Primary progressive aphasia (PPA) is a neurological disorder that affects your ability to speak, read, write, and understand others. When you have it, it becomes harder and harder to express your thoughts. Because it’s a progressive condition, your symptoms gradually get worse.
PPA is a type of dementia. Specifically, it’s a type of frontotemporal dementia (FTD), which means it happens due to breakdown of the frontal or temporal lobes of your brain that are involved in speech and language. If you have PPA, it can be a sign you have Alzheimer’s disease.
Primary Progressive Aphasia Types
There are three types, or variants, of PPA. Subtypes of PPA help give families a shorthand for describing symptoms their loved one has. But they’re not a foolproof way to classify the disorder. In fact, research shows up to 30% of people with PPA don’t fit the criteria for a subtype.
But doctors can decide which type you have based on your symptoms. The symptoms you have are based on which part of your brain is affected.
Semantic variant PPA (svPPA)
This type of PPA affects the temporal part of your brain. When you have svPPA, you have trouble with words. Specifically:
- Trouble understanding spoken or written language, particularly single words
- Trouble finding the right word
- Trouble understanding the meaning of words
- Not being able to name objects
- Trouble forming sentences
With svPPA, you may begin to use general words for objects instead of specific ones, such as “animal” instead of “dog.” Over time, your trouble understanding words makes it hard to follow normal conversation.
You may also change personality over time. You may become less aware of or empathetic to other people’s emotions, and you may appear confused and disoriented.
Nonfluent agrammatic PPA (nfaPPA)
Nonfluent agrammatic PPA (nfaPPA) affects the frontal lobe of your brain. When you have nfaPPA, you have trouble pronouncing words or getting words out as you speak.
You might:
- Speak in short phrases
- Leave out small words such as “a” or “the”
- Slur your words
- Have a voice change
- Stop mid-sentence or mid-word
- Mix up words
- Have trouble understanding long or complex sentences
- Have trouble using correct grammar
Typically, when you have nfaPPA, you can still read and write, and you can recognize faces and emotion. Over time, as words are harder to find, you may become more withdrawn and not speak as often.
Logopenic variant PPA (lvPPA)
When you have logopenic variant PPA (lvPPA), you have a hard time finding the words you want to say. This type of PPA typically comes from breakdown in your left posterior temporal cortex and inferior parietal lobe.
Symptoms include:
- Frequent pausing to find the right word when speaking
- Trouble with working memory
- Trouble repeating phrases you just heard
- Trouble reading and writing
Symptoms of Primary Progressive Aphasia
The symptoms of PPA come on gradually, and they slowly get worse over time. Typically, the first symptoms happen before age 65, with an average range of about 50-70 years old.
They include:
- Often struggling to find the correct word for an object
- Constantly pausing while speaking
- Poor grammar
- Slow speech
- Trouble understanding speech or what words mean
- Total loss of language skills
- Mispronouncing words
- Using the wrong word (“table” instead of “chair”)
Primary Progressive Aphasia Stages
In the mild stages of PPA, there may not be much noticeable difference in your manner. You might search for words or pause in speaking occasionally, but still be able to take part in your daily life as before.
When you have PPA, you may be able to care for yourself and do daily activities for several years. In the later stages of the disease, symptoms become more severe and independent living won’t be possible.
These include:
- Talking much less, and the speech you do use is hard to understand
- Trouble understanding others
- Problems making complex decisions (Managing money is difficult.)
- Trouble with judgment, planning, and concentration (Driving is no longer possible.)
- Needing help with basic tasks like washing, dressing, and eating
- Slow and stiff movement
- Poor balance and mobility
- Problems swallowing
- Trouble going to the bathroom
- Loss of social awareness and motivation
- Agitation and, sometimes, aggression
Primary Progressive Aphasia Causes
PPA happens when certain parts of your brain atrophy (shrink). Most of the atrophy in PPA is on the left side of your brain in the frontal, temporal, or parietal lobes. These are the parts of your brain responsible for speech and language.
Scientists link brain atrophy to the presence of certain proteins – tau, TDP-43, or amyloid – in your brain. The buildup of these proteins may reduce brain function in these areas, which is how you lose the ability to speak and communicate.
