Primary Progressive Aphasia

Casa Batlló, a building designed by Gaudí in Barcelona. (Dr. Noorali Bharwani)
Casa Batlló, a building designed by Gaudí in Barcelona. (Dr. Noorali Bharwani)

Primary progressive aphasia is a rare nervous system syndrome. It is an acquired condition that affects a person’s ability to communicate.

An aphasic person cannot express himself or herself when speaking, has trouble understanding speech, and has difficulty with reading and writing or finding words.

Brain damage causes aphasia. This quite often happens after a stroke or head injury. It can happen if a person has a brain tumour or Alzheimer’s disease. It is important to remember primary progressive aphasia is not Alzheimer’s disease. In primary progressive aphasia the problem is a disorder of language with preservation of other mental functions of daily living for at least two years. Symptoms may get worse after that.

The effects of aphasia differ from person to person and can sometimes be eased by speech therapy. Most people affected by this condition can maintain ability to take care of themselves and pursue hobbies. In some instances a person can remain employed.

Primary progressive aphasia may present in a number of different ways but it commonly appears initially as a disorder of speech, progressing to a near total inability to speak in its most severe stage, while comprehension remains relatively preserved.

Symptoms begin gradually, often before age 65, and worsen over time. People with primary progressive aphasia have a difficult road ahead. They are fighting against a condition in which they will continue to lose their ability to speak, read, write, and/or understand what they hear. The illness progresses slowly.

Medically speaking, primary progressive aphasia is caused by a shrinking of the frontal, temporal or parietal lobes in the brain, primarily on the left side. The condition affects the language centers in the brain.

Who is at a higher risk of being affected by primary progressive aphasia? A person having learning disabilities and a person who has certain gene mutations – meaning that it may run in the family.

An individual who has aphasia should carry an identification card and obtain materials available from the National Aphasia Association (www.aphasia.org). This helps in communicating about the person’s condition to others.

Unfortunately, people with primary progressive aphasia eventually lose the ability to speak and write, and to understand written and spoken language. As the disease progresses, other mental skills, such as memory, can become impaired. Some people develop other neurological conditions. With these complications, the affected person eventually will need help with day-to-day care.

People with primary progressive aphasia can also develop behavioral or social problems as the disease progresses, such as anxiety or irritability. Other problems might include blunted emotions, poor judgment or inappropriate social behavior.

The diagnosis of the condition is based on history of worsening communication skills, changes in thinking and behaviour over one to two years. Besides physical examination a doctor will order several test including blood tests, speech and language tests, genetic tests, MRI, etc.

Unfortunately, primary progressive aphasia cannot be cured, and there are no medications to treat it. The good news is, some therapies, like speech and language therapy, may help improve or maintain the ability to communicate and manage the condition.

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