Meet the pioneers in primary progressive aphasia research

Decades before the world learned of Bruce Willis’ aphasia diagnosis, Dr. Marcel Mesolam pioneered a new field of research on a similar neurological condition: primary progressive aphasia (PPA), a rare, overlooked and underserved syndrome that destroys a person’s language.

Marsel Mesulam called the condition a PPA.

Mesulam in the 1980s was directing the behavioral neuroscience unit at Harvard Medical School where he saw patients with dementia — then individually defined as having “amnesia” — or who had had a stroke, which was, at the time, the only recognized cause. He has dementia and a language disorder. But in 1982, he began seeing individuals with difficulty with language who had not had a stroke or been diagnosed with dementia. He wondered what caused the disturbance of this language?

“That’s when the condition was given the name primary progressive aphasia,” said Mesulam, who is now founder and director of the Mesolam Center for Cognitive Neurology and Alzheimer’s Disease at Northwestern University School of Medicine. PPA is also known as mesoulam disease.

PPA comes in various forms

Now recognized as one of the major forms of dementia, it is important to note that PPA differs from typical Alzheimer’s dementia because PPA does not affect a person’s memory in the early stages and often for several years before the disease spreads to other areas of the brain. PPA is an early-onset disease, often beginning before age 65.

PPA is difficult to diagnose. It takes expert consultation and a team approach to identification, said Sandra Weintraub, professor of psychiatry, behavioral sciences and neuroscience at Feinberg and a member of the Missoulam Center.

PPA comes in various forms. Some individuals with PPA may have difficulty finding words, just as a stroke survivor does. The speech contains many pauses and fillers such as “thing” or “things”. A person may have difficulty arranging words to form a grammatical sentence. Speech becomes telegraphic by deleting pronouns and articles. For example, instead of saying, “I can’t find the words to express what I want to say,” one Mesollam patient said, “Words are in my head and cut off.”

Others speak fluently but do not understand the words. For example, if you ask someone with a PPA to bring an orange from the kitchen, they might say, “An orange? What is that?” Or they might come back with an apple. It is as if the words belong to a foreign language. Mesulam said finding the names of common things is especially difficult.

People with PPA may be able to drive, perform daily functions such as banking or shopping, and most can usually work in the early stages of the disease if their job does not involve speech. For some, they may direct their abilities from other areas of the brain, such as music and drawing, said Dr. Purna Punakdarpour (right), professor of behavioral neuroscience at Feinberg and a member of the Mesulam Center.

All Virtual Speech Therapy

The Missoulam Center, based in Chicago, runs an observational research program that flies in participants from more than 30 states across the United States and Canada, said Christina Coventry, a Northwestern senior clinical researcher who administers the program.

telemedicine
The experience of telemedicine is showing positive results.

Having been consistently funded by the National Institutes of Health for more than a decade, the program is dedicated to improving education and awareness of the disease, helping people better understand the neurobiology underlying it and trying to improve care and delivery of interventions.

One such intervention is a new, entirely hypothetical, randomized controlled trial of a speech therapy intervention for people with PPA focused on maximizing communication and quality of life. To the center’s knowledge, this is the first such trial of PPA, said Emily Rogalsky, who is leading the trial. Rogalsky is professor of psychiatry and behavioral sciences at Feinberg and co-director of the Missoulam Center.

“People with PPA are distributed but experience with the syndrome is not,” Mesulam said. “About three-quarters of our participants come from out of state, even New Zealand, because it is very difficult for them to find expertise locally. Most speech pathologists have never heard of PPA, but instead have experience with stroke-related aphasia which usually improves over time. time. To deal with that, we started this online experiment to offer something unique.”

So far, he said, the trial has been received very positively and pilot study data have shown meaningful gains by people with PPA.

“The goal of the intervention is not necessarily to bring in more words but to improve the efficiency of communication and to be more efficient and less anxious when communicating,” Mesulam said. “We also help the spouse or other communication partner learn how to include evidence or strategies to stimulate communication more effectively.”

Strong support system is the key

Having support from family and friends who understand PPA is critical for those living with the condition, said Darby Moorhart, a research professor at the Missoulam Center. The center’s clinic offers unique care and support for families living with a diagnosis. It has developed and examined the utility of care models, support group approaches and interventions, and regularly holds specialized educational support conferences for families and professionals.

Another intervention from the center is PPA TeleSavvy, which Morhardt is currently experimenting with. Funded by the National Institute on Aging and the Emory Royal University Center, the program supports caregivers across the country on how best to address the unique challenges facing family caregivers of those living with PPA.

Mapping language in the brain

Since Mesulam discovered PPA in the 1980s, he said, “the field has exploded.”

“Every year there are hundreds of research papers written on the PPA. We have learned a tremendous amount about how it is diagnosed and the identity of the underlying diseases,” Mesoulam said. Moreover, this has been a great tool for science to understand the way language is mapped in the human brain.

An observational research program helped identify the syndrome and allowed the discovery of key insights into the diseases that cause PPA, including risk factors such as dyslexia.

What’s Next?

“There are at least six diseases that cause PPA, including atypical forms of Alzheimer’s disease, frontotemporal degeneration with tauopathy, Pick’s disease and three different forms of frontotemporal degeneration with a TDP-43 abnormality,” Mesoulam said. “Everyone at present is incurable. So, the first goal is not only to accurately diagnose the underlying diseases but also to be able to treat them.”

His second goal, Mesulam said, is to study those with PPA more deeply and learn more about language in the human brain, “which is the only unique function of the human brain that makes our brain human.”

When should you worry?

We all have moments when we can’t remember the name of someone or something. When should this type of word contact become a concern as a possible symptom of PPA?

“Treat it like any other disease,” Mesulam said. “If you feel a little pain in your knees every time you say, ‘Oh, I have rheumatoid arthritis,’ that’s clearly an overreaction. If you happen to have a slip of the tongue or can’t find a word or person name, that’s no It means PPA. But if it becomes severe and consistent, look into it.”

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