Progressive supranuclear palsy with palatal myoclonus.

We present a case of progressive supranuclear palsy (PSP) with palatal myoclonus occurred in a 64-year-old man. The nucleus olivaris of the medulla oblongata showed high signal intensity on T2-weighted MR images, indicating brainstem tegmental atrophy, which were confirmed as hypertrophy of the nucleus inferior olivaris at autopsy. The characteristic neuropathological findings were marked grumose degeneration of the dentate nucleus, degeneration and gliosis of the superior cerebellar peduncle, and hypertrophy of the bilateral olivary nuclei. As far as we know, although a few cases of PSP with olivary hypertrophy have been described, only one case has presented with palatal myoclonus.