Table of ContentsView AllTable of ContentsSymptomsDemographicsCausesTypesTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Demographics
Causes
Types
Treatment
Primary progressive aphasia, or PPA, is a type offrontotemporal dementia (FTD)that affects speech and language. The word “aphasia” refers to difficulty with expressive and/or receptive communication. This neurological syndrome causes people to slowly lose their ability to read, write, speak, and understand language.
UnlikeAlzheimer’s disease(AD), other cognitive functions tend to remain intact in early PPA.PPA is not the same as AD, although AD is thought to cause PPA in 30% to 40% of cases.
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Symptoms of PPA
Initial symptoms of PPA include difficultyrecalling a specific word, substituting a closely related word, such as “take” for “tack,” and comprehension problems.People with PPA can often perform intricate tasks but have difficulty with speech or language. For example, they may be able to build a complicated house but not be able to express themselves well verbally or understand what others are trying to communicate to them.
As the disease progresses, speaking and understanding written or spoken words becomes more difficult, and many people with PPA eventuallybecome mute. On average, a few years after these initial symptoms involving language appear, PPA begins to affectmemoryand other cognitive functions, as well asbehavior.
Who Gets PPA?
PPA is classified as a rare disease, however, many may be undiagnosed since they might not seek medical help or be misdiagnosed due to the unfamiliarity with PPA.
Interestingly, men develop PPA about twice as often as women. The average age of onset is between 50 and 70.Those who get PPA are more likely to have a relative with some type of neurological problem.
Causes of PPA
PPA happens when tissues in thefrontal, parietal, and temporal regions (lobes) of the brain gradually deteriorate over time, usually on theleft side of the brain. This is where several speech, language, and memory “control centers” are located.
Underlying neurodegenerative diseases causing PPA. The most common are:
In nearly all cases PPA is not genetic however, very seldom it can be hereditary. The most common gene implicated in familial PPA is the progranulin gene (GRN).
Categories of PPA
PPA can be subdivided into three categories:
Additionally, some research that involved providing language activities, communication techniques, counseling, and education to people living with PPA and their spouses demonstrated a significant improvement in communication and coping at its completion.
tDCS for PPA
Additional support unrelated to language ability can be helpful as well. Difficulties with sleep, anxiety, and mood are common. Certain medications, like selective serotonin reuptake inhibitors (SSRIs) can help to manage the anxiety, depression, and changes in behavior associated with PPA.
Good nutrition (fruits, green leafy and other vegetables, whole grains, fish, poultry, and healthy fats including nuts) should also be a goal. Try to limit poor food choices, skipped/forgotten meals, and unhealthy weight gain or loss.
Prognosis and Life Expectancy
While some people with PPA can continue working for quite some time, others find that they are unable to perform at their job, especially if their work requires a higher level of communication and collaboration with others. Language ability slowly declines over years.
PPA eventually progresses to more generalized/common forms of the person’s underlying neurological condition (Alzheimer’s, FTD). Because of this, changes in memory, attention, or personality develop, and movement or swallowing may become affected.
As with other frontotemporal dementias, the long-term prognosis is limited. The typical life expectancy from the onset of the disease is 3 to 12 years.Often, complications from PPA, such as swallowing difficulties, lead to the eventual decline.
A Word From Verywell
We at Verywell understand that primary progressive aphasia can be a difficult diagnosis to receive, both as an individual and as a family member of someone with PPA. Most people benefit from connecting with others in similar situations as they cope with the challenges that develop from PPA. One resource available nationwide is the Association for Frontotemporal Dementia. They offer several local support groups, as well as online information and phone support.
