Understanding Primary Progressive Aphasia with Naomi Nevler, MD
“Working at Penn, Dr. Murray Grossman unfolded FTD before my eyes. It was mesmorizing and so sad, at the same time.”
Naomi Nevler is a 2004 graduate of the Sourasky School of Medicine in Tel-Aviv University, Ramat-Aviv, Israel. In 2015 she completed her residency in Neurology at the Tel-Aviv Medical Center. Dr. Nevler’s experience treating patients who suffer from neurodegenerative disorders has led her to join Dr. Grossman’s lab at the Penn FTD Center with the intention of specializing in the field of Behavioral Neurology.
Naomi really broke down the ins and outs of PPA and did so beautifully. To learn more about her work in the field, click below!
This episode beautifully weaves the science and the stories, you guys! We are happy to present our new buddy, Naomi. As we begin, she thoroughly defines what Primary Progressive Aphasia (PPA) means. “Let's start from the end,” she explains. “Aphasia is a fancy term for a language disorder and progressive is something that gets worse over time, it progresses.” Lastly, the word primary, in this case, means there isn’t a secondary cause for the neurodegeneration - like a stroke, for example. Saying it in another way, the aphasia is from the disease itself, there is no other cause. With all that being said, PPA is the language component of the disease, this is where we see the parroting, the struggle to find the words, the loss of object knowledge.
“The brain is so complex, and often peculiar within a disease state.”
After the definition, Dr. Nevler describes the difference in PPA (did you know there are subtypes?!) after she states that PPA is normally found - you guessed it! - in the frontal and temporal lobes of the brain. She also dropped a little gold nugget that I would love to share because I found it fascinating. Naomi said that if the atrophy is heavy on the left side, you generally have more insight into the disease state. Crazy, right? The brain, man. The conversation evolves to the different types of PPA with Dr. Nevler explaining that there are two branches of PPA.
Difficulty Finding Words: typically presents with trouble naming objects, remembering people's names, and slowly progresses into loss of object knowledge (we’ll get into that more, later!). This type of PPA is known as Semantic PPA.
Difficulty Speaking: contrary to the semantic form, Non-Affluent PPA presents as difficulty forming a coherent sentence and problems with grammar. Under this form, Dr. Nevler explains, the person affected by PPA typically has the ability and lucidity to understand what is going on/what people are saying. They cannot, however, appropriately respond.
Within both of these variations of PPA, there is a comprehension component that presents itself as well. Studies and research have proven that there is a decrease in comprehension of language (all language: reading, writing, listening, speaking) that presents itself when an FTD diagnosis is suspected. That is to say, if your loved one cannot follow multi-step (or complex) instructions, PPA is likely at play.
Jumping back to loss of object knowledge - this is wild, yall. Alot of patients that present with a PPA leaning diagnosis, will eventually encounter loss of object knowledge. This is fancy lingo for not remembering/knowing what an object is used for (i.e. a fork is for eating, a toothbrush is used for oral hygiene, etc). They may be able to say the word, but won’t recall how to use the tool. How heartbreakingly sad.
Naomi suggests that the first thing we can do to support our loved one with PPA is to “provide emotional support” where and whenever possible. Dr. Nevler continues to explain that supporting them can take many forms but to really focus on “minimizing their response effort.” Don’t make them work hard to communicate AND use your familiarity to get in front of a request. She also mentions using apps and visual charts to help them communicate during the early stages of language loss. Lastly, Dr. Nevler reminds us all to trust our guts in caregiving and rely heavily on the fact that we know these people deeply and can, at times, preemptively recognize needs or wants.
“There is so much hope around this disease. There is so much more awareness and so much more advancements. This is all good.”
Maximizing communication in PPA is a partnership. Communication strategies can be designed for both the person with PPA and their care partners.
Consultation with a speech-language pathologist is valuable to carefully evaluate specific communication skills and tailor person-centered strategies and tools based on needs.
Most communication is nonverbal. Use a full array of gentle gestures, pictures, facial expressions, props, and visual cues to connect.
Especially early in the disease, awareness of the loss of language and communication skills can weigh heavily on an individual. Tend compassionately to grief, loss and possible depression.
Understanding PPA as a progressive disease is important so people can be aware of and anticipate possible changes in symptoms and care needs over time.
In semantic variant PPA, people often develop difficulty recognizing faces and understanding the emotions of others. In addition to these cognitive changes, disinhibited, rigid and compulsive behaviors eventually develop.
Resources from AFTD
AFTD has resources for those looking to learn more about PPA and the specific types. We offer overviews of each type of PPA along with webinars and additional resources on the pages below. There is even a printable handout at the bottom of each overview page that can be shared with others to better understand the symptoms: Primary Progressive Aphasia Overviews
As Dr. Nevler shares, there are communication strategies that can support the person with PPA. An introduction to communication tips for PPA can be found here: Communicating with Persons Living with PPA: Tips and Strategies
AFTD also offers versions of our Awareness Cards for PPA that can be shared in social and community settings to help others understand the person’s symptoms.
The AFTD HelpLine can help you connect you with additional resources and learn more about PPA support options for care partners and the person living with PPA. We offer a 6 week program called Living Well with PPA twice a year for those diagnosed within the past year. Connect with us on the HelpLine to learn more.info@theaftd.org or 866-507-7222.
