A Treatable Neurometabolic Disorder: Glutaric Aciduria Type 1

Figure 2

The child with a large head and his MRI brain revealing wide CSF spaces with temporal lobe hypoplasia, bilateral front parietal subdural effusions and dilatation of the sylvian fissures with open opercula (arrow), and high signal intensity seen in bilateral caudate nuclei, putamen, and deep subcortical white matter. Widening of the sylvian fissure gives the characteristic “bat-wing” appearance.