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

Progression of logopenic variant primary progressive aphasia to apraxia and semantic memory deficits.
 

Author(s): Michitaka Funayama, Yoshitaka Nakagawa, Yoko Yamaya, Fumihiro Yoshino, Masaru Mimura, Motoichiro Kato

Journal:

 

Due to the nature of neurodegenerative disorders, patients with primary progressive aphasia develop cognitive impairment other than aphasia as the disorder progresses. The progression of logopenic variant primary progressive aphasia (lvPPA), however, has not been well described. In ...

Last Updated: 17 Jan 2014

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Phonological facilitation of object naming in agrammatic and logopenic primary progressive aphasia (PPA).
 

Author(s): Jennifer E Mack, Soojin Cho-Reyes, James D Kloet, Sandra Weintraub, M-Marsel Mesulam, Cynthia K Thompson

Journal: Cogn Neuropsychol. 2013 ;30(3):172-93.

 

Phonological processing deficits are characteristic of both the agrammatic and logopenic subtypes of primary progressive aphasia (PPA-G and PPA-L). However, it is an open question which substages of phonological processing (i.e., phonological word form retrieval, phonological encoding) ...

Last Updated: 8 Oct 2013

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Grammatical comprehension deficits in non-fluent/agrammatic primary progressive aphasia.
 

Author(s): Dorothy Charles, Christopher Olm, John Powers, Sharon Ash, David J Irwin, Corey T McMillan, Katya Rascovsky, Murray Grossman

Journal: J. Neurol. Neurosurg. Psychiatr.. 2014 Mar;85(3):249-56.

 

Grammatical comprehension difficulty is an essential supporting feature of the non-fluent/agrammatic variant of primary progressive aphasia (naPPA), but well-controlled clinical measures of grammatical comprehension are unavailable.

Last Updated: 7 Feb 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 11 free, full-text review articles on human participants. First 3 results:

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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Primary progressive aphasia: from syndrome to disease.
 

Author(s): J A Matías-Guiu, R García-Ramos

Journal: Neurologia. ;28(6):366-74.

 

Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. Major breakthroughs made in recent years have lent us a better understanding of this syndrome, which may be the first manifestation of ...

Last Updated: 15 Jul 2013

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The non-fluent/agrammatic variant of primary progressive aphasia.
 

Author(s): Murray Grossman

Journal: Lancet Neurol. 2012 Jun;11(6):545-55.

 

The non-fluent/agrammatic variant of primary progressive aphasia (naPPA) is a young-onset neurodegenerative disorder characterised by poor grammatical comprehension and expression and a disorder of speech sound production. In an era of disease-modifying treatments, the identification ...

Last Updated: 21 May 2012

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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: 22 Jul 2014

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Impact of Emotional Mimicry and Oxytocin on Frontotemporal Dementia
 

Status: Recruiting

Condition Summary: Frontotemporal Dementia

 

Last Updated: 11 Sep 2014

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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: 15 Aug 2014

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