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Progressive supranuclear palsy with palatal myoclonus
N Suyama1, S Kobayashi, H Isino
1Department of Internal Medicine III, Shimane Medical University, Izumo, Japan.
Acta Neuropathologica
|September 18, 1997
Summary
This case study details progressive supranuclear palsy (PSP) with palatal myoclonus. Neuropathological findings revealed olivary nuclei hypertrophy, a rare association with PSP and myoclonus.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease.
- Palatal myoclonus is an involuntary movement disorder affecting the soft palate.
Observation:
- A 64-year-old man with progressive supranuclear palsy presented with palatal myoclonus.
- MRI revealed high signal intensity in the medulla oblongata's nucleus olivaris, suggesting brainstem tegmental atrophy.
Findings:
- Autopsy confirmed hypertrophy of the nucleus inferior olivaris.
- Key neuropathological findings included dentate nucleus degeneration, superior cerebellar peduncle damage, and bilateral olivary nuclei hypertrophy.
Implications:
- This case highlights a rare presentation of PSP with palatal myoclonus.
- The findings contribute to understanding the neuropathological spectrum of PSP and associated movement disorders.