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To understand the diagnosis of PPA, it’s helpful to know about the history of the word “dementia.” Prior to the 1980’s, it was assumed that the word “dementia” was synonymous with Alzheimer’s disease, which initially impacts remembering new information and gets worse over time. In 1982, Dr. Marsel Mesulam first described the condition of PPA, where memory is preserved, but language becomes more impaired over time.

 

PPA is a type of clinical dementia syndrome and has the same underlying causes as more well-known dementias, such as Alzheimer’s dementia. If the disease begins in the hippocampus, the region of the brain that controls memory, it results in the dementia syndrome of Alzheimer’s dementia. If the disease begins in the language region of the brain, it results in the dementia syndrome of primary progressive aphasia. PPA symptoms worsen over time as atrophy spreads to other parts of the brain, as in Alzheimer’s dementia. Symptoms such as memory loss and behavioral changes may arise. Families often need additional counseling to fully understand the diagnosis and prognosis of PPA.

 

There is no cure for PPA and the cause is unknown. Like Alzheimer’s disease, researchers believe that both environmental factors and genetic risk may play a role. It is helpful to refer to the literature regarding risk factors and prevention of Alzheimer’s disease, as this research is applicable to PPA. Lifestyle factors to promote general brain health, such as diet, exercise, socialization and participating in cognitively stimulating activities, are important considerations; however, we need more well-designed research into modifiable risk factors.

What are the main symptoms of PPA?

In short, people living with PPA may have difficulty with speaking (pulling out words during conversation or grammatically constructing a sentence), comprehension (understanding/processing what others are saying during conversation), reading and writing. As PPA progresses, other cognitive deficits may arise.

 

PPA has 3 described variants, depending on where the disease starts in the language region, resulting in specific language symptoms.

 

  • Semantic PPA – people have difficulty with comprehending language when others speak. This isn’t a hearing problem, but rather a processing problem in the brain. They may also have difficulty linking meaning to words, resulting in difficulty pulling out specific words in conversation.
  • Nonfluent/agrammatic PPA – people have difficulty with the grammatical construction of sentences. Speech may be effortful, and they may have trouble with pronunciation.
  • Logopenic PPA – people struggle with retrieving the right words in conversation and may frequently talk around the missing word

 

Many people living with PPA may exhibit symptoms from multiple variants, particularly as the disease progresses. It is important that families do not focus on the variant, but rather consider the individual’s language strengths vs. challenges and how these impact daily communication. Researchers continue to study the rate of progression for PPA; there is much individual variability in symptom presentation and progression.

What can people who’ve just been diagnosed with PPA or their loved ones do to help manage the condition?

While there is no cure for PPA, both pharmacological and non-pharmacological interventions may be considered to promote quality of life. These interventions must be tailored for each individual.

 

  • Pharmacological interventions: physicians may prescribe medications to help with symptoms of anxiety/depression, since people living with PPA often have preserved insight into their condition and prognosis.
  • Non-pharmacological interventions: It is important that a multidisciplinary team of professionals work together to help maximize the quality of life for the person with PPA and family members.
    • Social workers consider psychosocial factors that are impacting the person and family. They may provide disease education, counseling, care partner training and support, and connection with local resources.
    • Speech-Language Pathologists can help develop specific strategies to maximize communication during daily conversations and in desired activities. Speech therapy interventions may include personalized language exercises that target words and sentences used in daily conversations. These exercises aim to strengthen existing speech/language and cognitive abilities to potentially slow decline. Compensatory tools may be developed to supplement language. High-tech supports may include using a smart tablet to help with communication. Some individuals engage in “voice/message-banking,” which allows one to record their own speech in earlier stages. The stored messages may be used to help communicate when speaking becomes difficult. People with PPA may also engage in “story telling” and record personal stories on their smart device to share with others.
    • Low-tech supports may include a communication book, with key words and pictures of important people, places, and topics. Care partner training on how to support their loved one during conversations is also critical. The strategies need to be modified over time to meet the individual’s changing communication needs.
    • Occupational therapists and physical therapists may help with home safety, mobility, and activities of daily living.
    • Additional therapies: Support groups, intergenerational therapy, art therapy, music therapy, pet therapy, and others may all be considered, depending on the person’s interests.
  • Increasing awareness about PPA is critical, as many barriers prevent people with PPA from receiving the care they need (e.g., access to healthcare providers who are familiar with PPA; healthcare coverage of ongoing speech and language treatment as the disease progresses)