Fig 2. T2-weighted axial images in patient 1 at 24 years of age (disease duration, 16 years). A, There is atrophy of the mid pons, middle cerebellar peduncles, and cerebellar hemisphere and severe atrophy of the superior cerebellar peduncles. B, Marked abnormal hyperintensities are found bilaterally around the periaqueductal gray matter (arrows), and the midbrain tegmentum shows atrophy.