Certain things put you at a higher risk of getting PPA. These include:
- Childhood learning disabilities such as dyslexia
- Certain gene changes that run in families
Primary Progressive Aphasia Diagnosis
To find out if you have PPA, your doctor will ask about your symptoms. They’ll be especially interested if your symptoms have lasted for a year or longer. Your doctor may ask about your family history as well as your personal medical history to assess your risk of having PPA.
Your doctor can also give you certain tests and scans. These include:
- Neurological exam. This is a series of tests of your mental status, reflexes, nerves, mobility, strength, and coordination to see how well your brain and spinal cord are working.
- Speech-language evaluation. A specialist will look at how well you speak and understand speech. They may also look at how well you can name objects, recognize faces, and remember facts.
- Neuropsychological evaluation. During this exam, your doctor might ask you to write, draw, solve puzzles, or answer certain questions to see how well your brain is functioning.
- Blood test. A blood test can look for other potential causes of your speech problems such as infections or other medical conditions. You’ll also need a blood test to check for a genetic mutation.
- Brain scan. A magnetic resonance imaging (MRI) scan can give your doctor an actual diagnosis of PPA by showing them which parts of your brain tissue are shrinking. Positron emission tomography (PET) is a scan that can show your doctor what your brain looks like as it’s working and reveal parts of your brain that are having trouble with glucose metabolism.
Primary Progressive Aphasia Treatment
PPA doesn’t have a cure, and there aren’t yet any medications that treat it. Because 30%-40% of people with PPA have Alzheimer’s disease, some doctors prescribe drugs that treat Alzheimer’s. But studies haven’t shown that these drugs improve PPA.
Because PPA can cause mental health issues, your doctor may prescribe antidepressant medications, anti-anxiety medications, or medications that soothe agitation to help improve mood.
There are many nondrug ways to help improve someone’s quality of life and ability to communicate when they live with PPA. These strategies include:
Speech therapy. A speech-language pathologist is a specialist who can help you discover and learn new ways to communicate and improve the communication skills you still have.
Occupational and physical therapy. These specialists can help with balance and movement issues that may come along with PPA so you can better manage daily life.
Nonverbal communication tools. Tools such as sign language, visual aids, picture boards, or talk-to-text technology can all be ways to expand communication options for someone with PPA.
Primary Progressive Aphasia Outlook
PPA gets worse over time. The typical life expectancy of someone with PPA is 12 years after diagnosis. As the disease progresses, you lose language skills and the ability to communicate. Eventually, you will need help doing daily tasks.
Some people with PPA eventually aren’t able to make the sounds to speak, even if they can understand written language. This is called apraxia of speech.
Complications of Primary Progressive Aphasia
When you have PPA, you’ll likely have complications including:
- Loss of the ability to speak and write
- Loss of the ability to understand written and spoken language
- Depression
- Poor judgment
- Inappropriate social behavior
- Memory and attention changes
- Movement and swallowing difficulties
Rarely, when you have PPA, you may get other medical conditions such as:
- Parkinsonism – slowed movements, stiffness, and tremors
- Hyperreflexia – muscles that are overactive or have increased movement
- Corticobasal syndrome – a rare disease that causes shrinking in the brain
Takeaways
PPA is a condition that happens when parts of your brain atrophy (shrink) and cause you to lose language skills. It’s a type of dementia, and it may be a sign of Alzheimer’s. Over time, when you have PPA, you may completely lose the ability to talk or write as a form of communication.
Primary Progressive Aphasia FAQs
What’s the life expectancy after being diagnosed with aphasia?
How long you live with PPA depends on several things, including type, speed of progression, and how early you’re diagnosed. People with PPA typically live around 12 years after they first get a diagnosis.
What’s the difference between primary progressive aphasia and dementia?
PPA is a condition that falls under the larger category of dementia. Dementia affects your thinking skills and can affect your memory, personality, and behavior. PPA specifically affects the part of your brain that controls your speech and language and hinders your ability to communicate with other people and understand them.
Can someone with aphasia drive?
In the mild stages of aphasia, driving is often possible. Over time, as the condition gets worse, impaired judgment, mobility, and concentration make driving unsafe.
How quickly does PPA get worse?
The rate of progression varies from person to person, but the typical range of diagnosis to total loss of speech and writing is anywhere from three to 15 years.
