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What is Primary Progressive Aphasia (PPA)?

PPA (primary progressive aphasia) is a dementia that makes the person progressively lose the ability to communicate and comprehend language.

• PPA  is a clinical syndrome, one of several forms of brain disease lost in the medical shadow of their much better known relative, Alzheimer’s disease. While not as common as Alzheimer’s, PPA is often misdiagnosed and many patients lose valuable time trying inappropriate and ineffective treatments.

• PPA begins with the slow decline of the ability to use language in speaking, understanding, reading and writing.  The disease begins very gradually and initially is experienced as difficulty thinking of common words while speaking or writing.  PPA progressively worsens to the point where verbal communication by any means is very difficult. The ability to understand what others are saying or what is being read also declines.

• Unlike Alzheimer’s, PPA does not affect memory in the initial stages of the illness. While symptoms of Alzheimer’s are readily recognized by friends and relatives but not those affected, people with PPA are painfully aware of their struggle to communicate, often long before it is apparent to others.  They are very aware of their language deterioration but are unable to express themselves and often become depressed.

What causes PPA?

• PPA arises when parts of the brain that control language malfunction. The underlying diseases are called “degenerative” because they involve nerve cell death but cannot be attributed to clear-cut causes such as head trauma, stroke or cancer.

Why have I never heard of PPA?

• PPA is a relatively rare form of dementia that affects many fewer people than the most common type of dementia, Alzheimer’s Disease.
• Because PPA affects individuals at a younger age than most with dementia, doctors may not recognize the features and can misdiagnose the individual with another condition. Increasing awareness about PPA in the medical community will facilitate more efficient and accurate diagnoses.

How does PPA progress?

• The progression is variable and unfolds over many years. Word finding and word comprehension become more difficult over time. Sometimes additional problems can arise in personality (irritability, apathy or inappropriate behaviors), problem solving, memory or dexterity.

How is PPA different from Alzheimer’s Disease (AD)?

• In scientific language, the term AD denotes a specific pattern of abnormalities in the brain. These can only be detected by microscopic examination after death. In the vast majority of cases, AD starts by affecting the parts of the brain that control short-term memory so that the symptoms consist of forgetfulness instead of word‐finding difficulty.
• In about 30% of people who receive a diagnosis of PPA, the examination of the brain after death shows changes of AD. In such cases, PPA can be considered an atypical (unusual) form of AD.
• In the majority of people with PPA, the brain shows changes of Frontotemporal Lobar Degeneration (FTLD), also known as Frontotemporal Degeneration (FTD). FTLD and AD are two completely different diseases of nerve cells even though they both may cause PPA.
• Some types of PPA are more likely to be caused by AD, others by FTLD. Researchers are working to identify reasons for this.

How is PPA different than aphasia caused by a stroke or brain injury?

• Aphasia due to stroke or brain injury often develops suddenly. In contrast, people with PPA develop language difficulties slowly over time.
• People with aphasia from stroke or brain injury can often improve with speech therapy, while people with PPA continue to worsen.

Is there treatment for PPA?

• There is no medical treatment that has been proven to work for PPA.
• Because of the 30% probability of AD, some physicians will prescribe AD drugs such as Exelon, Razadyne, Aricept or Namenda. None have been shown to improve PPA.
• Speech therapy may offer benefits in the early stages by teaching more effective communication strategies and ways to compensate for language difficulties.
• Quality of life enrichment and support programs offer individuals and families ways of coping with a diagnosis of PPA.

Is PPA hereditary?

• PPA can be caused by hereditary forms of FTLD. However, it is extremely rare for more than one member of the same family to have PPA.

What are some features of PPA?

• Slowed or halting speech
• Word-finding hesitations
• Sentences with abnormal word order
• Substitution of words (e.g., “table” Instead of “chair”) or words that are incomprehensible
• Difficulty understanding conversation topics despite normal hearing
• Inability to recall names, even though the person is recognized
• New impairments in spelling

Does the diagnosis of PPA mean the end of an active life?

• People with PPA usually have to make major adjustments at work since almost all professions are heavily dependent on verbal communication.
• Many people with PPA remain independent for many years, participate fully in social and civic activities, travel widely and take up novel hobbies ranging from gardening to square dancing, painting, carpentry, etc.

Is there a cure?

• At present, there is no effective drug or medical treatment that has been proven to cure PPA.

Where can I go for more information?

• Northwestern University Cognitive Neurology and Alzheimer’s Disease Center: www.brain.northwestern.edu
• IMPPACT, the International PPA Connection: www.ppaconnection.org
• The Association for Frontotemporal Dementias: www.theafd.org
• The National Aphasia Association: www.aphasia.org