Canavan disease

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Canavan disease

Canavan disease (also known as spongy degeneration of the brain) is an inherited diseases that causes the progressive loss of the regions of the brain through which nerve impulses travel to other parts of the brain or to the spinal cord (white matter). Loss of white matter gives the brain a porous, degenerative appearance.

Babies with this disease appear normal after birth. Later they become floppy (hypotonic), are not able to control head movements, and by 5 to 8 months are developmentally delayed. Children with this disease usually have an enlarged head, mental retardation, seizures, and feeding difficulties.

The severity of Canavan disease varies. Although some children live into their early twenties, most die before age 4.

There is no treatment for the underlying cause of Canavan disease. Genetic testing is recommended to identify carriers of the disease and help guide decisions about having children.

Credits

Author	Jeannette Curtis
Author	Caroline Rea, RN, BS, MS
Editor	Kathleen M. Ariss, MS
Editor	Susan Van Houten, RN, BSN, MBA
Associate Editor	Tracy Landauer
Associate Editor	Pat Truman
Primary Medical Reviewer	Patrice Burgess, MD - Family Medicine
Primary Medical Reviewer	Adam Husney, MD - Family Medicine
Primary Medical Reviewer	Kathleen Romito, MD - Family Medicine
Last Updated	May 25, 2007

Last updated:	May 25, 2007
Author:	Caroline Rea, RN, BS, MS
Reviewed By:	Kathleen Romito, MD - Family Medicine
Editors:	Susan Van Houten, RN, BSN, MBA, Pat Truman

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