Canavan disease

noun

Can·​a·​van disease ˈka-nə-ˌvan- 

variants or less commonly Canavan's disease

ˈka-nə-ˌvanz- 

: a rare, usually fatal, demyelinating disease of infancy that is characterized by spongy degeneration of the brain caused by an enzyme deficiency and that typically affects individuals of eastern European Jewish ancestry

… was diagnosed before he turned 5 months old with Canavan disease—a rare neurological disorder in which genes don't produce the protective myelin in the brain, causing the brain's white matter to degenerate into spongy tissue riddled with microscopic fluid-filled spaces.

—Burt Constable

Note: Canavan disease is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

Examples of Canavan disease in a Sentence

Recent Examples on the Web

Ekstein went after the other recessive diseases affecting his community, such as Niemann-Pick, familial dysautonomia and Canavan disease. —Jeff Wheelwright, Discover Magazine, 20 May 2012

Word History

Etymology

after Myrtelle May Canavan †1953 U.S. pathologist

First Known Use

1965, in the meaning defined above

Medical Definition

Canavan disease

noun

Can·​a·​van disease ˈkan-ə-ˌvan- 

variants also Canavan's disease

-ˌvanz- 

: a rare usually fatal demyelinating disease of infancy that is characterized by spongy degeneration of the brain caused by an enzyme deficiency inherited as an autosomal recessive trait and that typically affects individuals of eastern European Jewish ancestry