Primary progressive aphasia

Common Name(s)

Primary progressive aphasia

Primary progressive aphasia (PPA) is a type of dementia characterized by language disturbance, including difficulty making or understanding speech (aphasia). PPA can be part of frontotemporal dementia or Alzheimer disease. Spatial skills and memory usually remain intact. In early stages, PPA often manifests as deficits in naming, word finding, or word comprehension. In late stages, affected individuals often become mute and lose their ability to communicate. In some cases, PPA is inherited in an autosomal dominant fashion and caused by mutations in the GRN gene.

PPA can be classified into three distinct variants based on language profiles, which include progressive non-fluent aphasia (PNFA), semantic dementia (SD), and the recently characterized 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: 10 Dec 2014

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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: 10 Dec 2014

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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 101 free, full-text research articles on human participants. First 3 results:

Frontal white matter tracts sustaining speech production in primary progressive aphasia.
 

Author(s): Maria Luisa Mandelli, Eduardo Caverzasi, Richard J Binney, Maya L Henry, Iryna Lobach, Nikolas Block, Bagrat Amirbekian, Nina Dronkers, Bruce L Miller, Roland G Henry, Maria Luisa Gorno-Tempini

Journal: J. Neurosci.. 2014 Jul;34(29):9754-67.

 

In primary progressive aphasia (PPA), speech and language difficulties are caused by neurodegeneration of specific brain networks. In the nonfluent/agrammatic variant (nfvPPA), motor speech and grammatical deficits are associated with atrophy in a left fronto-insular-striatal network ...

Last Updated: 17 Jul 2014

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Logopenic and nonfluent variants of primary progressive aphasia are differentiated by acoustic measures of speech production.
 

Author(s): Kirrie J Ballard, Sharon Savage, Cristian E Leyton, Adam P Vogel, Michael Hornberger, John R Hodges

Journal:

 

Differentiation of logopenic (lvPPA) and nonfluent/agrammatic (nfvPPA) variants of Primary Progressive Aphasia is important yet remains challenging since it hinges on expert based evaluation of speech and language production. In this study acoustic measures of speech in conjunction ...

Last Updated: 3 Mar 2014

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Progranulin-associated PiB-negative logopenic primary progressive aphasia.
 

Author(s): Keith A Josephs, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Prashanthi Vemuri, Matthew L Senjem, Ralph B Perkerson, Matthew C Baker, Val Lowe, Clifford R Jack, Rosa Rademakers, Jennifer L Whitwell

Journal: J. Neurol.. 2014 Mar;261(3):604-14.

 

The logopenic variant of primary progressive aphasia (lvPPA) strongly associates with Alzheimer's disease, but can also associate with frontotemporal lobar degeneration. We aimed to assess the frequency of lvPPA in patients with speech and language disorders without β-amyloid deposition, ...

Last Updated: 10 Mar 2014

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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 12 free, full-text review articles on human participants. First 3 results:

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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Are there susceptibility factors for primary progressive aphasia?
 

Author(s): Emily Rogalski, Sandra Weintraub, M-Marsel Mesulam

Journal: Brain Lang. 2013 Nov;127(2):135-8.

 

The determinants of selective vulnerability in neurodegenerative diseases remain elusive. The asymmetric loss of neurons in primary progressive aphasia offers a unique setting for addressing this question. Although no factor can yet account for the selective vulnerability of the left ...

Last Updated: 25 Nov 2013

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Symptoms, Diagnosis, and Treatment

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: 11 Feb 2015

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Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL)
 

Status: Recruiting

Condition Summary: FTLD; Progressive Supranuclear Palsy (PSP); Frontotemporal Dementia (FTD); Corticobasal Degeneration (CBD); PPA Syndrome; Behavioral Variant Frontotemporal Dementia (bvFTD); Semantic Variant Primary Progressive Aphasia (svPPA); Nonfluent Variant Primary Progressive Aphasia (nfvPPA); FTD With Amyotrophic Lateral Sclerosis (FTD/ALS); Amyotrophic Lateral Sclerosis (ALS); Oligosymptomatic PSP (oPSP); Corticobasal Syndrome (CBS)

 

Last Updated: 11 Feb 2015

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Phase 1/2 Study of Vorinostat Therapy in Niemann-Pick Disease, Type C1
 

Status: Recruiting

Condition Summary: Neimann-Pick Disease

 

Last Updated: 28 Jan 2015

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