Canavan disease--an expanded role for dentists.

Abstract

Canavan disease (CD) is a progressive neurodegenerative disease that occurs predominantly but not exclusively in Ashkenazi Jews. It is characterized by a normal birth, rapidly followed by signs of lack of interest, hypotonia, and megalencephaly, resulting in early death. Genetic studies suggest that among Ashkenazi Jews the carrier frequency is between 1/35 and 1/59. Dentists aware of CD can assist in the treatment of infants and children afflicted with this disorder. Early dental treatment may support parents and siblings in caring for the child with CD and help create good family dynamics.