Diagnosis and Treatment of Primary Progressive Aphasia: Where Do SLPs Belong?

By Rene L. Utianski, PhD, CCC-SLP
August 29, 2019

As I now work with patients with Primary Progressive Aphasia (PPA), I find myself reflecting on my initial experiences in helping to diagnose and care for them. Briefly, for those unfamiliar with PPA, it is an umbrella term that captures a heterogeneous group of neurological syndromes in which the course of language disturbance is one of insidious onset and progression. By definition, PPA reflects a predominant deficit in language rather than some other cognitive process (e.g., memory as is seen in Alzheimer’s disease dementia). PPA most commonly reflects frontotemporal lobar degeneration (FTLD), TDP-43 pathology, or Alzheimer’s disease pathology. Although advances in imaging let us (less than perfectly) predict the underlying pathology, most patients live with a clinical diagnosis of PPA without being certain of the specific neurological cause during life.

When I began to see these patients in the clinic, I did not feel confident in sharing the diagnosis with them and their loved ones. Rather, I found myself echoing what the patient had already shared—that it was progressive—and offering a label for the language difficulties—aphasia. Now, I can’t help but wonder, why was that?

I think the answer is twofold.

First, I was accustomed to caution when expressing many diagnoses. For instance, stating that a mixed flaccid-spastic dysarthria was not incompatible with the amyotrophic lateral sclerosis (ALS) diagnosis that that the patient knew was a possibility. But I have come to understand that in PPA the medical diagnosis and the speech-language diagnosis are arguably one and the same. It was very appropriate for me to share information about PPA, while it was up to the neurologist to provide an estimation of the underlying disease (e.g., FTLD or TDP-43, based on neuroimaging findings), including ruling out other possible causes (e.g., stroke, tumor, infection).

The second reason was a lack of confidence stemming from gaps in my knowledge. I knew the basics about PPA. I knew enough to confidently recognize it, but did not know much about the disease course. I was uncertain about counseling patients about what they could expect in the future or which management strategies might be most meaningful.

Once I expanded my knowledge base, I became more confident in my ability to recognize the distinguishing features of PPA and its sub-variants and found myself offering more complete education and counseling to patients and their care partners.  I subsequently was better able to assert myself to ensure accurate diagnoses for these patients. Not unlike many things, my increased knowledge was power.

What have I learned along the way?

When evaluating someone who may have PPA, it is important that speech-language pathologists (SLPs) ascertain the onset and course of the communication problem and determine through examination if it represents  a focal language deficit (aphasia) versus a disorder more in keeping with  a broader constellation of non-aphasic cognitive communication deficits. For instance, although a patient with clinically “typical” Alzheimer’s disease may have language difficulties, they are not the primary problem.

PPA can be a devastating diagnosis for many reasons, but especially because there are no disease-modifying options at this time. However, patients and their loved ones are often relieved to finally have a diagnosis that accurately captures their difficulty and makes some predictions about the future. Once a diagnosis is established, SLPs can offer a wealth of information to help patients, families, and care partners adapt to the loss of cognitive-communication functions. This, of course, requires a shift from “rehabilitation” as we are used to in the context of stroke and traumatic brain injury, where we expect improvement and work toward restoration of function. With a progressive condition, an “adaptive” approach is more appropriate. A major goal for many patients with PPA is to maintain as well as possible independence and participation in activities of daily living. Another management goal is to provide the patient’s family means of supporting communication as the patient requires additional assistance. In the context of degenerative disease, dynamic clinical decision-making is essential.

My experience has reinforced that diagnosing and managing PPA and other neurodegenerative diseases requires a team approach. The answer to the title question—where do SLPs belong? —is that it is right at the heart of the team as an optimally prepared member to recognize, diagnose, and treat the person with aphasia. We should collaborate closely with neurologists and other healthcare providers.

To increase your knowledge about PPA, see the National Aphasia Association website (https://www.aphasia.org/aphasia-resources/primary-progressive-aphasia/) and The Association for Frontotemporal Degeneration website (https://www.theaftd.org/what-is-ftd/primary-progressive-aphasia/) for general information.

For more specific guidance about diagnosis and treatment planning for the communication disorders associated with PPA, see the upcoming Primary Progressive Aphasia and Other Frontotemporal Dementias: Diagnosis and Treatment of Associated Communication Disorders. It is written by and for clinical speech-language pathologists and a resource I wish I had earlier in my career. Its intent is to help SLPs become more expert at identifying these conditions and develop confidence in diagnosing and treating affected individuals.