A Treatable Neurometabolic Disorder: Glutaric Aciduria Type 1

Figure 1

Showing patient with macrocephaly, typical facies, and MRI of his brain reveals frontotemporal atrophy, dilated sylvian fissures with open opercula (arrow), diffuse hyperintense lesions in bilateral basal ganglia, and both frontal white matter and bilateral periventricular area. Widening of the sylvian fissure gives the characteristic “bat-wing” appearance.