[An autopsy case of progressive supranuclear palsy with olivary hypertrophy]

Abstract

Clinical and pathologic findings of an autopsy case of progressive supranuclear palsy (PSP) with a 7 year clinical course are described. The patient exhibited clinical findings of typical PSP, cerebellar signs and rhythmical myoclonus that was about 2 Hz and synchronous in the eyes, palate, and pharynx, which is so called palatal myoclonus. Pathological findings compatible with those in PSP i.e. loss of nerve cells, neurofibrillary tangles (NFT), and gliosis were found in the globus pallidum, thalamus, subthalamic nucleus, substantia nigra, locus coeruleus, nucleus of Raphe, reticular formation, dentate nucleus, and inferior olives. Nerve cells in the nucleus basalis were preserved. Distinctive findings included marked degeneration of the dentate nucleus, prominent hypertrophy of the inferior olives, and atrophy and subcortical gliosis of the frontal lobe. Hypertrophy of the inferior olives and palatal myoclonus represent an unusual PSP. It is presumed hypoxic injury unmasked the palatal myoclonus in this setting of dentate nucleus and inferior olivary complex degeneration.