Treatment

Treatment of Communication Impairments in Primary Progressive Aphasia

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.

At present, there is no cure for the degenerative diseases that cause PPA. Medical treatments are generally in the realm of managing behavioral symptoms such as depression, anxiety, or agitation, which may occur later in the course of the illness.

Drug Trials

There is no current rationale for pharmacological treatment of PPA. The drugs approved for treating amnestic dementia related to Alzheimer's disease (AD) are cholinergic agents that are targeting the cholinergic loss that has been shown in Alzheimer's disease. The likelihood of the pathology in PPA being similar to Alzheimer's disease is very low. Therefore there is no scientific evidence to support the administration of AD drugs to PPA patients. One rationale for treatment of PPA may come from studies of the treatment of aphasia resulting from stroke. Although PPA is a degenerative and therefore progressive condition, similar language areas are affected in both PPA and aphasia due to cerebrovascular disease. There is potential for use of dopamine agonists like bromocriptine, or noradrenergic agents based on previous research with stroke patients.

Approaches to Treatment

There are two basic approaches to treatment for PPA. One approach is to focus treatment directly on the language skills that are impaired, and the other is to provide augmentative/alternative communication strategies or devices. We recommend that both treatment approaches be used with PPA patients. Beginning in early stages of the disease, treatment should be provided to enhance verbal language skills. For example, treatments focused on word-retrieval skills may be helpful.

Treatment focused on the use of augmentative/alternative communication strategies also should be provided, even in the early stages of PPA. These are strategies that either enhance verbal communication or replace it. We suggest that the patient (and family members) be trained in augmentative/alternative communication strategies such as:

• Use of a communication notebook
• Use of gestures
• Use of drawing

Results of the speech and language evaluation will determine which strategy (or strategies) is the best and some practice in using them will be provided during the evaluation. However, follow-up treatment with a Speech-Language Pathologist is important in order to further develop the strategy and provide practice in using it.

Communication Notebooks

Communication notebooks contain words and/or pictures organized by topics, such as the names of family members and friends, places that you like to go, foods, clothing items, etc. The notebook can be altered over time to increase or decrease the information contained in it and to make it maximally useful for communicating the kinds of things that you want to communicate. A Speech-Language Pathologist can help with the selection and organization of items in the notebook and provide practice in using it effectively.

The Use of Gestures

The use of gestures as an augmentative/alternative communication strategy can be used by many individuals with PPA. Gestures can facilitate verbal output in early stages of decline, and can be used as an alternative system in later stages. Most patients seen at Northwestern University have been provided a brief trial of treatment using gestures. Follow-up treatment will involve training a core set of gestures that represent functional items. These gestures are easy to identify by people who have not been trained in their use. Individuals with PPA and their family members can help to develop this core set of gestures. We do not recommend that American Sign Language (ASL) or other sign languages be pursued as alternative communication systems. This is because ASL is a rule-based system, like verbal languages, which may be difficult for individuals with PPA.

The Use of Drawing for Communication

Many individuals with PPA also can use drawing as an augmentative/alternative communication strategy, particularly those who show skill in drawing (although artistic skill is not required). Drawing also is a viable strategy for some patients to use as an alternative system in later stages of progression of the disorder. Several programs for training drawing are available.

Computerized Communication Devices

There are several computerized instruments that are available for individuals with limitations of speech. In some cases, these devices may be helpful. However, they require instruction and a fair amount of comprehension ability in order to use them properly. Therefore, they may be more frustrating than other simpler methods and their continued use may be limited as the illness progresses. However, they should be considered in individual patients where they might be appropriate.

Regardless of which strategy is provided to patients, it is important that use of the strategy in the natural environment be encouraged. Repetition and opportunities to speak are important. Sometimes there may be a tendency to avoid talking because of the difficulty or embarrassment, but every effort should be made to talk whenever possible.

In summary, there are several options for improving communication in patients with PPA. The following guidelines are offered to patients and their family members.

• A complete evaluation of speech, language, and other cognitive skills should be sought in early stages of the disease.
• Frequent follow-up evaluations are needed to determine patterns of language decline.
• Treatment should be provided in early stages, focused on aspects of speech and language that are impaired.
• The focus of treatment should be adjusted as language abilities decline.
• Augmentative communication strategies should be introduced in early stages. Patients provided these strategies in latter stages of language decline may have difficulty learning to use them.
• Involvement of family members or other individuals with whom the patient communicates is important, not only to enhance awareness of successful communication strategies, but also to practice using these strategies with the patient.
• As language declines, patients will rely more on augmentative communication strategies. Some strategies may be more useful than others with certain patients and some patients may use more than one.
• Treatment will not reverse the progression of the aphasia; however, it can greatly enhance communication ability.

General Suggestions for Communicating with Individuals with Primary Progressive Aphasia

1. Communication in a quiet, calm, relaxed environment is usually most successful. For example, avoid conversations when the TV is on; avoid situations in which multiple people are talking at once.
2. It is not necessary to avoid social situations. Instead, such activities should be encouraged. Whenever possible, continue normal activities.
3. Communication is usually most successful when individuals are not fatigued.
4. Communication, not perfection is the goal; therefore, avoid correcting grammar or pronunciation if the message is understood.
5. Be aware that communication abilities are often inconsistent in individuals with PPA. For example, a particular word that is easy to produce one day may be difficult the next. Avoid comments such as, “You said it yesterday, so why can’t you say it today?”
6. Treat persons with PPA as adults; allow independence in activities of daily living unless otherwise advised.

Things to Remember in all Communication Situations

Suggestions for Communicating with Individuals Who Have Difficulty with Expression

1. Do not speak for the individual with PPA unless it is absolutely necessary.
2. Allow adequate time for responding to questions or requests.
3. Try not to interrupt or supply words unless help is requested.
4. When you don’t understand what the individual is trying to say, ask simple questions that require yes/no answers or gestural (pointing) responses for clarification.
5. Never pretend that you understand, when you don’t. Admit your problem, saying, “I’m sorry, but I’m not sure I understand. Is what you are trying to say very important, or should we try again later?”
6. Some responses will be incomplete or may be unrelated or opposite to what the person actually wants to say. For incomplete responses such as “glass…..water,” you can complete the utterance by saying, “You want a glass of water.” If a wrong word is produced, it is not necessary to correct the word, but you can repeat what you think the person meant to say, such as when the individual says “knife,” but meant to say “fork,” respond to him/her by saying “here’s the knife.”
7. Avoid using open-ended questions when seeking clarification. For example, ask “Is it raining outside?” rather than “What is the weather like today?”
8. Encourage all modes of communication (speech, gesture, drawing and use of a communication notebook).
9. Suggestions for Communicating with Individuals

Suggestions for Communicating with Individuals Who Have Difficulty Understanding Language

1. Do not assume that a person with PPA understands everything that you are saying, even if he/she looks alert and nods appropriately while you are talking.
2. Conversely, do not presume that the individual with PPA does not understand what you are saying. Don’t talk about him/her in his/her presence without inclusion in the conversation.
3. Establish the individual’s attention by saying the person’s name or tapping his/her shoulder prior to the initiation of conversation.
4. Repeat or rephrase what you have said, as necessary.
5. Supplement directions with use of facial expression and/or use of gestures.
6. Keep your own communication simple, but adult. Use short, concrete statements rather than lengthy, complex questions and directions in daily conversation.
7. Avoid giving long, involved directions.
8. Avoid a rapid speech rate.
9. Do not shout or talk loudly.

Related Information

Introduction to PPA

Diagnosis

For Family and Friends

Brain Donation

Resources