Primary Progressive Aphasia (PPA) - Facts and Information
Author: Thomas C. Weiss : Contact: disabled-world.com
Published: 2010-03-26 : (Rev. 2018-03-19)
Synopsis and Key Points:
Primary progressive aphasia is a neurological syndrome in which language capabilities become progressively impaired.
Primary progressive aphasia is a rare form of neurological syndrome in which language capabilities become slowly and progressively impaired...
'Aphasia,' is an acquired disorder caused by brain damage which affects a persons ability to communicate. The main signs of aphasia are impairments in the ability to express oneself when speaking, difficulties with understanding speech, as well as difficulties with writing and reading. Aphasia is many times the result of a head injury or stroke, although it may also happen in conjunction with other neurological disorders, to include 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.
Primary progressive aphasia is a rare form of neurological syndrome in which language capabilities become slowly and progressively impaired. Primary progressive aphasia (PPA) is something that has been identified by Mesulam and colleagues as a progressive disorder of language. People who are affected maintain other mental abilities as well as activities of daily living for a period of at least two years. PPA is not Alzheimer's disease, and the majority of persons who experience PPA keep the ability to 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 in order to determine the presence of other conditions.
While PPA can present in a variety of 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 are; however, additional neurological disorder that exist in which progressive deterioration of language is only one part 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 forms of diseases when attempting to make a diagnosis of PPA in persons experiencing progressive language decline.
There are no specific tests that 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. In leiu 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 complexity of the disorder there are a variety of tests and questions for each individual.
In order to determine which memory and thinking functions might be affected in the person, as well as to what degree, the person is asked questions with the goal of measuring their cognitive functioning in relation to 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, as well as their 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 the use of therapies that can assist those affected by the disorder. Psychiatrists can make assessments 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 structure of the person's brain that are associated with memory. Brain imaging tests that may be used include Magnetic Resonance Imaging (MRI), Positron Emission Tomography (PET), or Computed Tomography (CT) scans. The majority of people with signs and symptoms of PPA will be asked to pursue and MRI, although some people may have other forms of imaging tests performed as well. A doctor will discuss the tests that are most appropriate in order to establish the correct diagnosis. Additional forms of tests can include:
- Electroencephalogram (EEG)
- Electromyogram (EMG)
- Urine Tests
- Cerebrospinal Fluid Tests (CSF)
Medical science has not discovered a cure for primary progressive aphasia (PPA) at this time. There are a number of 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 treatment of language impairment in persons with PPA is the improvement of their ability to communicate with others. The language problems experienced by persons with PPA can vary; because of this, the focus of the treatment for improvement of the communication abilities also varies. There is a need for a complete speech and language evaluation to determine the type of treatment that is 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 the realm of managing behavioral symptoms. These symptoms can include anxiety, agitation, or depression, which can happen in the later stages of the disorder. The medications that have been approved for treating amenestic dementia related to Alzheimer's disease include cholinergic agents that are targeting the cholinergic loss which has been demonstrated in the disease. The likelihood of the pathology in PPA being similar to that found in Alzheimer's disease is low. There is no scientific evidence to support the use of Alzheimer's medications for persons with PPA. One rationale for treatment of PPA comes from studies of the treatment of aphasia as a result of stroke. While PPA involves a degenerative, and therefore progressive condition, similar language areas are affected in both 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 the treatment of 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 oftentimes recommended. In the early stages of the disease, provision of treatment that enhances the person's verbal language skills is preferable. Treatments that focus on the person's word-retrieval skills, for example, can be helpful.
Treatment with a focus on the use of augmentative/alternative means of communication can also be provided to persons with PPA, even while they are in the early stages of the disorder. These strategies involve the enhancement of verbal communication, or its replacement. It is suggested that the person, as well as family members and friends, obtain training in augmentative/alternative communication strategies that can include the use of 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. Practice in using these strategies can the person and their family members and friends to determine which ones are best for them. Follow-up treatments with a speech-language pathologist is important for further development of appropriate strategies, as well as for practice in using them.
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