Primary Progressive Aphasia – Symptoms and Causes (2024)

Request an appointment

Print

Overview

Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system syndrome that affects the ability to communicate. People who have it may have difficulty expressing their thoughts and understanding or finding words.

Symptoms begin gradually, often before age 65. They get worse over time. People with primary progressive aphasia may lose the ability to speak and write. Finally, they are unable to understand written or spoken language.

This condition progresses slowly. People with primary progressive aphasia can continue to care for themselves and participate in daily activities for years.

Primary progressive aphasia is a form of frontotemporal dementia. Frontotemporal dementia is a cluster of conditions resulting from degeneration of the frontal or temporal lobes of the brain. These areas include brain tissue involved in speech and language.

Symptoms

Primary progressive aphasia symptoms vary depending on which part of the brain's language areas are involved. The condition has three types. Each type causes different symptoms. The symptoms develop over time and become progressively worse.

Semantic variant primary progressive aphasia

The symptoms include:

  • Problems understanding spoken or written language, especially single words.
  • Problems understanding the meaning of words.
  • Inability to name objects.
  • Problems formulating sentences.

Logopenic variant primary progressive aphasia

The symptoms include:

  • Problems understanding spoken language, especially long sentences.
  • Pause and hesitate while speaking as you search for words.
  • Inability to repeat phrases or sentences.

Non-fluent-agrammatic variant of primary progressive aphasia

The symptoms include:

  • Poor grammar in writing and speaking.
  • Problems understanding complex sentences.
  • Uses grammar incorrectly.
  • May have difficulty speaking. This includes making mistakes in speech sounds, also called apraxia of speech.

Primary progressive aphasia of speech is related to primary progressive aphasia, but people with this disorder do not have problems with language. They have difficulty speaking. This includes making mistakes in speech sounds or having difficulty pronouncing words quickly.

When should you consult a doctor?

If you have any concerns about your ability to communicate, contact your healthcare provider. If you have a family member or friend who has symptoms of primary progressive aphasia, talk to the person about your concerns. Offer to accompany the person to a healthcare provider.

If sudden changes in speech or communication occur, call 911 or your local emergency number.

Request an appointment

Reasons

Primary progressive aphasia is caused by the shrinking of certain parts of the brain, also called lobes. In this case, the frontal, temporal or parietal lobes are affected. When parts of the brain shrink, this is called atrophy. The atrophy caused by primary progressive aphasia occurs mainly on the left side of the brain. The affected areas are responsible for speech and language.

Atrophy is associated with the presence of certain proteins in the brain. The proteins may decrease brain activity or function.

Risk factors

Risk factors for primary progressive aphasia include:

  • Learning difficulties.People who had a learning disability in childhood, such as dyslexia, may have a slightly higher risk of primary progressive aphasia.
  • Certain gene changes.Rare gene changes have been linked to primary progressive aphasia. If other members of your family have also had it, you are more likely to get it.

Complications

People with primary progressive aphasia eventually lose the ability to speak and write. This can take 3 to 15 years. They also have difficulty understanding written and spoken language. Some people are unable to form sounds to speak, even if they still have the ability to write and understand language. This is called apraxia of speech.

As the disease progresses, other mental skills such as memory, planning and organization may be affected. Some people develop other symptoms, such as movement, balance and swallowing problems. Because of these complications, people with the disease will eventually need help with daily care.

People with primary progressive aphasia may also develop depression as the disease progresses. Other problems may include blunted emotions, such as not showing concern, poor judgment, or inappropriate social behavior.

