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PPA Diagnosis

How is PPA diagnosed? What are the guidelines for diagnosis recommended and used by most medical research centers?

1. History:

First, a careful history is taken to establish that a condition of dementia, as defined above, exists. This often requires that family members or friends be questioned about the patient’s behavior because sometimes the patient is unaware of the symptoms (as in the case of memory loss or personality changes) or may be unable to describe them due to aphasia.

2. Neurological Examination:

A neurological examination is done to determine if there are signs of dementia on a simple screening of mental functions (the mental status examination) and also if there are signs of motor or sensory symptoms that indicate other types of neurological disorders might be causing the dementia. The neurologist will also order tests, such as a magnetic resonance imaging (MRI) scan, to make sure that the symptoms are not due to factors such as stroke or tumor.

3. Neuropsychological Examination:

A neuropsychological examination provides a more detailed evaluation of mental functioning. This is especially important in the very early stages of illness when a routine screening evaluation may not detect the problems the patient is experiencing. This requires several hours and consists of paper-and-pencil or computer-administered tests of mental abilities, including attention and concentration, language, learning and memory, visual perception, reasoning and mood. The results can indicate if there are abnormalities of thinking and behavior and also their degree--mild, moderate or severe. It is often difficult to demonstrate that individuals with PPA have intact memory since we usually test memory by telling a person some information and then asking them to repeat it later on. In an individual with PPA, it may be impossible to repeat back the information because of the aphasia. Therefore, it is important that testing is done properly to make sure that there is not a true loss of memory. To date, neuropsychological evaluation is the only way to objectively identify a dementia. Currently, there are no blood tests or other physiological tests that are specific for dementia. This is especially important when symptoms are in the very early stages or mild.

4. Speech and Language Evaluation:

Since a decline in language abilities is the primary symptom of PPA, it is important to determine which components of language use are most affected, how severely affected they are, and what can be done to improve communication. A Speech-Language Pathologist evaluates different aspects of language in detail and can make recommendations for strategies to improve communication. Family members should be included in the treatment sessions to educate them about how to facilitate communication.

5. Psychosocial Evaluation:

PPA affects not only the individual who is suffering from this disorder, but also all people who are close to the patient. The disorder has an impact on relationships, the ability to continue working, the ability to perform many routine duties, and the ability to communicate even the simplest of needs. Although there are many resources available for individuals with memory loss, there are relatively fewer appropriate resources for individuals with PPA, their relatives and friends. Evaluation with a social worker who is familiar with PPA can address these issues and provide suggestions for dealing with day-to-day frustrations and problems.

6. Brain Imaging Studies:

The evaluation for dementia also includes a brain imaging study. This is done in the form of a computed axial tomography scan (CAT scan) or a magnetic resonance imaging scan (MRI scan). Both of these methods provide a picture of the brain so that any structural abnormalities, such as a stroke, tumor or hydrocephalus--all of which can give rise to dementia-like symptoms, can be detected. In the case of degenerative brain disease, the CAT scan and MRI scan may show “atrophy,” which suggests a “shrinkage” of the brain tissue, or, especially in early stages, they may not show anything. See Figure 2 below. In fact, the report often comes back “normal.” But this only means that there is no evidence for a tumor or stroke. It cannot tell us anything about the microscopic degenerative changes that have occurred.

7. Psychiatric Evaluation:

Sometimes there will also be a need for a psychiatric evaluation. This may be the case when it is not clear if the changes in behavior are due to depression or another psychiatric disturbance. Also, some individuals, especially those with PPA, may become saddened by their condition and may require treatment for depression.

Figure 2:

Normal

PPA

The picture on the left shows one level of the brain in an individual without dementia. The X-like shape contains fluid that feeds the brain. Both sides are similar in appearance. On the right is the same view in the brain of someone with PPA. The darkened spaces on the right side of the figure represent atrophy in the language areas.

 

Related Information

Introduction to PPA

Treatment

For Family and Friends

Brain Donation

Resources

Visit our Volunteer For A Study page. If you would like to participate in a research study and/or clinical trial, please contact us at 312-695-2343 or by e-mail at CNADC-Admin@northwestern.edu