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

Dyslexic Characteristics of Chinese-Speaking Semantic Variant of Primary Progressive Aphasia.
 

Author(s): Simon Kang Seng Ting, Heidi Foo, Pei Shi Chia, Shahul Hameed, Kok Pin Ng, Adeline Ng, Nagaendran Kandiah

Journal: J Neuropsychiatry Clin Neurosci. 2018 ;30(1):31-37.

 

Reading disorder is a recognized feature in primary progressive aphasia (PPA). Surface dyslexia, characterized by regularization errors, is typically seen in the English-speaking semantic variant of PPA (svPPA). However, dyslexic characteristics of other languages, particularly logographical ...

Last Updated: 31 Dec 1969

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Emotion detection deficits and changes in personality traits linked to loss of white matter integrity in primary progressive aphasia.
 

Author(s): Namita Multani, Sebastiano Galantucci, Stephen M Wilson, Tal Shany-Ur, Pardis Poorzand, Matthew E Growdon, Jung Yun Jang, Joel H Kramer, Bruce L Miller, Katherine P Rankin, Maria Luisa Gorno-Tempini, Maria Carmela Tartaglia

Journal:

 

Non-cognitive features including personality changes are increasingly recognized in the three PPA variants (semantic-svPPA, non fluent-nfvPPA, and logopenic-lvPPA). However, differences in emotion processing among the PPA variants and its association with white matter tracts are unknown. ...

Last Updated: 31 Dec 1969

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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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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

Last Updated: 12 Jun 2018

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Transcranial Magnetic Stimulation in Primary Progressive Aphasia
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 9 Jul 2018

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Language in Primary Progressive Aphasia
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia

 

Last Updated: 23 Jul 2018

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