12 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Mesulam M.Primary progressive aphasia: A dementia of the language network.Dement Neuropsychol. 2013;7(1):2-9. doi:10.1590/S1980-57642013DN70100002Northwestern University Medicine.Symptoms & Causes of PPA.Grossman M.Linguistic aspects of primary progressive aphasia.Annu Rev Linguist. 2018;4:377-403. doi:10.1146/annurev-linguistics-011516-034253Mesulam MM, Rogalski EJ, Wieneke C, et al.Primary progressive aphasia and the evolving neurology of the language network.Nat Rev Neurol. 2014;10(10):554-569. doi:10.1038/nrneurol.2014.159Matías-Guiu JA, García-Ramos R.Primary progressive aphasia: from syndrome to disease.Neurologia. 2013;28(6):366-374. doi:10.1016/j.nrl.2012.04.003Henry ML, Hubbard HI, Grasso SM, et al.Treatment for word retrieval in semantic and logopenic variants of primary progressive aphasia: immediate and long-term outcomes.J Speech Lang Hear Res. 2019;62(8):2723-2749. doi:10.1044/2018_JSLHR-L-18-0144Tee BL, Gorno-Tempini ML.Primary progressive aphasia: a model for neurodegenerative disease.Curr Opin Neurol. 2019;32(2):255-265. doi:10.1097/WCO.0000000000000673Tsai RM, Boxer AL.Treatment of frontotemporal dementia.Curr Treat Options Neurol; 16(11):319.Jokel R, Meltzer J, D.R. J et al.Group intervention for individuals with primary progressive aphasia and their spouses: Who comes first?J Commun Disord. 2017;66:51-64. doi:10.1016/j.jcomdis.2017.04.002Coemans S, Struys E, Vandenborre D, et al.A Systematic Review of Transcranial Direct Current Stimulation in Primary Progressive Aphasia: Methodological Considerations.Front Aging Neurosci. 2021;13:710818. Published 2021 Oct 7. doi:10.3389/fnagi.2021.710818National Institutes for Health. Genetic and Rare Diseases Information Center.Primary Progressive Aphasia: prognosis.Association for Frontotemporal Degeneration.Find support near you.Additional ReadingNational Aphasia Association. Diagnosing Primary Progressive Aphasia. Diagnosing Primary Progressive AphasiaNational Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/National Institutes of Health. Office of Rare Diseases Research. Primary Progressive Aphasia. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8541Northwestern University. Feinberg School of Medicine. Primary Progressive Aphasia. http://www.brain.northwestern.edu/ppa/University of California, San Francisco. Forms of Frontotemporal Dementia. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
12 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Mesulam M.Primary progressive aphasia: A dementia of the language network.Dement Neuropsychol. 2013;7(1):2-9. doi:10.1590/S1980-57642013DN70100002Northwestern University Medicine.Symptoms & Causes of PPA.Grossman M.Linguistic aspects of primary progressive aphasia.Annu Rev Linguist. 2018;4:377-403. doi:10.1146/annurev-linguistics-011516-034253Mesulam MM, Rogalski EJ, Wieneke C, et al.Primary progressive aphasia and the evolving neurology of the language network.Nat Rev Neurol. 2014;10(10):554-569. doi:10.1038/nrneurol.2014.159Matías-Guiu JA, García-Ramos R.Primary progressive aphasia: from syndrome to disease.Neurologia. 2013;28(6):366-374. doi:10.1016/j.nrl.2012.04.003Henry ML, Hubbard HI, Grasso SM, et al.Treatment for word retrieval in semantic and logopenic variants of primary progressive aphasia: immediate and long-term outcomes.J Speech Lang Hear Res. 2019;62(8):2723-2749. doi:10.1044/2018_JSLHR-L-18-0144Tee BL, Gorno-Tempini ML.Primary progressive aphasia: a model for neurodegenerative disease.Curr Opin Neurol. 2019;32(2):255-265. doi:10.1097/WCO.0000000000000673Tsai RM, Boxer AL.Treatment of frontotemporal dementia.Curr Treat Options Neurol; 16(11):319.Jokel R, Meltzer J, D.R. J et al.Group intervention for individuals with primary progressive aphasia and their spouses: Who comes first?J Commun Disord. 2017;66:51-64. doi:10.1016/j.jcomdis.2017.04.002Coemans S, Struys E, Vandenborre D, et al.A Systematic Review of Transcranial Direct Current Stimulation in Primary Progressive Aphasia: Methodological Considerations.Front Aging Neurosci. 2021;13:710818. Published 2021 Oct 7. doi:10.3389/fnagi.2021.710818National Institutes for Health. Genetic and Rare Diseases Information Center.Primary Progressive Aphasia: prognosis.Association for Frontotemporal Degeneration.Find support near you.Additional ReadingNational Aphasia Association. Diagnosing Primary Progressive Aphasia. Diagnosing Primary Progressive AphasiaNational Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/National Institutes of Health. Office of Rare Diseases Research. Primary Progressive Aphasia. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8541Northwestern University. Feinberg School of Medicine. Primary Progressive Aphasia. http://www.brain.northwestern.edu/ppa/University of California, San Francisco. Forms of Frontotemporal Dementia. