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About Canavan Disease

children who have Canavan diseaseCanavan disease is a progressive, fatal neurological disorder that begins in infancy. It is caused by an inherited genetic abnormality: the lack of an essential enzyme causes deterioration of the white matter (myelin) in the brain, thereby preventing the proper transmission of nerve signals.

Symptoms of Canavan disease vary, but generally include rapidly increasing head circumference, lack of head control, reduced visual responsiveness and abnormal muscle tone such as stiffness or floppiness. Children with Canavan disease cannot crawl, walk, sit or talk. Over time they may suffer seizures, become paralyzed, developmentally delayed or blind and have trouble swallowing. Deafness may also result. 

Symptoms usually become apparent when the infant is three to nine months old. Many children do not live past age 10. At the present time there is no cure for Canavan disease. Treatment involves managing the symptoms.

The rate of prevalance of Canavan Disease is highest in persons of Ashkenazi (German and Eastern European) Jewish ancestry. It is estimated that 1 in 40 Ashkenazi Jews is a carrier of the Canavan gene. It is also found in other ethnic groups at a lower prevalence. In fact, most of the children born with Canavan disease today have no known Jewish heritage.

Although it’s still in the trial phase, there is currently promising research into treatments such as gene replacement therapy.

Medline Plus has further scientific information on Canavan disease with extensive links to other scientific and medical sites.