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Medical Definition of Canavan-van Bogaert-Bertrand disease
1. Autosomal recessive degenerative disease of infancy; mostly in Jewish infants; onset typically within first 3-4 months of birth, consisting of blindness, psychomotor regression, enlarged head, optic atrophy, hypotonia, spasticity, increased N-acetylaspartic acid urinary excretion. MRI shows enlarged brain, decreased attenuation of cerebral and cerebellar white matter, and normal ventricles. Pathologically, there is increased brain volume and weight, and spongy degeneration in the subcortical white matter. See: leukodystrophy. Synonym: Canavan's sclerosis, Canavan-van Bogaert-Bertrand disease, spongy degeneration of infancy. (05 Mar 2000)
Lexicographical Neighbors of Canavan-van Bogaert-Bertrand Disease
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