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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July 9, 2012
Hypertrophic Olivary Degeneration (HOD)
- HOD refers to transsynaptic degeneration 2° to lesions such as infarcts, demyelination, and hemorrhage that damage neuronal connections (Guillain-Mollaret triangle) between red nucleus, inferior olivary nucleus, and contralateral dentate nucleus of the cerebellum.
- Clinical findings include palatal myoclonus, dentatorubral tremor, and ocular myoclonus which rarely improve.
- Key Diagnostic Features: Inferior olivary hypertrophy results within 4 to 6 months of insult to the ipsilateral red nucleus or contralateral dentate nucleus. This olivary hypertrophy resolves approximately 18 months following onset, though hyperintense signal within the inferior olivary nucleus can persist.
- DDx: Infarct, tumor, demyelination
- No Rx. Symptomatic Rx.
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