is focused on identifying impairments so we know what to rehabilitate. Not so much for our folks with PPA. You see, despite our commitment to doing everything we can to support these folks through their disease process… exhaustive assessment often doesn’t match up with what the patient wants or needs. Sometimes the ongoing assessment required by physicians or insurance can have negative functional and emotional implications for the patient. That constant highlighting of what you can’t do anymore, especially if you’re living independently, is exhausting. Instead, remember to use a listen-first approach—a phenomenal construct that could serve us all in our lives outside of SLP-ing.

Because PPA is progressive, the better we understand the patient’s strengths, the better we can use those to our advantage in treatment planning. 

The R.A.I.S.E. Framework is just one example of guidelines in strength-based assessment and treatment with an essential focus on what’s important to the patient. Can they still read the Wall Street Journal every morning? Can they still chat with people at the dog park? Again, it’s the daily activities of the individual. Not to be mistaken with activities of daily living…although, also important. I identify as a nature-loving feminist, an SLP, a wife and mother, and a darn good cook! 

Everyone will identify their strengths and priorities differently, and it really helps to get to know how the patient sees themselves. The R.A.I.S.E. framework does this by establishing a relationship with the patient, using a flexible and informal assessment approach, including chances for feedback from the patient, supporting self-advocacy and independence, and evolving the assessment as the condition progresses. Learn more about this framework, including two case examples in this open-access article.

Keeping the R.A.I.S.E framework in mind, your initial assessment might look something like this:

A thorough case history:

• Initial presentation?
• Emergence of additional symptoms?
• Severity of linguistic impairment?
• Family history?
• Hearing and vision impairments?
• Here’s a clever trick: Record your patient speaking during this case history. This way you can review it later to assess their conversational skills and motor speech instead of asking them to do additional assessment tasks.

Involve the patient’s primary care partner(s):

• Ask about all the various care partners and other major players in the person’s life.
• Who is best to incorporate into treatment sessions?
• Is there a care partner that is better to relay compensatory strategies and home practice to?
• What types of changes do care partners notice? Anything that the patient isn’t noticing yet?

Consider the following:

• What accommodations are already being made? Self-cueing strategies already being used?
• What is the patient’s understanding of their own deficits?
• What are their expectations of therapy?
• What are the main factors of the patient’s identity?
• What has the impact been on the patient’s daily activities?
• Would they consider AAC options?
• What are the patient’s current communicative strengths?

We know this is a lot of information for you to keep track of. You need enough information to identify the possible PPA variant and create a patient-centered treatment plan. That's why we created this downloadable assessment tracker and variant chart based on the information above. Once you’re done assessing and ready to write goals, take extra care to assure that they’re patient-focused, functional, and attainable. 

Goal Writing for PPA

Goal writing can be especially tricky with neurodegenerative conditions. You’ll want to focus on attainable goals that really tap into what the patient and their loved ones value. Goal Attainment Scaling and Patient Reported Outcome Measures (PROMS) are two tools that can help guide clinicians in establishing, measuring, and meeting goals. You can also check out the CPIB for a free 10-item scale measuring functional communication participation.

Does your patient want to communicate more effectively in their garden club? Use a goal like this: 

• Patient will demonstrate use of supplemental communication (e.g., writing) and/or self-cueing (e.g., circumlocution) to increase the amount of conversational turns during garden club from a baseline of 2 conversational turns to a goal of 5 conversational turns within the next three months. 

Is your patient’s care partner well-intended but taking over conversations in a way that bothers the person with PPA? Try this goal: 

• Care partner will increase wait time from a baseline of less than 1 second in between conversational turns to at least 7 seconds during dinner conversations three times weekly. 

Remember, if you’ve got the right goals, you’ll be able to show the patient is benefiting from SLP services, despite the inevitable decline of standardized subtest scores. If your patient isn’t progressing towards your goals, consider whether those goals are appropriate. 
 

Untangled Yet? 

Probably not completely. As you know, the brain is an inherently tangled place, so understanding rare diagnoses and accounting for individual variability will never fully untangle this complex condition. But hopefully, you’ve unraveled enough to feel more confident—or at least you don’t have web in your mouth? 

When you're ready, Informed SLP members can click over to Part Two of this series, where we'll dive into treatment. 

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