Primary progressive aphasia (PPA)

Primary progressive aphasia (PPA) is a rare brain disease that slowly takes away language. Aphasia means trouble using or understanding words. In PPA, the first and main problem is with speaking, finding words, reading, writing or understanding what others say.^1,8 Memory and daily skills often stay fairly good for the first few years.⁵

PPA is not caused by a stroke. It is caused by a slow disease that damages the language areas of the brain, most often on the left side.^5,6 It usually begins before age 65, so it often affects people who are still working.¹¹ Knowing about PPA can help families get the right diagnosis and the right kind of speech therapy early.

What PPA looks like

PPA starts slowly. At first, a person may pause often to search for words, or use a vague word like "thing" instead of the right one. Over months and years, the problems grow.^5,6

Common early signs include:^1,6

• Trouble finding the right word in a conversation
• Trouble naming objects or people
• Speech that is slow, halting or hard to get out
• Leaving out small words like "to," "from" or "the"
• Mixing up the order of words
• Trouble understanding single words or long sentences
• New problems with spelling, reading or writing

Unlike aphasia after a stroke, PPA does not get better over time. It slowly gets worse.⁵ Many people with PPA know that something is wrong. This can bring frustration, embarrassment or sadness.

The three types of PPA

In 2011, an international group of experts agreed on three main types (also called variants). Doctors name the type by the language skills that are hit hardest at first.³ Some people do not fit neatly into one type.

Semantic type

People slowly lose the meaning of words. They may ask, "What is a fork?" Over time, they may also have trouble recognizing familiar faces and everyday objects.¹ They may misspell words that are not spelled the way they sound.⁶ Many people with this type later develop the behavior changes of frontotemporal dementia.⁷

Nonfluent/agrammatic type

Talking takes a lot of effort. Sentences get shorter and lose their grammar. Many people have apraxia of speech (trouble planning the mouth movements for speech sounds).⁶ Speech may stop completely in time.¹ Some people later develop movement problems like those of corticobasal syndrome, a condition that affects movement and coordination.^1,7

Logopenic type

The person often stops mid-sentence to hunt for a word. Grammar and the meaning of single words are mostly kept.¹ This type is usually caused by the same brain changes as Alzheimer's disease. Over time, it often develops into more typical Alzheimer's symptoms, such as memory loss.^2,7

How common is PPA?

PPA is rare. One review estimated that about 3 to 4 people in every 100,000 have it.⁷ In a registry study, new cases of PPA were about 30 times less common than new cases of typical Alzheimer's dementia.⁷

Symptoms usually begin before age 65.¹¹ PPA is part of the group of frontotemporal disorders. About 6 in 10 people with these disorders are between 45 and 64.¹

What causes PPA?

PPA happens when nerve cells in the language areas of the brain are damaged and die. These areas are mainly in the frontal, temporal and parietal lobes on the left side.^5,6 Abnormal proteins build up and harm the cells. The proteins differ by type, as shown in the table above.⁷

In most people, no clear cause is found.⁶ A small number of cases run in families and are tied to rare gene changes.⁶ In FTD as a whole, about one-third of cases are inherited, often linked to genes called MAPT, GRN and C9orf72.¹ Some research suggests that a childhood learning problem, such as dyslexia, may raise the risk.⁶ If PPA or FTD runs in your family, ask about genetic counseling and testing.

How PPA is diagnosed

PPA is often missed or misdiagnosed.⁴ Its signs can look like other conditions, and many doctors rarely see it.² A neurologist or memory clinic with experience in FTD and language disorders is often the best place to start. See which specialist to see.

Doctors diagnose PPA when three things are true:⁴

• Language gets worse slowly, over time.
• At first, language is the main problem.
• A brain disease (not a stroke or tumor) is the cause.

1. A full medical visit. The doctor asks about symptoms, health history and family history. Blood tests check for other causes.² Bring a family member who has noticed the changes.
2. A language exam. A speech-language pathologist (SLP) or neurologist tests naming, repeating, understanding sentences, grammar, reading and writing.^7,11 This helps decide the type.
3. Thinking tests. Memory, attention and problem-solving are checked. See memory and thinking tests.
4. Brain scans. An MRI can show shrinking in language areas and rule out a stroke or tumor. It may look normal early on. A PET scan can add more detail.² See brain scans.
5. Tests for Alzheimer's. A spinal fluid test or amyloid PET scan can show whether Alzheimer's is the cause. This is common in the logopenic type.^2,7 See spinal fluid tests.

The Association for Frontotemporal Degeneration (AFTD) offers a PPA diagnostic checklist that families can fill out and bring to the doctor.⁴

Treatment: what helps

There is no cure for PPA. There is also no medicine that slows or stops it.¹ Treatment has two goals: keep language skills as long as possible, and learn new tools and ways to communicate.¹

Start therapy early. Tools are easier to learn while language is still fairly strong. Practice now on words and phrases that matter most to the person, such as names of family, favorite foods and medical needs. Plans should change as the disease changes.¹

Find the right therapist. Look for an SLP who knows PPA. Many SLPs are trained mainly for aphasia after stroke, which needs a different approach.¹ Therapy by video from home is an option for many people.⁸

Medicines. No medicine is known to slow or stop PPA.¹ If tests show Alzheimer's disease is the cause, ask the doctor whether any dementia medicines might fit. Do not start or stop any medicine on your own. Talk with your doctor or pharmacist before changing anything.

Does Medicare pay for speech therapy?

Medicare Part B covers outpatient speech-language therapy when a doctor or other qualified provider says it is needed. This includes therapy to keep skills or slow decline, not only to improve.¹⁰ After the yearly Part B deductible, you usually pay 20%. There is no yearly dollar cap on medically necessary therapy.¹⁰ See what Medicare covers in dementia.

Tips for talking with someone who has PPA

More ideas are on our page about talking with someone who has dementia.

How PPA changes over time

PPA gets worse slowly. Over time, many people lose the ability to speak and write. This often happens over about 3 to 15 years.⁶ Many people may stop speaking and lose much of their understanding within about 10 years of diagnosis.¹¹ The pace is different for each person.

As the disease spreads to other parts of the brain, memory, planning and judgment can decline.^1,6 Some people develop behavior changes like those in FTD.¹ Others have problems with movement, balance or swallowing.⁶ In time, most people need help with daily care.⁶

Planning ahead

Because language will fade, planning early matters even more with PPA. While the person can still share their wishes:

• Fill out an advance directive and name a health care agent.
• Set up a durable power of attorney for money matters.
• Talk about driving and work.
• Record stories, favorite phrases and messages for family.

Social Security. PPA is on Social Security's Compassionate Allowances list. This can speed up a disability claim for people who can no longer work.¹¹ See Social Security disability for young-onset dementia.

Support. The AFTD HelpLine (1-866-507-7222) offers information and support for people with PPA and their families.⁴ Care partners often feel loss as the person's voice fades. See grieving someone still here and caregiver stress and burnout. You can also ask about clinical trials for PPA and FTD.

When to get help

• See a doctor soon if you or a loved one has word-finding or speech problems that slowly get worse over months. Ask for a referral to a neurologist or memory clinic.
• Ask for a speech-language therapy referral as soon as PPA is diagnosed.⁸
• See the doctor about new problems, such as coughing or choking when eating, weight loss, falls, or sudden worse confusion.
• If you or the person you care for has thoughts of suicide or feels hopeless, call or text 988 any time. If someone is in danger right now, call 911.

Education only. This page is general information written from the sources listed. It is not medical, legal or financial advice and does not replace a doctor, therapist or lawyer who knows your situation. How we write and check pages.