CT and MR imaging of Canavan disease.

Canavan (Van Bogaert-Bertrand) disease is a rare dysmyelinating disorder of the CNS that presents in the first year of life with spasticity, blindness, and megalencephaly [1 , 2] . The origin of this disease has not been definitely established, but it may relate to a deficiency in aspartoacyclase, an enzyme in the myelin synthesis pathway [3] . Without brain biopsy, it may be quite difficult to distinguish clinically from a similar disorder, Alexander disease, although a serum marker may be forthcoming [3-5] . CT in Canavan disease usually reveals diffuse, symmetrically decreased attenuation within the subcortical white matter without lobar predominance or areas of abnormal contrast enhancement [1 , 6, 7] . Late cerebral atrophy has also been described [8]. We report here the MR findings (with CT correlation) in two siblings with Canavan disease and offer a review of the literature.