Primary Progressive Aphasia

What is Primary Progressive Aphasia?

Primary progressive aphasia (PPA) is a rare neurological syndrome that affects a person’s ability to communicate. People who have it can have trouble understanding spoken and/or written information as well as problems expressing their thoughts and difficulties finding words. Symptoms begin gradually, often before age 65, and worsen over time. People with PPA can lose the ability to speak and write and, eventually, lose the ability to understand written or spoken language. Primary Progressive Aphasia progresses slowly, so you may continue caring for yourself and participating in daily life activities for several years after the disorder's onset.

Primary progressive aphasia is a type of frontotemporal dementia, a cluster of related disorders that results from the degeneration of the frontal or temporal lobes of the brain, which include brain tissue involved in speech and language.  (modified from mayoclinic.org)

Symptoms of Primary Progressive Aphasia:

Primary progressive aphasia symptoms vary, depending on which portion of the brain's language areas are involved. The condition has three types, which cause different symptoms.

Individuals with Semantic variant primary progressive aphasia may present with:

• Difficulty comprehending spoken or written language, particularly single words
• Trouble comprehending word meanings
• Struggling to name objects

Individuals with Logopenic variant primary progressive aphasia may present with:

• Difficulty retrieving words and word substitutions
• Frequently pausing in speech while searching for words
• Difficulty repeating phrases or sentences

Individuals with Non-fluent agrammatic variant primary progressive aphasia may present with:

• Poor grammar in written and spoken form
• Trouble understanding complex sentences
• Using grammar incorrectly
• May be accompanied by speaking problems such as errors in speech sounds (known as apraxia of speech)

Other symptoms include:

• Losing the ability to speak and write, and to understand written and spoken language. Some people develop substantial difficulty forming sounds to speak (a problem called apraxia of speech), even when their ability to write and comprehend are not significantly impaired.

As the disease progresses, other mental skills, such as memory, can become impaired. Some people develop other neurological symptoms such as problems with movement. With these complications, the affected person eventually will need help with day-to-day care.

People with primary progressive aphasia can also develop depression or behavioral or social problems as the disease progresses. Other problems might include blunted emotions such as unconcern, poor judgment or inappropriate social behavior. (mayoclinic.org)

Causes of Primary Progressive Aphasia

Primary progressive aphasia is caused by a shrinking (atrophy) of certain sections (lobes) of the brain responsible for speech and language. In this case, the frontal, temporal or parietal lobes, primarily on the left side of the brain, are affected.

Atrophy is associated with the presence of abnormal proteins, and brain activity or function in affected areas might be reduced.

If you had a childhood learning disability, particularly developmental dyslexia, you might be at somewhat higher risk of primary progressive aphasia.

Certain rare gene mutations have also been linked to the disorder. If other members of your family have had primary progressive aphasia, you might be more likely to develop it. (modified from mayoclinic.org)

Speech-Language Pathology Evaluation & Therapy

Speech-Language Pathologists (SLP) have specialty training in evaluating individuals with primary progressive aphasia.

A speech-language pathologist will evaluate listening, speaking, reading and/or writing and will assess functional communication.  A SLP will consider input from family and other caregivers through a detailed clinical interview.   A speech language pathologist will also incorporate individualized goals and will work to help individuals with PPA cope with the diagnosis and optimize quality of life. 

Additional Information:

• National Aphasia Association - PPA
• National Aphasia Association – Understanding PPA
• UCSF – Weill Institute for Neurosciences – Primary Progressive Aphasia

Videos:

Living with LPA (Logopenic PPA): YouTube video

Research:

Northwestern – Join a Research Study for PPA - Join a Study: Mesulam Center for Cognitive Neurology and Alzheimer’s Disease: Feinberg School of Medicine: Northwestern University