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.

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.

Last Updated: 10 Dec 2014

View Details
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.

Last Updated: 14 Feb 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

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

View Details
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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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 107 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

Go To URL
Beyond the temporal pole: limbic memory circuit in the semantic variant of primary progressive aphasia.
 

Author(s): Rachel H Tan, Stephanie Wong, Jillian J Kril, Olivier Piguet, Michael Hornberger, John R Hodges, Glenda M Halliday

Journal: Brain. 2014 Jul;137(Pt 7):2065-76.

 

Despite accruing evidence for relative preservation of episodic memory in the semantic variant of primary progressive aphasia (previously semantic dementia), the neural basis for this remains unclear, particularly in light of their well-established hippocampal involvement. We recently ...

Last Updated: 21 Jun 2014

Go To URL
Nonverbal oral apraxia in primary progressive aphasia and apraxia of speech.
 

Author(s): Hugo Botha, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Jennifer L Whitwell, Keith A Josephs

Journal: Neurology. 2014 May;82(19):1729-35.

 

The goal of this study was to explore the prevalence of nonverbal oral apraxia (NVOA), its association with other forms of apraxia, and associated imaging findings in patients with primary progressive aphasia (PPA) and progressive apraxia of speech (PAOS).

Last Updated: 13 May 2014

Go To URL

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

Go To URL
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

Go To URL
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

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

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

 
 
Top

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

Go to URL
Communication Bridge Speech Therapy Research Study
 

Status: Recruiting

Condition Summary: Primary Progressive Aphasia; Alzheimer's Disease; Frontotemporal Dementia

 

Last Updated: 11 May 2015

Go to URL
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: 19 May 2015

Go to URL