Primary Progressive Apraxia of Speech

Primary Progressive Apraxia of Speech (PPAOS) is a unique syndrome (i.e., a set of symptoms the individual experiences and that are noticeable to others) that can be caused by neurodegenerative brain disease. In PPAOS, the major initial symptom is difficulty with the articulation of speech and problems sequencing sounds within a word (known as “apraxia of speech”). For example, trying to say the word “book”, the individual struggles and says “b-br-koook”.) In the early stages, there is no evidence of difficulties with language, that is, word finding and grammar are normal (no aphasia) but rather communication is hindered by speech output problems.

Primary Progressive Aphasia (PPA) is used to refer to the syndrome in which speech is typically normal but the ability to come up with words and to put them together with proper grammar is difficult. As the brain disease causing the symptoms worsens, it may have an impact on regions close to the speech region, such as the grammar region. Thus some individuals with PPAOS may also develop symptoms of aphasia in addition to the speech production problems as symptoms progress.

There are many different neurodegenerative brain diseases that can cause PPA and PPAOS but the most common cause of PPAOS, as we have learned from studying brain autopsies, is a class of diseases known as 4R tauopathy. The two forms of this disease most common in PPAOS are referred to as “Corticobasal Degeneration (CBD)” and “Progressive Supranuclear Palsy (PSP)” as confirmed microscopically after brain autopsy study.

The individual may notice some difficulty pronouncing words that they are thinking of. Over time speech may become distorted, interfering with normal communication. Individuals with PPAOS, unlike those with PPA, are often able to write what they cannot speak to communicate since, especially initially, the language is normal and may remain normal for some time. Some individuals with PPAOS may over time develop motor limitations in other parts of the body, such as walking or using one’s hands but this is variable from person to person.

Interventions for PPAOS include, but are not limited to, speech language therapy, particularly developing the ability to use alternative communication methods, such as a speech production app. Since individuals with PPAOS do not experience other cognitive or behavioral difficulties, there is also a need to enhance nonverbal skills, such as art or music. Finally, living with PPAOS is challenging for both the affected individual and those who care for/live with them and needed resources change over time. Specialized social work interventions to address these needs are critical. These include effective communication strategies, ways of managing stress, and educating those affected about the illness, what to expect and how to manage over time.