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Mesulam M.Primary progressive aphasia: A dementia of the language network.Dement Neuropsychol. 2013;7(1):2-9. doi:10.1590/S1980-57642013DN70100002Northwestern University Medicine.Symptoms & Causes of PPA.Grossman M.Linguistic aspects of primary progressive aphasia.Annu Rev Linguist. 2018;4:377-403. doi:10.1146/annurev-linguistics-011516-034253Mesulam MM, Rogalski EJ, Wieneke C, et al.Primary progressive aphasia and the evolving neurology of the language network.Nat Rev Neurol. 2014;10(10):554-569. doi:10.1038/nrneurol.2014.159Matías-Guiu JA, García-Ramos R.Primary progressive aphasia: from syndrome to disease.Neurologia. 2013;28(6):366-374. doi:10.1016/j.nrl.2012.04.003Henry ML, Hubbard HI, Grasso SM, et al.Treatment for word retrieval in semantic and logopenic variants of primary progressive aphasia: immediate and long-term outcomes.J Speech Lang Hear Res. 2019;62(8):2723-2749. doi:10.1044/2018_JSLHR-L-18-0144Tee BL, Gorno-Tempini ML.Primary progressive aphasia: a model for neurodegenerative disease.Curr Opin Neurol. 2019;32(2):255-265. doi:10.1097/WCO.0000000000000673Tsai RM, Boxer AL.Treatment of frontotemporal dementia.Curr Treat Options Neurol; 16(11):319.Jokel R, Meltzer J, D.R. J et al.Group intervention for individuals with primary progressive aphasia and their spouses: Who comes first?J Commun Disord. 2017;66:51-64. doi:10.1016/j.jcomdis.2017.04.002Coemans S, Struys E, Vandenborre D, et al.A Systematic Review of Transcranial Direct Current Stimulation in Primary Progressive Aphasia: Methodological Considerations.Front Aging Neurosci. 2021;13:710818. Published 2021 Oct 7. doi:10.3389/fnagi.2021.710818National Institutes for Health. Genetic and Rare Diseases Information Center.Primary Progressive Aphasia: prognosis.Association for Frontotemporal Degeneration.Find support near you.
Mesulam M.Primary progressive aphasia: A dementia of the language network.Dement Neuropsychol. 2013;7(1):2-9. doi:10.1590/S1980-57642013DN70100002
Northwestern University Medicine.Symptoms & Causes of PPA.
Grossman M.Linguistic aspects of primary progressive aphasia.Annu Rev Linguist. 2018;4:377-403. doi:10.1146/annurev-linguistics-011516-034253
Mesulam MM, Rogalski EJ, Wieneke C, et al.Primary progressive aphasia and the evolving neurology of the language network.Nat Rev Neurol. 2014;10(10):554-569. doi:10.1038/nrneurol.2014.159
Matías-Guiu JA, García-Ramos R.Primary progressive aphasia: from syndrome to disease.Neurologia. 2013;28(6):366-374. doi:10.1016/j.nrl.2012.04.003
Henry ML, Hubbard HI, Grasso SM, et al.Treatment for word retrieval in semantic and logopenic variants of primary progressive aphasia: immediate and long-term outcomes.J Speech Lang Hear Res. 2019;62(8):2723-2749. doi:10.1044/2018_JSLHR-L-18-0144
Tee BL, Gorno-Tempini ML.Primary progressive aphasia: a model for neurodegenerative disease.Curr Opin Neurol. 2019;32(2):255-265. doi:10.1097/WCO.0000000000000673
Tsai RM, Boxer AL.Treatment of frontotemporal dementia.Curr Treat Options Neurol; 16(11):319.
Jokel R, Meltzer J, D.R. J et al.Group intervention for individuals with primary progressive aphasia and their spouses: Who comes first?J Commun Disord. 2017;66:51-64. doi:10.1016/j.jcomdis.2017.04.002
Coemans S, Struys E, Vandenborre D, et al.A Systematic Review of Transcranial Direct Current Stimulation in Primary Progressive Aphasia: Methodological Considerations.Front Aging Neurosci. 2021;13:710818. Published 2021 Oct 7. doi:10.3389/fnagi.2021.710818
National Institutes for Health. Genetic and Rare Diseases Information Center.Primary Progressive Aphasia: prognosis.
Association for Frontotemporal Degeneration.Find support near you.
National Aphasia Association. Diagnosing Primary Progressive Aphasia. Diagnosing Primary Progressive AphasiaNational Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/National Institutes of Health. Office of Rare Diseases Research. Primary Progressive Aphasia. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8541Northwestern University. Feinberg School of Medicine. Primary Progressive Aphasia. http://www.brain.northwestern.edu/ppa/University of California, San Francisco. Forms of Frontotemporal Dementia. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
National Aphasia Association. Diagnosing Primary Progressive Aphasia. Diagnosing Primary Progressive Aphasia
National Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/
National Institutes of Health. Office of Rare Diseases Research. Primary Progressive Aphasia. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8541
Northwestern University. Feinberg School of Medicine. Primary Progressive Aphasia. http://www.brain.northwestern.edu/ppa/
University of California, San Francisco. Forms of Frontotemporal Dementia. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple
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