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Primary Progressive Aphasia: Causes, Symptoms, and Treatment
Written by Joe Carney
about the author
8 minute readLast updated April 18, 2023
Aphasia is brain damage that makes it hard to speak, read, write, and understand spoken words. It affects 2 million people in the U.S. and is more common than Parkinson’s disease, according to the National Aphasia Association. One type of aphasia that leads to dementia is primary progressive aphasia or PPA. Read on to learn more about PPA, including symptoms, risk factors, diagnosis, treatment, and what to do after a diagnosis.
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What is primary progressive aphasia?
Primary progressive aphasia (PPA) is a type of dementia that progressively makes it harder to speak, read, write, and follow conversations. The causes of PPA are relatively unknown but thought to be related to environmental or genetic factors which cause damage to the frontal and temporal regions of the brain, according to the Cleveland Clinic. PPA is estimated to affect as many as 30,000 people in the U.S., according to the Genetic and Rare Diseases Information Center. However, data on PPA is limited because it’s a rare condition, so PPA may affect fewer people. Most people with primary progressive aphasia start showing symptoms in their 50s or 60s, depending on the type of PPA they have.
Symptoms and types of PPA
Most experts currently recognize three kinds of PPA: semantic, logopenic, and nonfluent/agrammatic. Symptoms vary from patient to patient but typically develop later in life, between ages 50-70. If you or your relative is starting to show any of the symptoms below — or a loss of any other language skills — speak with your or your loved one’s doctor. Patients with logopenic PPA may also say things that sound like words, but aren’t. This can sound similar to developmental speech patterns in children.
1. Semantic PPA
Symptoms:- Difficulty remembering the names of specific people, places, or things.
- Trouble following conversations
2. Logopenic PPA
Symptoms:- Mispronouncing words
- Repeating words or phrases
- Difficulty finding the right word
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3. Nonfluent/agrammatic PPA
Symptoms:- Slurring words
- Trouble forming sentences
- Pausing in the middle of sentences or words
PPA risk factors
While the scientific community doesn’t perfectly understand the risk factors of PPA, there are a couple worth noting: Among PPA patients with GRN mutations, logopenic PPA is the most common, according to a study from Neurology.
Genetic predisposal
PPA may be linked to mutations of the GRN gene. However, some patients who have these gene mutations might not develop PPA. Even without GRN mutations, a person may still be at greater risk if PPA runs in their family.Learning disabilities
Childhood learning disabilities, specifically dyslexia, are higher among those with PPA compared to other types of dementia, according to a study from the Archives of Neurology. Dyslexia early in life is thought to signal a vulnerability in the language centers of the brain in patients with PPA.How PPA is diagnosed
If a doctor thinks you or your loved one may have PPA based on current symptoms, they can find out through a series of steps. First, a doctor will evaluate the patient’s risk factors, like medical and family history. Then, they can take brain scans, like MRIs and CT scans, to identify damage to the frontotemporal regions of the brain to give a diagnosis.
Treatments for PPA
While there aren’t any known cures for PPA, there are two main ways that patients can manage their symptoms and potentially slow the disease’s progression. Treatments for managing PPA include: Unfortunately, PPA is a progressive condition that doesn’t improve with time, as other aphasias can. Therefore, the goal of speech therapy is to maintain language skills for as long as possible, rather than to rebuild lost skills. In speech therapy, patients can do exercises that keep their skills as sharp as possible. A speech-language pathologist can also recommend some communication tools for dealing with lost skills.
- Speech therapy
- Medications for Alzheimer’s disease, if Alzheimer’s is the root cause
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What to do after a PPA diagnosis
After a PPA diagnosis, it’s important to understand the patient’s future wishes while their communication skills are at their best. Many PPA patients start showing symptoms before they’re seniors, so it’s also important to know their future care preferences as early as possible. Establishing a power of attorney after a PPA diagnosis can be a crucial step. This ensures that the person’s wishes are clearly outlined. It also designates a trusted individual to represent the person with PPA in legal, financial, or medical affairs down the road.
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References
Cleveland Clinic. (2022, April 12). Primary progressive aphasia (PPA).
Genetic and Rare Diseases Information Center. (2021, November 8). Primary progressive aphasia.
Montembeault, M., Brambati, S. M., Gorno-Tempini, M. L., & Migliaccio, R. (2018, August 21). Clinical, anatomical, and pathological features in the three variants of primary progressive aphasia: a review. Frontiers in Neurology.
National Aphasia Association. Aphasia FAQs.
Rogalski, E., Johnson, N., Weintraub, S., & Mesulam, M. (2008, February). Increased frequency of learning disabilities in patients with primary progressive aphasia and their first-degree relatives. Archives of Neurology.
Saracino, D., Géraudie, A., Remes, A. M., Ferrieux, S., Noguès-Lassiaille, M., Bottani, S., Cipriano, L., Houot, M., Funkiewiez, A., Camuzat, A., Rinaldi, D., Teichmann, M., Pariente, J., Couratier, P., Boutoleau-Bretonnière, C., Auriacombe, S., Etcharry-Bouyx, F., Levy, R., Migliaccio, R., Solje, E., & Le Ber, I. (2021, July 6). Primary progressive aphasia associated with GRN mutations: new insights into the nonamyloid logopenic variant. Neurology.
University of California San Francisco Weill Institute for Neuroscience. Speech and language.
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