Primary Progressive Aphasia and Its Effects on Language
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5/14/21
Primary Progressive
Aphasia and Its
Effects on
Language
Maya Henry, Ph.D., CCC-SLP
Departments of Speech,
Language, and Hearing
Sciences & Neurology
University of Texas at Austin
2021 AFTD Education Conference
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• Most common complaint:
“I just can’t remember the name….”
(especially proper names)
Language and • Word-finding does decline with age
normal aging • Difficulty with access rather than a
deterioration of knowledge
• Older adults outperform younger adults on
vocabulary tests
• Semantic memory (conceptual knowledge)
is preserved into old age (Burke & Shafto,
2008)
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• When the decline is more rapid than would
be expected for age and/or changes are
observed at ages
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Aphasia caused by stroke
Healthy
brain
Stroke
L R
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Critical regions for language
B=Broca’s area
B
W=Wernicke’s area
W
Stroke affecting Broca’s area Stroke affecting Wernicke’s area
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Language Areas of Left Hemisphere
SMG
B AG
W
PITC
brain from Nolte, 2002
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We now understand that speech and language are
supported by “networks” of critical left hemisphere regions
Speech Production Speech perception
Semantics Written language
Battistella et al., 2020, J Neurosci
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What about aphasia that is not caused
by stroke?
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“Something’s really wrong…”
“So, in my opinion it started about two years ago and I was walking my dog… and I
started looking down and saying What is that? I walk on it all the time but I don’t know
what that’s called any more. Nobody else knew. They probably recognized it but no
one ever told me. So, this is the first time I went to the doctor and I’m like, something’s
really wrong.”
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Aphasia caused by neurodegeneration
stroke
atrophy
L R
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Primary Progressive Aphasia (PPA)
• Slowly progressive aphasia caused by neurodegenerative
disease
• There must be no focal lesion (e.g., stroke)
• Most prominent clinical feature is difficulty with
speech/language
• Often affects individuals
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What causes PPA?
• PPA is caused by disease affecting the language areas
of the brain
• Frontotemporal lobar degeneration (FTLD)
• Corticobasal degeneration (CBD)
• Progressive Supranuclear Palsy (PSP)
• Amyotrophic Lateral Sclerosis (ALS)
• Alzheimer’s disease (AD)
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Speech/language problems in PPA
• Early on, most people complain of not being able to
find words
• “I know what it is, but I can’t say it.”
• Many will have trouble getting words to come out
correctly
• Saying “dog” instead of “cat”
• Saying “bog” instead of “dog”
• Some people have difficulty remembering concepts
• “A pencil...I know I’ve heard that before, but I’m
not sure what it is...”
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Speech/language problems in PPA
• Some will have trouble speaking in sentences
• “My house...and...son...and...dinner”
• Some will have trouble coordinating the muscles of
articulation
• Some will have a hard time remembering information that
they hear
• Remembering a phone number to write it down
• Most will have some trouble reading and writing
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PPA syndromes
• Nonfluent/agrammatic variant
• Slow, halting, effortful speech
• Difficulty with talking in and comprehending sentences
• Difficulty with motor aspects of speech
• Semantic variant = FTD
• Fluent, but empty communication
• Loss of conceptual knowledge about things and people
• Naming and comprehension problems
• Logopenic variant
• Pauses for word-finding
• Difficulty “holding on to” heard information
• Difficulty processing sounds in words
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Variants of PPA linked to underlying patterns of
atrophy caused by different diseases
Nonfluent variant = Tau
Semantic variant = TDP-43
Logopenic variant = AD
Wilson et al., 2010
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What is not affected in PPA
• At least early on...
• Memory for events
• Reasoning skills
• Sense of humor and general intelligence
• Sense of direction and other spatial abilities
• Artistic abilities
• Physical skills
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What can be expected as PPA progresses?
• Over time, speaking will likely become increasingly difficult
• Understanding what others say may become more difficult as
well
• More difficulty with things that are NOT language
• Memory and attention
• Calculations/math
• Problem solving
• Visuospatial processing
• Abnormal behaviors
• Motoric impairments
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What are researchers doing?
• Research currently underway is
attempting to understand
• What disease processes cause PPA
• What parts of the brain are
affected
• What medical treatments may help
• What behavioral treatments may
help
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Treatment for speech and language in PPA?
• Robust literature base supporting intervention for speech and
language in stroke patients
• Speech-language therapy in PPA yields promising results as
well (see Cathery-Goulart et al., 2013; Cadorio et al., 2017; Cotelli et al., 2020; Volkmer et al., 2020)
• However, considerably less research in this population
• “Therapeutic nihilism” on the part of referring/treating clinicians
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The goal of speech-language intervention
throughout the course of PPA:
Functional communication
• Maximize communication at each stage of illness
• Consider the individual in the context of their
environment(s) and functional needs
• Tailor treatment approach to current status; take into
account likely progression
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Aphasia Treatment Continuum
Restitutive
Aided approaches
Environmental support and partner training
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Treatment for word-finding in PPA
(Henry et al., 2013, Brain and Language; Henry et al, 2019, JSLHR)
• Lexical Retrieval Cascade Treatment
• Designed to capitalize on spared cognitive-linguistic
processes and encourage self-cueing (Henry et al., 2013)
• Involves repeated practice of strategies with photos of own
items
• When you can’t say a word, what can you say???
