Primary Progressive Aphasia: A Definitive End with an Artistic Interlude
The Mesulam Center interviewed researchers, those of whom are involved in primary progressive aphasia research, about a unique gift many of their research participants possessed and left behind at the Center — art.
By: Ananya Chandhok
Christina Ann Coventry, MSN, RN, spent her time at the Mesulam Center with persons living with primary progressive aphasia (PPA) — a rare dementia syndrome that affects a person’s language.
During her tenure at the Center, Coventry tested trial participants, recruited them, and learned the ins and outs of their lives, but “one pleasant, old southern lady” left a lasting mark on Coventry.
“She would do these beautiful, giant paintings,” Coventry said. “She did them well up until she couldn’t speak at all, but she was still doing this. Making artwork.”
The individual has since passed, and her husband donated her brain for research.
“He would call annually,” Coventry said. “And he would ask, ‘Is my wife’s brain still there?’”
He kept in touch with the Mesulam Center, where Coventry would update him on new findings. To him, she still lived on.
This is one person who lived with PPA, but how her artistic ability and memories lasted beyond her is true for many before and after.
Researchers spoke to the Mesulam Center about PPA participants’ artwork for an online archive. The team hopes to memorialize the pieces and remember individuals’ research contributions, even after their passing.
What is Primary Progressive Aphasia (PPA)?
PPA is just a syndrome, said Marsel Mesulam, MD, founding director emeritus of the Mesulam Center and Ruth Dunbar Professor in Neuroscience and Neurology.
In fact, several diseases can potentially result in PPA.
“There are three, four, five kinds of PPA,” Mesulam said. “And there are about ten diseases that [lead to] PPA, so in a ten by five matrix, [there are] a lot of combinations.”
Mesulam first identified PPA as a syndrome in 1982, and it is often referred to as Mesulam’s syndrome.
This is the progressive loss of words’ meanings, according to the Association for Frontotemporal Degeneration (AFTD).
“The unique thing about the human brain is language,” Mesulam said. “There are so many things in everyday life that depend very strongly on the ability to understand what others are saying and to communicate what we think…”
Understanding how PPA affects communication is a team effort at the Center.
Elena Barbieri, PhD, research assistant professor of Physical Medical and Rehabilitation at the Mesulam Center, is involved with recruiting new participants into the PPA program.
Barbieri described early semantic PPA as “different from anything” she’s seen before, based on her experience with stroke-based aphasia.
“It’s very eye-opening because they have this dissociation in the way they talk,” Barbieri said. “[With] some [who are] at the beginning of the disease, you can barely tell there’s something wrong with them.”
Sometimes participants may be able to give generalized answers about their daily routine, but the aha moment is when Dr. Mesulam asks them to identify objects, Barbieri said.
An elephant, a giraffe, and a lion – plastic of course – are witnesses to individuals who would’ve otherwise dodged an aphasia diagnosis. When asked to point to one of the three, persons living with aphasia can’t associate the word with the object, Barbieri said.
“When they [persons living with aphasia] are working with these mediums, they get to exist in a space where the issues they’re having with language don’t really play a role. I do think when you create art, the materials lead you.”
Michelle Anne Los, MS, Clinical Operations Manager and PPA Team Lead at the Mesulam Center
Where Words Fail, Art Speaks
Woodworking, glassblowing, painting, sculpting, and photography are just a few of the skills individuals living with PPA have homed in on, early in their diagnosis.
“It’s difficult to communicate [for PPA participants] because they can’t put their thoughts into words or because they don’t understand what others are saying,” Mesulam said. “And for some, both.”
Despite this loss, or rather in light of it, the persons’ interaction with their environments increases, Mesulam said.
Artistry has no bounds for some of these individuals. Even writing, despite PPA-related communication difficulties, becomes an outlet.
“I know a participant here who always wanted to write a novel, and then when he started developing PPA despite the difficulties with language, he wrote and published it,” Barbieri said.
The disease doesn’t give individuals new talents. Instead, they are nonverbally “re-rooting their talents and engagement with the world,” Mesulam said.
“If all that is important is to understand the plot on television, or to listen to news, or read newspapers, these people would be devastated,” Mesulam said. “But they are not.”
Individuals can experience other heightened non-verbal habits too, in addition to using traditional creative outlets.
Becoming a Sudoku expert, making 3,000-piece jigsaw puzzles, and breeding pigeons isn’t uncommon, Mesulam said.
Individuals' intensified “preoccupations” allow them to be flexible, so they can switch how they communicate, Mesulam said.
Coventry is still “mind blown” at how some individuals are “total rocks” while facing PPA.
“I would look at these people’s MRI scans, and their brain would be mostly missing in places because it would be so atrophied,” Coventry said. “And yet, this person would be baking cakes, going to the store…just living their lives.”
Individuals obsessed over certain habits when their words fell short, Mesulam said.
“Because the involvement of the brain is limited, they really hang onto some of these non-verbal means of expression with a vengeance,” Mesulam said.
“If one road is blocked, then [they] try to find some other way,” Mesulam said.
Holding onto Hope
Sometimes, these artistic abilities appear, but they have nothing to do with language skills, Barbieri said.
With disease progression, however, these skills are also affected.
In the short-term, Barbieri has “a little bit of hope.”
“This fills their life,” Barbieri said. “Getting recognition from other people–clinicians, and family members, I imagine, calms their increasing struggles.”
Partners find comfort and a way to be “distracted” from the disease through this ability too, Barbieri said.
“They [spouses] talk about difficulties with language,” Barbieri said. “But they also talk about how good they are at golfing, sculpting, and so forth.”
Often spouses’ small actions help their person more than they can imagine.
“They are doing all they can,” Mesulam said. “So, the best thing you [spouses] can do is to complete their sentences, say things in a simple way, and [help them] with the words they can’t find.”
Eventually, individuals’ support systems became the Mesulam Center’s backbone.
“We’re part of the same team,” Mesulam said. “Their [families’] contribution to this research is priceless. We can’t do it without them.”
To this day, individuals’ photographs, paintings, and even a patchwork quilt adorn the Mesulam Center’s walls — a reminder of PPA participants’ resilience till the end.
“They lived on in our work,” Coventry said.