Treatments for primary progressive aphasia are aimed at managing symptoms, enhancing communication, and improving quality of life.

Primary progressive aphasia (PPA) is a rare neurological disorder caused by the gradual degeneration of brain cells, specifically in areas related to language processing.

The disease leads to a gradual decline in your language skills, including speaking, understanding words, and forming sentences. However, your ability to remember and understand nonverbal concepts and communication may remain relatively intact, particularly during the early stages.

While no cure exists, treatments are available to effectively manage symptoms and enhance communication strategies.

While research has increased our understanding of primary progressive aphasia (PPA)’s underlying mechanisms, the primary treatment emphasis remains on alleviating symptoms, enhancing communication strategies, and enhancing the overall quality of life for affected individuals.

There’s no cure for PPA, but these approaches may help:

  • Speech and language therapy: Studies show that training programs that emphasize language skills (e.g., sentence production, naming tests, verbal fluency) can be helpful for people with PPA.
  • Communication strategies: Learning alternative methods of communication, such as using gestures, writing, or trying augmentative and alternative communication (AAC) devices, can help improve communication as language abilities begin to decline.
  • Transcranial direct current stimulation (tDCS): This noninvasive technique involves applying a mild electrical current to the scalp to modulate brain activity. A review shows that tDCS may help improve language skills, but further research is needed.
  • Medications: Current research indicates that medications that have been examined as possible treatments for PPA have limited effectiveness in improving cognitive deficits. However, certain medications, such as antidepressants, anti-anxiety medications, sleep aids, antipsychotics, cholinesterase inhibitors (ChEIs), and N-Methyl-D-aspartate receptor antagonists (NMDA antagonists), may be used for various symptoms related to PPA.

The variants and stages of PPA play an important role in treatment planning.

There are three main variants of PPA:

  • Nonfluent/Agrammatic PPA: This variant involves trouble speaking smoothly, making grammar mistakes and sentence structure errors, while understanding and recalling single words are mostly unaffected. This variant is associated with frontotemporal lobar degeneration (FTLD).
  • Semantic PPA: In this variant, there’s a slow decline in understanding words and objects, which leads to problems comprehending language, recognizing things, and connecting related information. This variant can be associated with frontotemporal lobar degeneration (FTLD) and may share characteristics with Alzheimer’s disease (AD).
  • Logopenic PPA: This variant is marked by word-finding difficulties, slow speech rate, and frequent pauses, often accompanied by impaired working memory, while grammar and comprehension are relatively preserved. About 86% of cases of lvPPA are linked to Alzheimer’s pathology and can be considered an atypical variant of early-onset AD.

Unlike some other neurodegenerative diseases like Alzheimer’s or Parkinson’s, PPA doesn’t have a standardized set of stages that everyone follows.

In a study that has been preprinted (not yet peer-reviewed), researchers aimed to establish a clear framework for understanding the progression of PPA.

They asked caregivers of people with PPA to describe the symptoms and how they change over time. Based on the responses, they came up with six stages for each form, ranging from 1 (very mild) to 6 (profound).

Here are the stages, according to the study:

  • Stage 1: Very mild: Symptoms may not be recognized and could be mistaken for stress or aging. They may be sporadic, hard to describe, and only recognized in hindsight.
  • Stage 2: Mild: Communication difficulties and other subtle challenges in daily activities become noticeable to you and to others.
  • Stage 3: Moderate: You might need help with daily tasks and could cease working. Communication difficulties might affect your goals and social interactions, leading to frustration.
  • Stage 4: Severe: During this stage, communication might become significantly more challenging. Living independently could become difficult or impossible.
  • Stage 5: Very severe: You may notice broader cognitive and behavioral changes and that meaningful communication becomes increasingly rare. You might require assistance with personal care and experience more pronounced physical symptoms (coordination, walking).
  • Stage 6: Profound: At this point, communication may become nearly impossible. You might also find it challenging to respond to your surroundings and may experience a significant decrease in mobility.

How quickly does primary PPA?

The progression of primary progressive aphasia can vary widely from person to person. On average, it tends to develop gradually over several years, often ranging from 2 to 10 years or more.

However, the rate of progression can be influenced by factors such as the specific variant of PPA, individual differences, and underlying causes.

The survival rate for PPA varies depending on the specific variant and individual factors.

One study investigated the survival rates of 83 deceased individuals with different variants of PPA after onset:

  • Non-fluent variant (nfvPPA): average survival of 7.1 years
  • Logopenic variant (lvPPA): average survival of 7.6 years
  • Semantic variant (svPPA): average survival of 12 years

Common causes of death included natural cardiopulmonary arrest and pneumonia. Notably, nfvPPA cases often experienced aspiration pneumonia.

Primary progressive aphasia (PPA) is a rare neurological condition that primarily affects language abilities, causing difficulties in speaking, understanding, and expressing thoughts.

While there’s no cure, treatment focuses on managing symptoms and improving communication skills through interventions like speech therapy, cognitive training, and supportive strategies.

These treatments can help you maintain your ability to communicate, engage in daily activities, and enhance your overall quality of life.