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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April 15, 2013
Hypertrophic Olivary Degeneration (HOD)
- HOD is a unique type of transneuronal degeneration caused by a variety of lesions affecting the dentato-rubro-olivary pathway (triangle of Guillain and Mollaret).
- Clinical Presentation: Palatal tremor/myoclonus and/or upper extremity tremor
- Key Diagnostic Features: 3 distinct MR stages on T2W images:
- hyperintense signal without hypertrophy of inferior olivary nucleus (ION) within first 6 months of ictus
- increased signal and hypertrophy of ION in-between 6 months to 3-4 years
- and T2 signal prolongation of ION without hypertrophy (-/+ atrophy) after 3-4 years. The T2 signal prolongation may persist indefinitely.
- DDx: Infarction, demyelinating disorders, tumor, infectious/inflammatory processes, Wallerian degeneration