Employees of the Af Mayo clinic

Primary progressive aphasia treatment at Mayo Clinic

Request an appointment

1 april 2023

Print

  1. Primary progressive aphasia. National Aphasia Association. http://www.aphasia.org/aphasia-resources/primary-progressive-aphasia/. Accessed February 20, 2023.
  2. Lee SE, et al. Frontotemporal dementia: clinical features and diagnosis. https://www.uptodate.com/contents/search. Accessed February 20, 2023.
  3. Jankovic J, et al., eds. Alzheimer's disease and other forms of dementia. In: Bradley and Daroff's neurology in clinical practice. 8th edition. Elsevier; 2022. https://www.clinicalkey.com. Accessed February 20, 2023.
  4. Budson AE, et al. Primary progressive aphasia and apraxia of speech. In: Memory loss, Alzheimer's disease and dementia. 3rd edition. Elsevier; 2022. https://www.clinicalkey.com. Accessed February 20, 2023.
  5. Baratono S, et al. What are the main diagnostic cognitive disorders and subtypes of dementia and how are all diagnostic data integrated to make a diagnosis? Clinics in geriatric medicine. 2023; doi:10.1016/j.cger.2022.08.002.
  6. Coemans S, et al. Executive functions in primary progressive aphasia: a meta-analysis. Cortex. 2022; doi:10.1016/j.cortex.2022.
  7. Nicolo Aiello E, et al. Primary progressive aphasia and motor neuron disease: an overview. Frontiers in aging neuroscience. 2022; doi:10.3389/fnagi.2022.1003792.
  8. Aphasia. American Speech-Language-Hearing Association. https://www.asha.org/public/speech/disorders/aphasia/. Accessed February 20, 2023.
  9. A diagnosis of primary progressive aphasia? What can you expect? National Aphasia Association. https://www.aphasia.org/stories/ppa-what-to-expect/. Accessed February 21, 2023.
  10. Ami TR. Allscripts EPSi. Mayo-kliniek. Jan. 10, 2023.
  11. Srinivasan J, et al., ed. Dementia: mild cognitive impairment, Alzheimer's disease, Lewy Body dementia, frontotemporal lobar dementia, vascular dementia. In: Netters Neurology. 3rd edition. Elsevier; 2020. https://www.clinicalkey.com. Accessed February 21, 2023.
  12. Tippett DC, et al. Clinical and neuroimaging features of primary progressive aphasia. Handbook of clinical neurology. 2022; doi:10.1016/B978-0-12-823384-9.00016-5.
  13. Tartaglia MC, et al. Recent developments in frontotemporal dementia. Canadian Journal of Neurological Sciences. 2022; doi:10.1017/cjn.2022.69.
  14. Providing care to a person with a frontotemporal disorder. National Institute on Aging. https://www.nia.nih.gov/health/providing-care-person-frontotemporal-disorder. Accessed February 21, 2023.
  15. Support for people with FTD. Association for Frontotemporal Degeneration. https://www.theaftd.org/living-with-ftd/support-for-people-with-ftd/. Accessed February 21, 2023.
  16. Alzheimer's Disease Research Centers. National Institute on Aging. https://www.nia.nih.gov/health/alzheimers-disease-research-centers. Accessed February 20, 2023.
  17. Arizona Alzheimer's Consortium. http://azalz.org/about-us/. Accessed February 20, 2023.
  18. Botha H, et al. Primary progressive aphasia and apraxia of speech. Continuum. 2019; doi:10.1212/CON.00000000000000699.
  19. Utianski RL (expert opinion). Mayo Clinic. February 22, 2023.
  20. Botha H, et al. Primary progressive aphasia and apraxia of speech. Continuum. 2019; doi:10.1212/CON.00000000000000699.

Related

Associated procedures

  • Genetic test
  • DHR
  • Positron emission tomography scanning
  • SPECT scanning

Primary progressive aphasia

  • Care at MayoClinic

Advertisem*nt

Mayo Clinic does not endorse any companies or products. Advertising revenue supports our nonprofit mission.

Advertising and sponsorship

CON-20155110

  • Primary progressive aphasia
Primary Progressive Aphasia – Symptoms and Causes (2024)

FAQs

Primary Progressive Aphasia – Symptoms and Causes? ›

Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. Sometimes, this breakdown starts because of a gene mutation that you're born with.

What is primary progressive aphasia caused by? ›

Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. Sometimes, this breakdown starts because of a gene mutation that you're born with.

How quickly does primary progressive aphasia progress? ›

People with primary progressive aphasia eventually lose the ability to speak and write. This may take anywhere from 3 to 15 years. They also have trouble understanding written and spoken language. Some people are not able to form sounds to speak, even when they still have the ability to write and comprehend language.

