Primary progressive aphasia

Common Name(s)

Primary progressive aphasia

Primary progressive aphasia (PPA) is a disorder characterized by language disturbance, including difficulty making or understanding speech (aphasia). In the early stages, PPA often causes difficulty with naming, word finding, or word comprehension. In later stages, affected people often become mute and lose their ability to communicate. PPA may sometimes be the initial manifestation of another neurodegenerative disease, such as frontotemporal dementia or Alzheimer disease. In some cases, PPA is caused by mutations in the GRN gene and is inherited in an autosomal dominant maner.

PPA can be classified into three distinct types which include progressive non-fluent aphasia (PNFA), semantic dementia (SD), and the logopenic variant (LPA).
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary progressive aphasia" for support, advocacy or research.

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Association for Frontotemporal Degeneration

The Association for Frontotemporal Degeneration (AFTD) is a nationwide non-profit organization whose mission is to promote and fund research into finding the cause and cure for the frontotemporal dementias; to provide information, education, and support to persons diagnosed with frontotemporal dementias (FTD) and their families and caregivers; and to educate physicians and allied health professionals about FTD.

Last Updated: 8 Jul 2015

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The Aphasia Hope Foundation

Aphasia Hope Foundation is a public 501(c) 3 non-profit foundation that has a two-fold mission: (1) to promote research into the prevention and cure of aphasia and (2) to ensure all survivors of aphasia and their caregivers are aware of and have access to the best possible treatments available.

Last Updated: 14 Feb 2013

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Primary progressive aphasia" for support, advocacy or research.

Logo
Association for Frontotemporal Degeneration

The Association for Frontotemporal Degeneration (AFTD) is a nationwide non-profit organization whose mission is to promote and fund research into finding the cause and cure for the frontotemporal dementias; to provide information, education, and support to persons diagnosed with frontotemporal dementias (FTD) and their families and caregivers; and to educate physicians and allied health professionals about FTD.

http://www.theaftd.org

Last Updated: 8 Jul 2015

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The Aphasia Hope Foundation

Aphasia Hope Foundation is a public 501(c) 3 non-profit foundation that has a two-fold mission: (1) to promote research into the prevention and cure of aphasia and (2) to ensure all survivors of aphasia and their caregivers are aware of and have access to the best possible treatments available.

http://www.aphasiahope.org

Last Updated: 14 Feb 2013

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Primary progressive aphasia" returned 130 free, full-text research articles on human participants. First 3 results:

The Brain Network of Naming: A Lesson from Primary Progressive Aphasia.
 

Author(s): Raffaella Migliaccio, Claire Boutet, Romain Valabregue, Sophie Ferrieux, Marie Nogues, Stéphane Lehéricy, Didier Dormont, Richard Levy, Bruno Dubois, Marc Teichmann

Journal:

 

Word finding depends on the processing of semantic and lexical information, and it involves an intermediate level for mapping semantic-to-lexical information which also subserves lexical-to-semantic mapping during word comprehension. However, the brain regions implementing these components ...

Last Updated: 23 Feb 2016

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Phonological Processing in Primary Progressive Aphasia.
 

Author(s): Maya L Henry, Stephen M Wilson, Miranda C Babiak, Maria Luisa Mandelli, Pelagie M Beeson, Zachary A Miller, Maria Luisa Gorno-Tempini

Journal: J Cogn Neurosci. 2016 Feb;28(2):210-22.

 

Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory-phonological) and ventral (lexical-semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective ...

Last Updated: 31 Dec 2015

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Phonological short-term memory in logopenic variant primary progressive aphasia and mild Alzheimer's disease.
 

Author(s): Aaron M Meyer, Sarah F Snider, Rachael E Campbell, Rhonda B Friedman

Journal: Cortex. 2015 Oct;71():183-9.

 

It has been argued that individuals with logopenic variant primary progressive aphasia (lvPPA) have an impairment of the phonological loop, which is a component of the short-term memory (STM) system. In contrast, this type of impairment is not thought to be present in mild typical ...

Last Updated: 19 Sep 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Primary progressive aphasia" returned 14 free, full-text review articles on human participants. First 3 results:

Primary progressive aphasia and the evolving neurology of the language network.
 

Author(s): M-Marsel Mesulam, Emily J Rogalski, Christina Wieneke, Robert S Hurley, Changiz Geula, Eileen H Bigio, Cynthia K Thompson, Sandra Weintraub

Journal: Nat Rev Neurol. 2014 Oct;10(10):554-69.

 

Primary progressive aphasia (PPA) is caused by selective neurodegeneration of the language-dominant cerebral hemisphere; a language deficit initially arises as the only consequential impairment and remains predominant throughout most of the course of the disease. Agrammatic, logopenic ...

Last Updated: 6 Oct 2014

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Primary progressive aphasia and apraxia of speech.
 

Author(s): Youngsin Jung, Joseph R Duffy, Keith A Josephs

Journal: Semin Neurol. 2013 Sep;33(4):342-7.

 

Primary progressive aphasia is a neurodegenerative syndrome characterized by progressive language dysfunction. The majority of primary progressive aphasia cases can be classified into three subtypes: nonfluent/agrammatic, semantic, and logopenic variants. Each variant presents with ...

Last Updated: 15 Nov 2013

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Behavioural interventions for enhancing life participation in behavioural variant frontotemporal dementia and primary progressive aphasia.
 

Author(s): Kathleen B Kortte, Emily J Rogalski

Journal: Int Rev Psychiatry. 2013 Apr;25(2):237-45.

 

Primary progressive aphasia (PPA) and behavioural-variant frontotemporal dementia (bvFTD) are clinical syndromes under the umbrella term 'frontotemporal dementia' (FTD) and are caused by a neurodegenerative disease with an onset most typically in the productive years of adulthood. ...

Last Updated: 24 Apr 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Language in Primary Progressive Aphasia
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 10 Jun 2016

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Assessment of Hyperphosphorylated Tau PET Binding in Primary Progressive Aphasia
 

Status: Not yet recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 7 Apr 2016

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tDCS Intervention in Primary Progressive Aphasia
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia; MCI; FTD

 

Last Updated: 4 May 2016

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