MRI abnormalities in Behr syndrome.

The etiology of Behr syndrome remains uncertain. A 6-year-old girl with a progressive syndrome of optic atrophy, ataxia, myoclonic epilepsy, urinary incontinence, and pyramidal tract degeneration suggestive of Behr syndrome is described. Diffuse, symmetric white matter abnormalities were demonstrated by magnetic resonance imaging suggesting that Behr syndrome may represent a disorder of white matter associated with an unknown biochemical abnormality.