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Primary Progressive Aphasia

Primary progressive aphasia (PPA) is a type of Frontotemporal dementia (FTD), sometimes called frontal lobe dementia, that may begin in people as early as their forties. "Aphasia" is a general term used to refer to deficits in language functions, such as speaking, understanding what others are saying, and naming common objects. In Primary progressive aphasia one or more of these functions can become impaired. Symptoms often begin gradually and progress slowly over a period of years. As the disease progresses, memory and attention may also be impaired and patients may show personality and behavior changes. Many, but not all, people with Primary progressive aphasia eventually develop symptoms of dementia.

What is Aphasia?

Aphasia is a neurological disorder caused by damage to the portions of the brain that are responsible for language. Primary signs of the disorder include difficulty in expressing oneself when speaking, trouble understanding speech, and difficulty with reading and writing. Aphasia is not a disease, but a symptom of brain damage. Most commonly seen in adults who have suffered a stroke, aphasia can also result from a brain tumor, infection, head injury, or dementia that damages the brain. It is estimated that about 1 million people in the United States today suffer from aphasia. The type and severity of language dysfunction depends on the precise location and extent of the damaged brain tissue.

Generally, aphasia can be divided into four broad categories: (1) Expressive aphasia involves difficulty in conveying thoughts through speech or writing. The patient knows what he wants to say, but cannot find the words he needs. (2) Receptive aphasia involves difficulty understanding spoken or written language. The patient hears the voice or sees the print but cannot make sense of the words. (3) Patients with anomic or amnesia aphasia, the least severe form of aphasia, have difficulty in using the correct names for particular objects, people, places, or events. (4) Global aphasia results from severe and extensive damage to the language areas of the brain. Patients lose almost all language function, both comprehension and expression. They cannot speak or understand speech, nor can they read or write.

Is there any treatment?

In some instances, an individual will completely recover from primary progressive aphasia without treatment. In most cases, however, language therapy should begin as soon as possible and be tailored to the individual needs of the patient. Rehabilitation with a speech pathologist involves extensive exercises in which patients with primary progressive aphasia read, write, follow directions, and repeat what they hear. Computer-aided therapy may supplement standard language therapy.

What is the prognosis?

The outcome of aphasia is difficult to predict given the wide range of variability of the condition. Generally, patients with primary progressive aphasia who are younger or have less extensive brain damage fare better. The location of the injury is also important and is another clue to prognosis. In general, patients tend to recover skills in language comprehension more completely than those skills involving expression.

What research is being done?

The NINDS and the National Institute on Deafness and Other Communication Disorders conduct and support a broad range of scientific investigations to increase our understanding of aphasia, find better treatments, and discover improved methods to restore lost function to people who have aphasia, including Primary progressive aphasia.


More on Dementia

What is Dementia

Types of Dementia

Secondary Dementia

Dementia in Children

Causes of Dementia

Other Causes of Dementia

Conditions That Are Not Dementia

Risk Factors for Dementia

Dementia Diagnosis

Treatment for Dementia

Dementia Prevention

Care For Dementia Patient

Research On Dementia


Sources:

National Institutes of Health
9000 Rockville Pike
Bethesda, Maryland 20892
www.nih.gov
Tel: 301-496-4000
----------------
National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
www.ninds.nih.gov
Tel: 800-352-9424
----------------
National Institute of Mental Health
6001 Executive Blvd. Rm. 8184, MSC 9663
Bethesda, MD 20892-9663
www.nimh.nih.gov
Tel: 301-443-4513
Fax: 301-443-4279
----------------
Alzheimer's Disease Education and Referral Center
P.O. Box 8250
Silver Spring, MD 20907-8250
www.alzheimers.nia.nih.gov
Tel: 800-438-4380
Fax: 301-495-3334
----------------
The Alzheimer's Research Trust of U.K.
The Stables
Station Road
Great Shelford
Cambridge
CB22 5LR - UK
www.alzheimers-research.org.uk
Tel: 01223 843899
Fax: 01223 843325





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