Frequently Asked Questions
Understanding the disease
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Is there specific knowledge related to the interpretation (by the person) and expression of pain in people with PPA?
To our knowledge, the topic of pain perception and interpretation in people with PPA has been very little explored to date.
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What level of functioning (autonomy, behaviors, level of support required, etc.) should be anticipated, according to the disease's evolution over the years?
It all depends on the variant. The logopenic variant evolves like Alzheimer's disease memory variant after a few years. The semantic variant evolves very slowly with multiple behavioral changes. The non-fluent variant is often accompanied by motor changes as in Parkinson's disease and can therefore become very disabling physically as well. We have conducted a study on the evolution of the three PPA variants and the results will be available on the PPA Platform in about 6 months.
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In what broad nosographic framework are PPAs classified?
The semantic variant and the non-fluent agrammatic variant belong to the family of frontotemporal neurocognitive disorders, while the logopenic variant falls within the family of Alzheimer's disease.
We classify variants based on the underlying pathological substrate: TDP-43 in svPPA, Alzheimer pathology in lvPPA, and tau protein in nfvPPA.
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How to differentiate the logopenic variant from language Alzheimer's?
The logopenic variant of PPA is often called language Alzheimer's. To diagnose a logopenic variant, communication difficulties must be the first symptoms to appear and be predominant in the clinical picture. The diagnostic criteria of Gorno-Tempini et al. (2011) must also be respected (see PPA Platform). However, Alzheimer's disease could present with significant language impairments that accompany other cognitive changes (e.g., memory, executive functions), without necessarily meeting the diagnostic criteria for lvPPA. In this case, we would speak of Alzheimer's disease with predominant language impairments.
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According to the prognosis of PPA type, what is the evolution of symptoms? Are there different evolution patterns?
Each PPA variant has its unique signature in terms of evolution. We invite you to consult the PPA Platform to learn more about the expected evolution for each variant.
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What is the life expectancy of a person with PPA, according to the three variants?
Life expectancy depends on several factors, particularly comorbidities (vascular risk factors, cancer, etc.). The logopenic variant evolves like Alzheimer's disease memory variant, often over a period of approximately 10-15 years. The semantic variant evolves very slowly with multiple behavioral changes, over a duration of approximately 15-20 years. The non-fluent variant is often accompanied by motor changes as in Parkinson's disease and can therefore become very disabling physically. Its evolution is faster, over a period of approximately 8-10 years.
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What are the impacts on receptive language for non-fluent and logopenic PPA? E.g.: Comprehension can be affected in semantic PPA, due to semantic loss.
Comprehension is affected differently in PPA variants. For the semantic variant, understanding of isolated words and concepts can be impaired early in the disease. The person may, for example, have difficulty understanding the meaning of certain words used in conversations or have difficulty accessing knowledge about certain concepts (e.g., its utility, its form). The impacts are therefore significant in daily life. In the non-fluent/agrammatic variant, it is mainly the comprehension of sentences with complex syntactic structures that is impaired (e.g., It's the girl who is being followed by the boy). Finally, in the logopenic variant, comprehension is generally quite well preserved.
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Can PPA be prevented? Can the disease be slowed down?
In general, certain modifiable risk factors can help prevent neurocognitive disorders (Dementia Prevention - 12 Modifiable Risk Factors And Their Means Of Prevention). In the case of the logopenic variant, acetylcholinesterase inhibitors, also prescribed in Alzheimer's disease, can help stabilize symptoms for a few years.
Treatment
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What is the state of science regarding the effects of semaglutide on PPA?
To our knowledge, no study is currently underway in PPA, but several studies are underway in Alzheimer's disease - memory variant, in combination or not with monoclonal antibodies Lecanemab and Donanemab.
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Is there ongoing research regarding the characteristics of augmentative and alternative communication (AAC) tools to favor based on different PPA profiles?
There is currently very little scientific evidence related to augmentative and alternative communication in PPA. It is mainly the knowledge about each variant and their expected evolution that guides us in choosing tools to favor (e.g., phone versus electronic tablet in relation to the presence of motor apraxia, content organization in relation to the presence of semantic impairment).
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Is the anti-TDP-43 agent in development? Do we have an idea of the timelines?
There is currently a lot of ongoing research on a molecule against TDP-43, but no significant development so far.
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Is Aricept still given in cases of early diagnosis of PPA?
Acetylcholinesterase inhibitors (e.g., Aricept, Exelon) are routinely prescribed only for diagnoses of logopenic variant of PPA, due to the amyloid pathology underlying common to Alzheimer's disease.
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Among the three intervention approaches suggested during the webinar, which one is suggested according to the type of PPA?
In speech-language pathology, a combination of approaches is generally used, depending on the different intervention objectives pursued. In all variants, it is relevant to offer an ecosystemic approach to inform and equip relatives as well as a compensation approach to support communication, whether through teaching communication strategies or using compensatory tools. The decision to undertake or not a restoration approach depends on the stage of disease evolution and the person's cognitive profile.
Patient support
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How can relatives help by adapting their language? Could you give examples, tips, ways to help at the discussion level for relatives?
The communication toolbox, created by Université Laval, presents a set of strategies that can facilitate communication with a person experiencing communication difficulties.
BoiteAOutilsCommunicationnelle.pdf (in French only)