Primary progressive aphasia is a rare neurological system syndrome that impairs the language capabilities. It is a type of frontotemporal degeneration, a cluster of related disorders that originate in the frontal or temporal lobes of the brain. People who suffer from this syndrome may have trouble expressing their thoughts and comprehending or finding words. However, they may continue caring for themselves and participating in daily life activities for several years after the disorder's onset, as the condition progresses slowly.
People with PPA can experience many different types of language symptoms that begin gradually and tend to worsen over time. In many instances, the first indication that something is wrong may initially be attributed to stress or anxiety. PPA symptoms may vary by individual, depending on which portion of the brain's language center is involved. They initially experience one or more of the following symptoms:
- Slowed or halting speech;
- Decreased use of language;
- Word-finding hesitations;
- Sentences with abnormal word order in speech or e-mails;
- Using mispronounced or incomprehensible words;
- Difficulty understanding or following conversation despite normal hearing;
- Sudden lapse in understanding simple words;
- Forgetting the names of familiar objects;
- Inability to think of names of people, even though the person is recognized;
- Problems with writing, spelling and reading;
- Problems in arithmetic and calculations.
Primary progressive aphasia is caused by an atrophy of the frontal and temporal lobes in the brain, primarily on the left side of the brain, i.e the parts of the brain that control speech and language. Primary progressive aphasia affects the language center in the brain. Scar tissue and abnormal proteins also may be present, and brain activity is often reduced.
There are no known environmental risk factors for the progressive aphasias. However, one observational, retrospective study suggested that vasectomy could be a risk factor for PPA in men. These results have yet to be replicated or demonstrated by prospective studies. PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration are at slightly greater risk of developing PPA or another form of the condition.
Risk factors for primary progressive aphasia include having learning disabilities and having certain gene mutations.
As the disease progresses, other mental skills, such as memory, may become impaired. Some people may develop other neurological conditions over time. If these complications occur, the affected person eventually will need help with day-to-day care. Some people with PPA may become mute and may eventually lose the ability to understand written and spoken language.
People with primary progressive aphasia may also develop behavioral or social problems, such as being anxious or irritable. Other problems may include blunted emotions, poor judgment or inappropriate social behavior.