Wolfram Syndrome Presenting Marked Brain MR Imaging Abnormalities with Few Neurologic Abnormalities

Axial T2-weighted MR images show atrophy of the brain stem and middle cerebellar peduncles and areas of elevated signal intensity in the pons (A and B). Cerebellar atrophy is also recognized. Signal-intensity abnormalities in optic radiation on both sides are also seen (C, arrowheads). A sagittal T1-weighted MR image shows intracranial optic nerve atrophy (D, arrows) and absence of T1-hyperinensity normally recognized in the posterior pituitary lobe. A coronal T1-weighted image shows atrophy of both intraorbital optic nerves (E).