Canavan disease (Spongy degeneration of the brain, Aspartoacylase deficiency)

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Canavan disease

Alternate Names : Spongy degeneration of the brain, Aspartoacylase deficiency

Definition

Canavan disease is an inherited condition that affects the breakdown and use (metabolism) of aspartic acid.

Overview, Causes, & Risk Factors

Canavan disease is passed down (inherited) through families. It is more common among Ashkenazi Jews than in the general population.

The lack of the enzyme, aspartoacylase, leads to a buildup of material called N-acetylaspartic acid in the brain. This causes the white matter of the brain to break down (deteriorate).

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Canavan disease : Definition
Canavan disease : Overview, Causes, & Risk Factors
Canavan disease : Symptoms & Signs
Canavan disease : Diagnosis & Tests
Canavan disease : Treatment
Canavan disease : Prognosis (Expectations)
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Review Date : 10/14/2009
Reviewed By : Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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