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

Differentiating between subtypes of primary progressive aphasia and mild cognitive impairment on a modified version of the Frontal Behavioral Inventory.
 

Author(s): Donna C Tippett, Carol B Thompson, Cornelia Demsky, Rajani Sebastian, Amy Wright, Argye E Hillis

Journal:

 

Behavioral assessment has been investigated in frontotemporal lobar degeneration and Alzheimer's disease, but has not been explored extensively in subtypes of primary progressive aphasia (PPA). We explored the ability of a modified version of the Frontal Behavioral Inventory (FBI-mod) ...

Last Updated: 31 Dec 1969

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Data-driven classification of patients with primary progressive aphasia.
 

Author(s): Paul Hoffman, Seyed Ahmad Sajjadi, Karalyn Patterson, Peter J Nestor

Journal: Brain Lang. 2017 Nov;174():86-93.

 

Current diagnostic criteria classify primary progressive aphasia into three variants-semantic (sv), nonfluent (nfv) and logopenic (lv) PPA-though the adequacy of this scheme is debated. This study took a data-driven approach, applying k-means clustering to data from 43 PPA patients. ...

Last Updated: 31 Dec 1969

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Cholinergic depletion and basal forebrain volume in primary progressive aphasia.
 

Author(s): Jolien Schaeverbeke, Charlotte Evenepoel, Rose Bruffaerts, Koen Van Laere, Guy Bormans, Eva Dries, Thomas Tousseyn, Natalie Nelissen, Ronald Peeters, Mathieu Vandenbulcke, Patrick Dupont, Rik Vandenberghe

Journal:

 

Primary progressive aphasia (PPA) is a heterogeneous syndrome with various neuropathological causes for which no medical treatment with proven efficacy exists. Basal forebrain (BF) volume loss has been reported in PPA but its relation to cholinergic depletion is still unclear. The ...

Last Updated: 31 Dec 1969

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

Transcranial direct current stimulation in post stroke aphasia and primary progressive aphasia: Current knowledge and future clinical applications.
 

Author(s): Rajani Sebastian, Kyrana Tsapkini, Donna C Tippett

Journal: NeuroRehabilitation. 2016 Jun;39(1):141-52.

 

The application of transcranial direct current stimulation (tDCS) in chronic post stroke aphasia is documented in a substantial literature, and there is some new evidence that tDCS can augment favorable language outcomes in primary progressive aphasia. Anodal tDCS is most often applied ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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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: 31 Dec 1969

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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: 12 Sep 2017

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Communication Bridge: A Person-centered Internet-based Intervention for Individuals With Primary Progressive Aphasia
 

Status: Not yet recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 8 Dec 2017

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Assessment of Hyperphosphorylated Tau PET Binding in Primary Progressive Aphasia
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 11 Oct 2017

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