Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 3, 2010
Hypertrophic Olivary Degeneration
- Hypertrophic olivary degeneration is a unique type of transsynaptic degeneration resulting in hypertrophy of degenerated inferior olivary neurons rather than atrophy.
- It results from damage to neuronal pathways in the Guillain-Mollaret triangle between the dentate nucleus of the cerebellum, red nucleus, and inferior olivary nucleus.
- Palatal myoclonus is the classic clinical manifestation due to disruption of the central tegmental tract between the red nucleus and inferior olivary nucleus.
- Most commonly occurs following focal lesions to the brainstem, such as hemorrhage, which in this case was related to a cavernous hemangioma.
- Typically appears 4-6 months after brainstem insult and resolves by 3-4 years, though the T2 hyperintensity may persist longer.