Primary progressive aphasia

Diagnosis

To diagnose primary progressive aphasia, a neurologist or speech and language pathologist will likely review your symptoms and order tests.

Communication troubles that get worse without significant changes in thinking and behavior for 2 to 3 years are a hallmark of primary progressive aphasia.

Tests and procedures used to diagnose primary progressive aphasia may include:

Neurological exam

Healthcare professionals might conduct a neurological exam, a speech-language evaluation and a neuropsychological evaluation. Tests measure speech, language comprehension and skills. They also measure your recognition and naming of objects, recall, and other factors.

Blood and body fluid tests

Your healthcare professional might order blood tests or a lumbar puncture, also known as a spinal tap, to check for infections or look for other medical conditions. Genetic tests can determine whether you have gene changes linked to primary progressive aphasia or other neurological conditions.

Brain scans

A brain MRI can help diagnose primary progressive aphasia. The test can detect shrinking of specific areas of the brain. MRI scans also can detect strokes, tumors and other conditions that affect brain function.

You also might get a fluorodeoxyglucose positron emission tomography scan, also known as a FDG PET scan, which shows brain function. This test can show changes in how the brain uses glucose in the areas that are related to language. You also may have other PET scans to look for the presence of certain proteins linked to Alzheimer's disease.

Treatment

Primary progressive aphasia can't be cured, and there are no medicines to treat it. However, some therapies might help improve or maintain your ability to communicate and manage your condition.

Speech and language therapy

Working with a speech-language pathologist, focusing primarily on ways to make up for lost language skills, can be helpful. Although speech and language therapy can't stop the progression of the condition, it can help you manage your condition. The therapy also may slow the progression of some symptoms. Your therapist may recommend that your caregivers also attend the appointments.

Physical and occupational therapy

If your symptoms affect movement and balance, working with a physical therapist and an occupational therapist can help manage the symptoms.

Coping and support

Losing the ability to communicate can be stressful and frustrating. This is true for both the person with primary progressive aphasia and for friends and family. If you're a caregiver of someone with primary progressive aphasia, taking these steps can help everyone cope:

• Learn all you can about the condition.
• Have the person with the condition carry an identification card and other materials that can help explain the condition to others.
• Give the person time to talk.
• Speak slowly in simple, adult sentences and listen carefully.
• Talk with the person with the condition about how you can help.
• Take care of your personal needs. Get enough rest and make time for social activities.

Family members eventually may need to make long-term care choices for the person with primary progressive aphasia. They also may need to plan the person's finances and help make legal decisions to prepare for more-serious stages of the condition. Start this process early so the person with primary progressive aphasia can be involved.

Support groups may be offered for caregivers and people with primary progressive aphasia or related conditions. Ask your social worker or other members of your healthcare team about community resources or support groups. Other sources of information include the National Aphasia Association and the Association for Frontotemporal Degeneration.

Preparing for your appointment

If you're experiencing symptoms, you might start by seeing your primary healthcare professional. This person may refer you to a doctor trained in brain and nervous system conditions, known as a neurologist. You also may be referred to a speech-language pathologist.

What you can do

Here's some information to help you get ready for your appointment.

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

For primary progressive aphasia, some questions to ask might include:

• What's likely causing my symptoms?
• What tests do I need?
• Is my condition likely temporary or ongoing?
• Should I see a specialist?
• Should I consider genetic testing?
• Are there brochures or other printed material I can have? What websites do you recommend?
• What's the best course of action?
• Are there other choices to the primary approach you're suggesting?
• I have other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• What is going to happen to me over time?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare professional is likely to ask you several questions, such as:

• When did your symptoms begin?
• What symptoms came first?
• Have you had any new symptoms?
• Have your symptoms come and gone or stayed steady?
• How bad are your symptoms?
• Have your symptoms gotten worse?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?
• Have other family members had similar conditions?