A 58-year-old female comes to clinic six months after an ischemic stroke in the paramedian pontine reticular formation (PPRF). She initially presented with acute vertigo, ataxia, and mild hemiplegia, which have significantly improved with therapy. Her only complaint now is that a few weeks ago she developed an incessant clicking in her ears. Her exam is noteworthy for continuous rapid spasms of the soft palate and increased nystagmus.
Repeat MRI demonstrates enlargement of the inferior olive with a new non-enhancing T2 hyperintensity. This is consistent with hypertrophic olivary degeneration, a form of multisynaptic transneuronal degeneration caused by disruption of the dento-rubro-olivary pathway (DROP) in the Guillain-Mollaret triangle. While strokes are a relatively common etiology of this rare condition, any lesion affecting these pathways can be implicated.
Cytoplasmic vacuolar degeneration and astrocytosis are seen in histopathology. The gross enlargement recedes into atrophy over years, although T2 hyperintensity is ongoing.
Palatal tremor and pendular nystagmus comprise the classic clinical features of a PPRF lesion. Dentorubral (Holm’s) tremor and ocular myoclonus may also be present. Mild symptoms are usually self-limited. For those with very bothersome palatal myoclonus, medical management is challenging. Clonazepam, anti-seizure medications, and botulinum toxin injection have been tried with some successes.
- Figure 1 Medical Case: Hypertrophic Olivary Degeneration
- MRI Findings in Nonlesional Hypertrophic Olivary Degeneration
- Oculopalatal Tremor
- Wang H, Wang Y, Wang R, Li Y, Wang P, Li J, Du J. Hypertrophic olivary degeneration: A comprehensive review focusing on etiology. Brain Res. 2019 Sep 1;1718:53-63. doi: 10.1016/j.brainres.2019.04.024. Epub 2019 Apr 23. PMID: 31026459.
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