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Primary Progressive Aphasia (PPA): Symptoms, Diagnosing, Treatment

Published: 2010/03/26 - Updated: 2023/02/01
Author: Thomas C. Weiss - Contact: Contact Details
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Synopsis: Primary progressive aphasia (PPA) is a rare form of neurological syndrome in which language capabilities become slowly and progressively impaired. People who are affected maintain other mental abilities as well as activities of daily living for at least two years. PPA is not Alzheimer's disease, and most persons who experience PPA can pursue hobbies, care for themselves, and potentially remain employed.

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Defining PPA

'Aphasia' is an acquired disorder caused by brain damage affecting a person's communication ability. The main signs of aphasia are impairments in the ability to express oneself when speaking, difficulties with understanding speech, and difficulties with writing and reading. Aphasia is often the result of a head injury or stroke, although it may also happen in conjunction with other neurological disorders, including Alzheimer's disease or brain tumor. The effects of aphasia vary among individuals and may be eased through speech therapy. Non-verbal communication strategies can assist in communicating with persons who experience aphasia.

The U.S. Social Security Administration (SSA) has included Primary Progressive Aphasia as a Compassionate Allowance to expedite a disability claim.

Primary progressive aphasia is a rare neurological syndrome in which language capabilities become slowly and progressively impaired. Primary progressive aphasia (PPA) is something that Mesulam and colleagues have identified as a progressive disorder of language. People who are affected maintain other mental abilities as well as activities of daily living for at least two years. PPA is not Alzheimer's disease, and most persons who experience PPA can pursue hobbies, care for themselves, and potentially remain employed. PPA involves a disorder of language; the signs and symptoms of other forms of clinical syndromes are not found through testing that is routinely performed to determine the presence of other conditions.

While PPA can present in various forms, the disorder commonly appears initially as a disorder of speaking, which progresses to a nearly total inability to speak as it reaches its most severe stage. Even so, the person's comprehension remains relatively intact. There is a less common variety of PPA that starts with the person experiencing an impairment related to word finding followed by progressive deterioration of comprehension and naming, with relatively preserved articulation.

There is, however, an additional neurological disorder that exists in which progressive deterioration of language is only one part of a more broad and progressive decline of the person's mental functions that involve attention, memory, visuospatial skills, carrying out of complex motor activities, and reasoning. These neurological diseases include Pick's disease, Cruetzfeld-Jakob disease, and Alzheimer's disease. Appropriate neurological examinations can exclude these diseases when diagnosing PPA in persons experiencing progressive language decline.

Diagnosing PPA

No specific tests lead to a diagnosis of PPA. Current levels of medical knowledge only allow for findings in the affected person's brain that may cause PPA to be detected once the person has died through an autopsy. Instead of direct testing methods, PPA is diagnosed by considering the symptoms the person is experiencing and excluding conditions that may be causing the signs and symptoms. Due to the disorder's complexity, various tests and questions exist for each individual.

To determine which memory and thinking functions might be affected in the person, as well as to what degree, the person is asked questions to measure their cognitive functioning about learning, attention, language, recall, and visuospatial abilities. The results are compared to those of persons who are from the individual's same age and educational range. The person and those familiar with them are interviewed concerning the person's emotional state and day-to-day routines. They are asked about any head trauma, drug or alcohol abuse, or potential causes for memory loss. The person's family members and friends may provide important information concerning the person's personality and behavioral changes.

Speech pathologists are experienced at working with people who experience PPA and using therapies that can assist those affected by the disorder. Psychiatrists can assess and uncover potential depression or other forms of mental illnesses. A sample of the affected person's blood can also be checked for conditions or infections. Conditions such as anemia, medication levels, vitamin deficiency, thyroid disorders, liver or kidney disorders, or additional factors may cause memory loss.

Imaging tests of the person's brain can help to detect tumors, strokes, or other conditions that might have affected their brain. They may show changes to the person's brain's structure associated with memory. Brain imaging tests that may be used include Magnetic Resonance Imaging (MRI), Positron Emission Tomography (PET), or Computed Tomography (CT) scans. Most people with signs and symptoms of PPA will be asked to pursue an MRI, although some may also have other imaging tests performed. A doctor will discuss the most appropriate tests to establish the correct diagnosis. Additional forms of tests can include:

Treating PPA

Medical science has not yet discovered a cure for primary progressive aphasia (PPA). There are several medications, as well as other approaches, that have the potential to improve the quality of life and daily functioning of persons who have been affected by PPA. One effective management technique is a coordinated approach to the person's treatment options, one of which is speech therapy.

The main goal related to treating language impairment in persons with PPA is improving their ability to communicate with others. The language problems experienced by persons with PPA can vary; the treatment's focus on improving communication abilities also varies. There is a need for a complete speech and language evaluation to determine the treatment type most appropriate for the particular individual.

The fact that there is no cure for the degenerative diseases that cause PPA means that medical treatments commonly involve managing behavioral symptoms. These symptoms can include anxiety, agitation, or depression, which can happen in the later stages of the disorder. The medications approved for treating amnestic dementia related to Alzheimer's disease include cholinergic agents targeting the cholinergic loss demonstrated in the disease. The likelihood of the pathology in PPA being similar to that found in Alzheimer's disease is low. No scientific evidence supports using Alzheimer's medications for persons with PPA. One rationale for the treatment of PPA comes from studies of the treatment of aphasia as a result of a stroke. While PPA involves a degenerative and progressive condition, similar language areas are affected in persons with PPA and those with aphasia because of cerebrovascular disease. Because of this, there is potential for the administration of dopamine agonists such as bromocriptine or noradrenergic agents.

There are a couple of basic approaches to treating PPA. One of them is to focus treatment on the language skill impairments the person is experiencing. The other is to provide the person with augmentative/alternative communication devices or strategies. Approaching both strategies is often recommended. In the early stages of the disease, providing treatment that enhances the person's verbal language skills is preferable. For example, treatments that focus on the person's word-retrieval skills can be helpful.

Treatment with a focus on augmentative/alternative means of communication can also be provided to persons with PPA, even in the early stages of the disorder. These strategies involve the enhancement of verbal communication or its replacement. It is suggested that the person and family members and friends obtain training in augmentative/alternative communication strategies, including using gestures, drawing, or a communication notebook.

The results of the person with PPA's language and speech evaluation will determine which strategy or strategies are the best. Practicing these strategies can help the person, and their family members and friends determine which ones are best for them. Follow-up treatments with a speech-language pathologist are important for the further development of appropriate strategies, as well as for practice in using them.

Author Credentials:

Thomas C. Weiss is a researcher and editor for Disabled World. Thomas attended college and university courses earning a Masters, Bachelors and two Associate degrees, as well as pursing Disability Studies. As a Nursing Assistant Thomas has assisted people from a variety of racial, religious, gender, class, and age groups by providing care for people with all forms of disabilities from Multiple Sclerosis to Parkinson's; para and quadriplegia to Spina Bifida.

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Cite This Page (APA): Thomas C. Weiss. (2010, March 26). Primary Progressive Aphasia (PPA): Symptoms, Diagnosing, Treatment. Disabled World. Retrieved October 4, 2023 from www.disabled-world.com/health/neurology/primary-progressive-aphasia.php

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