Diagnosis & Treatment

Diagnosis of Primary Progressive Aphasia (PPA)

A thorough evaluation of PPA includes the following:

History: First, a careful history is taken to establish that a condition of dementia 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.

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 (e.g., blood tests, spinal tap, brain imaging studies) to further investigate the cause of the symptoms.

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.

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.

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.

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. However, especially in early stages, they may not show anything. 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.

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.

There are many thousands of people with PPA. Nonetheless, compared to the millions of patients with Alzheimer-type amnestic dementias, PPA is rare.  Furthermore, it can start in a person’s 40s and 50s, an age range that physicians do not usually associate with neurodegenerative diseases. Therefore, some people with PPA often see multiple doctors and receive many different diagnoses before receiving the diagnosis of PPA. 

Treatment

There are no pills yet for PPA.  Because of the 30%-40% probability of Alzheimer’s disease (AD), some physicians will prescribe AD drugs such as Exelon (rivastigmine), Razadyne (galantamine), Aricept (donepezil) or Namenda (memantine). None have been shown to improve PPA. Medicine is also sometimes prescribed to manage behavioral symptoms such as depression, anxiety, or agitation, which may occur later in the course of the illness.

There are, however, life-enriching interventions and speech therapies that can help improve a diagnosed person’s quality of life. The primary goal of treatment for language impairments in individuals with PPA is to improve the ability to communicate. Because the type of language problems experienced by patients with PPA may vary, the focus of treatment for improving communication ability will also vary. A complete speech and language evaluation provides the information needed to determine the type of treatment that is most appropriate.

There are two basic approaches to speech therapy for PPA. One approach is to focus treatment directly on the language skills that are impaired (for example, skills to enhance word-retrieval abilities), and the other is to provide augmentative/alternative communication strategies or devices. We recommend that both treatment approaches be used in people with PPA.  Regardless of which strategies are provided to people with PPA, it is important that the family is involved in treatment and that the use of the strategy in the natural environment is encouraged.

Suggestions for Effective Communication for Supportive Communication Partners

General

Listening

Speaking

Writing

Gestures

Body Language

Communication is a two-way street. Both diagnosed persons and their communication partners will need to adapt to new methods of communication. Working with a speech-language pathologist can help make this transition. Remember, the goal is communication, not perfection.