Explaining Primary Progressive Aphasia: Neurological Condition Affecting Bruce Willis Is Often Misdiagnosed, Misunderstood

(Rockville, MD) With primary progressive aphasia (PPA) once again in the spotlight following Emma Heming Willis’s recent appearance on The Today Show, the American Speech-Language-Hearing Association (ASHA) is sharing information about the signs of PPA, how it differs from other conditions, and how speech-language pathologists (SLPs) can help families improve communication with—and overall quality of life for—loved ones who are diagnosed.

What Is PPA?

PPA, the disease that actor Bruce Willis has, is a rare and lesser-known neurological condition. It is caused by changes in the frontal and temporal lobes of the brain, which are largely responsible for language and executive functioning. These areas also act as the body’s center for making decisions and navigating daily life and activities.

PPA is more likely to occur in people in their 40s to 60s. There is no cure or direct treatment, but SLPs can help people manage the changes that occur in communication and thinking—and put supports and care plans in place for addressing the progression of the disease (see “How SLPs Can Help,” below).

How Is PPA Different From Other Conditions?

PPA is not the same as the following diseases or conditions:

• Aphasia caused by stroke or other brain injuries. PPA is progressive, so the communication challenges may start slowly—not suddenly—and will get worse over time.
• Alzheimer’s disease and other forms of dementia, where memory changes are the earliest and most recognizable differences. PPA primarily impacts language and communication skills as the earliest signs.
• Depression or anxiety, although some of the communication or behavioral changes may be similar. Someone with PPA who has trouble interacting with others may socially withdraw or show changes in personality, which can lead to an early but inaccurate diagnosis of mental health disorders.

Communication Changes With PPA

Some common communication changes that people with PPA may experience early in the disease include:

• talking or signing slower than usual;
• having difficulty thinking of words, even the names of familiar objects and people;
• leaving words out or mixing up the order of words in sentences;
• using a different word than the one they mean (e.g., “table” instead of “chair”);
• having difficulty understanding what words mean;
• struggling to follow a conversation; and/or
• making mistakes in spelling that they wouldn’t typically make.

How SLPs Can Help

SLPs help by learning an individual’s communication priorities early—and then putting systems in place that are supportive as the disease progresses. As Emma Heming Willis said, PPA happens to the whole family. Consequently, SLP services extend beyond the person with PPA. They include any or all of the following:

• Explaining the communication changes that a person is experiencing—and finding strategies that support their participation in the things they want, need, and love to do.
• Introducing communication strategies and tools that may not be needed today but will be in the future—so that they are well established before the person needs them.
• Pairing different communication situations with different strategies, like using quick gestures or other signals for medical needs and recording the person’s speech for later use for personal messages like their favorite dad jokes and bedtime stories.
• Working with the person’s family and friends to be supportive communication partners—and adapting to changes as they occur.
• Empowering the person to advocate for their communication needs everywhere they go—and helping connect the person with resources and new communities.
• Helping a person maintain effective communication, which may look different as their condition progresses.

How Families Can Support Communication

People with PPA may be unable to fully realize their communication challenges. It’s important for loved ones to continue to include the person in conversations and decision making. Tips for how to do that include the following:

• Validate frustrations, and use supports like writing or drawing to make the interaction more successful. Communication breakdowns are particularly hard for people in the earlier stages of the disease.
• Slow down when you are speaking with someone who has PPA. Use direct language, and keep sentences short.
• Give the person time to communicate (rather than finishing the sentence for them).
• Add nonverbal cues to your speech (e.g., pointing to an object while asking a question about it) if a person’s understanding is affected.
• Avoid open-ended questions, and provide choices to pick from.
• Break down directions into small steps.

Find an SLP at www.asha.org/profind. 

About the American Speech-Language-Hearing Association (ASHA)
ASHA is the national professional, scientific, and credentialing association for 228,000 members and affiliates who are audiologists; speech-language pathologists; speech, language, and hearing scientists; audiology and speech-language pathology assistants; and students. Audiologists specialize in preventing and assessing hearing and balance disorders as well as providing audiologic treatment, including hearing aids. Speech-language pathologists (SLPs) identify, assess, and treat speech, language, and swallowing disorders.