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Strategies for self-cueing
Digital Homework
Can you describe it?
Can you think of the first letter? First sound?
Here’s the word, practice reading it.
Now practice writing and saying it.
Is this something you find in your refrigerator?
What’s it called again?
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Naming Treatment Outcomes
(n=18; Henry et al.,2019, JSLHR)
Performance on trained and untrained sets of words as well as standardized tests over time. * = significant difference relative to pre-
treatment; † = significant difference relative to post-treatment. BNT= Boston Naming Test; WAB = Western Aphasia Battery
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Treatment for speech production and grammar
(Henry et al., Brain, 2018)
• Video-Implemented Script Training for Aphasia (VISTA)
• Repeated practice talking about important topics using
“scripted” content developed collaboratively with the clinician
• Largely homework-based (unison speech production with video)
• Practice using scripts in conversation
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Hard script:
Easy script:
Home practice Fly-fishing is a passion of mine for
Football is a great
sport. My favorite
video: numerous reasons, but mostly for the
wonderful places it takes me to. The
NFL team is the waters and the ecosystems are
Green Bay Packers. inevitably beautiful and interesting. I
My college team is also enjoy the fact that fly-fishing is
the UW Badgers. I so demanding, challenging and
love to watch totally absorbing. It serves a
football all the therapeutic role that releases me
time. from the stresses of everyday life.
When I am not fishing, I often find
30 minutes of unison speech myself planning a trip to one of the
production per day places I love to fish most, including
Connecticut, Montana, Alaska,
Canada, or Texas.
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Script training outcomes
Henry et al., 2018, Brain
A
Asterisk = significant difference relative to pre-treatment;
Diamond = significant difference relative to trained
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Treatment Continuum
Restitutive
Aided approaches
Environmental support and partner training
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Aided Approaches:
“To put the patient’s residual lexicon
visually in front of him/her so that the
patient can access needed vocabulary to
participate in daily activities as language
skills decline.”
--M. Fried-Oken
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Low tech options
– Communication books
– Photo albums
– Pictures
– Newspapers
– Communication boards
– Cards
– Paper and pencil
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High tech options
– Dedicated speech generating devices
– Mobile technology options
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Treatment Continuum
Restitutive
Aided approaches
Environmental support and partner training
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Environmental support, communication partner
training
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Communication strategies for care partners
■ Eye contact ■ Increase wait time for response
■ Face to face ■ Use multiple modes of communication
(message redundancy and modeling)
■ Quiet environment
– Write
■ Get attention before speaking (holding hands,
– Draw
physical touch)
– Use gestures
■ Introduce topic before starting a sentence
– Use book
■ Ask questions to confirm
■ Prompt and encourage use of other
■ Restate, repeat, or paraphrase to confirm communication modes
■ Guess using limited information to reduce ■ Decrease complexity of speech
frustration
■ Redirect when off task
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Aphasia-friendly communication can be summed
up with this acrostic for the word aphasia:
Ask simple, direct questions
Provide multiple communication options
Help communicate if asked
Acknowledge frustration
Speak slowly and clearly
If you don’t understand, say so
Allow extra time
https://www.aphasia.org/stories/thats-a-fact-quick-tips-for-aphasia-friendly-communication-part-two/
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Other things to remember:
• Avoid speaking on someone’s behalf unless absolutely
necessary; ask for permission to do so
• Use multi-modal communication and encourage the
person with aphasia to do so as well
• Be positive about attempts to communicate
• Don’t focus on errors or feel the need to correct them
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Multimodality communication
• Use all available communication modalities (verbal and
non-verbal)
• Use your hands (gesture) to communicate
• Write down words (or parts of words) that may help to
communicate a message
• Draw a picture
• Take advantage of facial expressions and eye contact
• Provides redundancy of your message and models
positive behaviors for the person with aphasia
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Encourage circumlocution
• Circumlocution = “talking around” a word that can’t be retrieved
• “If you can’t thing of the word, describe it…”
• “you know, it’s the thing that you write with…it has ink…”
• Even an incomplete description is likely to be helpful
• Provides valuable information to the listener
• May help the individual with aphasia to self-cue
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Other things to remember
• Fatigue affects communication
• Save important discussions for morning or sometime when the
person with aphasia is well-rested
• Communication abilities may be inconsistent in PPA
• Just because they could say it yesterday doesn’t mean they can say
it today
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Summary
• PPA is a progressive loss of speech and language ability caused by
neurodegenerative disease
• Brain networks responsible for motor speech/grammar, semantic (conceptual)
processing, or phonological (speech sound) processing may be affected
• Speech-language intervention for progressive aphasia is efficacious and
warranted
• Speech-language intervention in PPA should be the standard of care, not the
exception
• Restitutive, compensatory, and environmental approaches are beneficial
• Communication partners may help to promote and facilitate
communication for the person with PPA
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If you know someone who may have progressive
aphasia, send them our way…
• We work with individuals all over the country and the world via telerehabilitation
• We can offer treatment to bilingual and multilingual speakers
• Our website: https://slhs.utexas.edu/research/aphasia-research-treatment-lab
• Email: aphasialab@austin.utexas.edu
NIH (NIDCD R01DC016291; R03DC013403;
F32DC010945; F31DC010945; NIAP01 AG019724; and
NINDS R01NS050915)
@utaphasialabs
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Questions? Comments?
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