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Key TakeawaysPrimary progressive aphasia (PPA) is a less common form of dementia, and one that not as many people are aware of.It primarily affects speech and language, with memory loss becoming more apparent further on in the condition.While it’s less common, research into PPA is ongoing and there is support available for people living with the condition and their loved ones.

Key Takeaways

Primary progressive aphasia (PPA) is a less common form of dementia, and one that not as many people are aware of.It primarily affects speech and language, with memory loss becoming more apparent further on in the condition.While it’s less common, research into PPA is ongoing and there is support available for people living with the condition and their loved ones.

According to the Centers for Disease Control and Prevention, around five million adults were living with dementia in the U.S. in 2014.Most of these adults would have been living with Alzheimer’s disease—it’s the most common form, and it’s the one that probably comes to mind for many of us when we think about dementia.

However, there aremany different types—some of which we’re far less familiar with. One such type of dementia is primary progressive aphasia, or PPA.

However, while recovery from some forms of aphasia can be possible, PPA will only get worse over time.

What is Primary Progressive Aphasia?

Whereas we often associate dementia withmemory loss, memory in people with PPA is generally not overly affected until the later stages.

The loss of speech skills often begins subtly, before progressing to an almost complete inability to speak. As the condition progresses, it can begin to resemble other dementias more readily, with changes inmemory,personality, judgment, andattention, and eventually movement and swallowing may be affected.

Charles Marshall, PhD, MRCPWe need to improve awareness that dementia can affect language as well as memory to ensure that people with PPA are diagnosed promptly and accurately.

Charles Marshall, PhD, MRCP

We need to improve awareness that dementia can affect language as well as memory to ensure that people with PPA are diagnosed promptly and accurately.

People tend to be affected with PPA in late-middle-age, meaning that it can be an early-onset dementia.

The age of onset can differ from patient to patient, however, as can the progression of the condition; someone may live for over a decade after diagnosis, and keep a high level of independence for a number of years.

Why there can be so much difference between individuals with PPA is something that scientists are researching.

PPA can, in around 10% of cases, be genetic. This means that “a specific faulty gene might be causing these problems, but this is only the case for a small minority of people with PPA,” saysChris Hardy, PhD, a senior research fellow at the Dementia Research Centre at the University College London Queen Square Institute of Neurology.

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How is PPA Caused?

Because PPA differs from other forms of dementia likeAlzheimer’s, people with the condition can often take care of themselves independently, live their normal life, and even work, while they have the condition.

Charles Marshall, PhD, MRCPWe hope that research to improve this understanding will allow us to develop treatments for these devastating conditions.

We hope that research to improve this understanding will allow us to develop treatments for these devastating conditions.

“Primary progressive aphasias are unusual types of dementia where certain proteins build up in the brain and damage nerve cells in the brain’s language areas,” saysCharles Marshall, PhD, MRCP, a clinical senior lecturer and honorary consultant neurologist at the Wolfson Institute of Population Health.

“We need to improve awareness that dementia can affect language as well as memory to ensure that people with PPA are diagnosed promptly and accurately,” Marshall explains.

“We don’t completely understand why the abnormal proteins accumulate in the brain, or how it is that they selectively damage nerve cells that are important for language. We hope that research to improve this understanding will allow us to develop treatments for these devastating conditions.”

There’s currently more research being carried out into PPA, including at the Dementia Research Centre, something that Hardy hopes “will lead to breakthroughs in the future in terms of diagnosis, care, support, and clinical trials.”

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The Subtypes of PPA

There are three main types of PPA: the nonfluent/agrammatic variant, the semantic variant, and the logopenic variant.

What Are the Symptoms?

With different types of PPA, the symptoms can differ. However, across the condition as a whole, the following are among the initial symptoms to watch out for:

It’s worth remembering that not everyone will have symptoms fitting into one of the three main types, and Hardy explains that these individuals might be described as having a diagnosis of “mixed PPA” or “PPA not otherwise specified.”

