Introduction
Hypertrophic olivary degeneration (HOD) is the result of an insult to the dentato-rubro-olivary pathway ( Fig. 22.1 ), otherwise known as the Guillain-Mollaret triangle (GMT). After an insult (infarct, hemorrhage, trauma, tumor, surgery) that disrupts the GMT, hypertrophic degeneration of the affected inferior olivary nucleus (ION) develops. The counterintuitive degenerative hypertrophy (rather than atrophy) of the ION can lead to confusion. Of note, lesions involving this functional circuit may produce palatal myoclonus, which is one of the few involuntary movements that do not extinguish during sleep. Other classic clinical findings associated with HOD are dentato-rubral tremor and ocular myoclonus.
Temporal Evolution: Overview
The hallmarks of HOD are T2 hyperintensity and enlargement of the ION. The classic teaching for the imaging diagnosis of HOD depends on the identification of a nonenhancing mildly expansile T2 hyperintense olivary lesion in association with a lesion/insult to the contralateral dentate nucleus, contralateral superior cerebellar peduncle, ipsilateral red nucleus, or ipsilateral pontine tegmentum. However, an understanding of the temporal evolution of HOD is also necessary for the accurate interpretation of changing imaging patterns ( Fig. 22.2 ). Three distinct phases are evident on magnetic resonance imaging (MRI): (1) ION T2 hyperintensity without hypertrophy within 6 months. (2) ION T2 hyperintensity with hypertrophy usually resolving by 3 to 4 years. (3) ION atrophy after several years with T2 hyperintensity persisting indefinitely ( Fig. 22.3 ).