What is the life expectancy of someone with primary progressive aphasia? ›

Limited research suggests that those with primary progressive aphasia (PPA) live on average from 7 to 12 years after symptoms onset. However, PPA does not appear to be a direct cause of death. Primary progressive aphasia (PPA) is a rare neurological disorder that involves the gradual deterioration of language skills.

Does primary progressive aphasia get better? ›

Unlike many people who develop aphasia from head injury or stroke, people with PPA do not typically improve with time, but a therapist may be helpful in maximizing abilities and exploring other ways to communicate.

Is PPA a form of dementia? ›

In most cases, PPA is caused by frontotemporal dementia. Most people who develop PPA will be in their 50s and 60s. There are three separate types of PPA: Semantic dementia.

Can you recover from primary progressive aphasia? ›

Primary progressive aphasia can't be cured, and there are no medicines to treat it. However, some therapies might help improve or maintain your ability to communicate and manage your condition.

What is the final stage of PPA? ›

What can you expect in the later stages of PPA? Although PPA primarily affects language and communication skills, it ultimately leads to dementia . This means it will result in broader cognitive decline, including difficulties with memory, problem-solving, and overall thinking abilities.

What are the warning signs of aphasia? ›

Symptoms
  • Speak in short or incomplete sentences.
  • Speak in sentences that don't make sense.
  • Substitute one word for another or one sound for another.
  • Speak unrecognizable words.
  • Have difficulty finding words.
  • Not understand other people's conversation.
  • Not understand what they read.
  • Write sentences that don't make sense.

How rare is primary progressive aphasia? ›

PPA is classified as a rare disease (defined as a condition which affects fewer than 200,000 people in the United States) by the Genetic and Rare Diseases Information Center of the National Center for Advancing Translational Sciences (NCATS) 4.

Can people with primary progressive aphasia drive? ›

Driving. Having a diagnosis of dementia does not necessarily mean a person cannot drive. However, if you have been diagnosed with PPA, or any other form of dementia, you have a legal responsibility to inform the Driver and Vehicle Licensing Agency (DVLA) about your diagnosis.

Does primary progressive aphasia run in families? ›

In the vast majority of individuals, PPA is not genetic. However, in a small number of families, PPA can be caused by hereditary forms of FTLD. The most common gene implicated in these families is the progranulin gene (GRN).

What is the average age for aphasia? ›

Aphasia can affect people of all ages, but it's most common in people over the age of 65.

What is the main cause of aphasia? ›

Aphasia usually happens suddenly after a stroke or a head injury. But it can also come on gradually from a slow-growing brain tumor or a disease that causes progressive, permanent damage (degenerative). The severity of aphasia depends on a number of things, including the cause and the extent of the brain damage.

What are the stages of PPA? ›

Unlike other neurodegenerative disorders, PPA lacks standardized stages. Its progression varies but generally involves worsening language abilities and cognitive impairment over time.

Can medications cause aphasia? ›

... far, several medications have been reported to cause aphasia, including: ipilimumab; immunomodulatory drugs (thalidomide, lenalidomide, pomalidomide); lamotrigine; vigabatrin; sulfasalazine; cyclosporine A; ifosfamide; phenylpropanolamine; naftidrofuryl oxalate; and some con- trast mediums (Table 1).

Top Articles
Latest Posts
Article information

Author: Delena Feil

Last Updated:

Views: 6616

Rating: 4.4 / 5 (45 voted)

Reviews: 92% of readers found this page helpful

Author information

Name: Delena Feil

Birthday: 1998-08-29

Address: 747 Lubowitz Run, Sidmouth, HI 90646-5543

Phone: +99513241752844

Job: Design Supervisor

Hobby: Digital arts, Lacemaking, Air sports, Running, Scouting, Shooting, Puzzles

Introduction: My name is Delena Feil, I am a clean, splendid, calm, fancy, jolly, bright, faithful person who loves writing and wants to share my knowledge and understanding with you.