Hypertrophic olivary degeneration (HOD) is secondary degeneration of the inferior olivary nucleus (ION) due to a primary lesion in the dento-rubro-olivary pathway. This pathway is known as the Guillain and Mollert triangle, containing the dentate nucleus and the contralateral red and inferior olivary nuclei (figure e-1 on the Neurology® Web site at www.neurology.org). The commonest presenting symptom is palatal myoclonus occurring 8-12 months after the primary insult. MRI of the ION initially has normal results (figure 1). Three phases of HOD exist on MRI: hyperintense signal change without hypertrophy, hyperintense signal change with hypertrophy (figure 2), and regression of hypertrophy with persistent hyperintense signal.(1.)

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http://dx.doi.org/10.1212/WNL.0b013e3182918c91DOI Listing

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  • * Cavernomas and orbital cavernous hemangiomas are specific types of vascular malformations linked to HOD, but their combination is extremely rare, with only one previous case reported.
  • * A notable case involved a 26-year-old male with eye bulging and myoclonus, where MRI revealed HOD from cavernous malformations, highlighting the need to consider vascular issues as potential causes of HOD.
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  • The inferior olive (IO) is a key brainstem region affected by spinocerebellar ataxias (SCAs), impacting motor learning, but its degeneration mechanisms remain unclear.
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Background: Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration, caused by injury to the dentato-rubro-olivary pathway (DROP). Radiologically, this manifests as T2 hyperintensity, with or without enlargement of the inferior olivary nucleus. The purpose of the study was to evaluate the incidence, associated imaging characteristics, potential etiologies, latency period, and temporal progression of HOD in patients undergoing surgical resection of posterior fossa tumors (PFTs).

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We present a case of 12-year-old boy evaluated in view of refractory ascites in whom 18 F-FDG PET/CT incidentally revealed hypermetabolism in the medulla that was proven to be hypertrophic olivary degeneration on MRI.

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