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Getting Support

There’s no cure for primary progressive aphasia, but there are ways to help manage the condition.

Some people with PPA find it useful to have speech therapy, while some carry identification cards that explain the condition to other people for ease of communication—particularly when they’re independent enough to be able to go out on their own but perhaps not capable of explaining their condition through speech.

Supporting a loved one with PPA can be difficult, but there are things that you can do to help them.

Do your best to be patient and understanding, checking that you understand what they mean, and speak clearly too. Your loved one might find talking one-on-one easier than talking in larger group settings, or find it easier to write things down than speak.

Hardy suggests joining groups or organizations, like the U.K.-basedRare Dementia Support, while there’s also the U.S.-basedAssociation for Frontotemporal Degeneration.

There are various barriers preventing people from getting the right care, which can include speech and language therapies and even just healthcare providers who are familiar with the condition.

As PPA is still something many people aren’t familiar with, getting the right support can be tricky, but it makes raising awareness all the more important.

What This Means For YouDementia can be difficult to think about or discuss—particularly when it comes to types we don’t know as much about. If you think that you or a loved one might be experiencing symptoms of primary progressive aphasia, speak to your doctor, or get in touch with theDementia Research Centreto find out more about ongoing research into the condition.

What This Means For You

Dementia can be difficult to think about or discuss—particularly when it comes to types we don’t know as much about. If you think that you or a loved one might be experiencing symptoms of primary progressive aphasia, speak to your doctor, or get in touch with theDementia Research Centreto find out more about ongoing research into the condition.

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5 SourcesVerywell Mind 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.Centers for Disease Control and Prevention.What is Dementia?Mesulam M.Primary progressive aphasia.Ann Neurol. 2001;49(4):425-432. doi:10.1002/ana.91Marshall C, Hardy C, Volkmer A, et al.Primary progressive aphasia: a clinical approach.J Neurol. 2018;265(6):1474-1490. doi:10.1007/s00415-018-8762-6National Aphasia Association.Primary Progressive Aphasia.Mesulam Center for Cognitive Neurology and Alzheimer’s Disease.Symptoms & Causes of PPA.

5 Sources

Verywell Mind 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.Centers for Disease Control and Prevention.What is Dementia?Mesulam M.Primary progressive aphasia.Ann Neurol. 2001;49(4):425-432. doi:10.1002/ana.91Marshall C, Hardy C, Volkmer A, et al.Primary progressive aphasia: a clinical approach.J Neurol. 2018;265(6):1474-1490. doi:10.1007/s00415-018-8762-6National Aphasia Association.Primary Progressive Aphasia.Mesulam Center for Cognitive Neurology and Alzheimer’s Disease.Symptoms & Causes of PPA.

Verywell Mind 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.

Centers for Disease Control and Prevention.What is Dementia?Mesulam M.Primary progressive aphasia.Ann Neurol. 2001;49(4):425-432. doi:10.1002/ana.91Marshall C, Hardy C, Volkmer A, et al.Primary progressive aphasia: a clinical approach.J Neurol. 2018;265(6):1474-1490. doi:10.1007/s00415-018-8762-6National Aphasia Association.Primary Progressive Aphasia.Mesulam Center for Cognitive Neurology and Alzheimer’s Disease.Symptoms & Causes of PPA.

Centers for Disease Control and Prevention.What is Dementia?

Mesulam M.Primary progressive aphasia.Ann Neurol. 2001;49(4):425-432. doi:10.1002/ana.91

Marshall C, Hardy C, Volkmer A, et al.Primary progressive aphasia: a clinical approach.J Neurol. 2018;265(6):1474-1490. doi:10.1007/s00415-018-8762-6

National Aphasia Association.Primary Progressive Aphasia.

Mesulam Center for Cognitive Neurology and Alzheimer’s Disease.Symptoms & Causes of PPA.

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