Hypertrophic Olivary Degeneration


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.

Figure 22.1, Guillain-Mollaret triangle (dentato-rubro-olivary pathway). This functional circuit is responsible for modulating spinal cord motor activity. It is composed of the ipsilateral red nucleus (RN) (red circle) of the midbrain, the ipsilateral inferior olivary nucleus (ION) (green oval) of the medulla, and the contralateral dentate nucleus (DN) (orange circle) of the cerebellum. The RN communicates with the ipsilateral ION via the central tegmental tract (CTT). The ION communicates with the contralateral DN via the inferior cerebellar peduncle. The DN communicates with the contralateral red nucleus via the superior cerebellar peduncle (SCP). The lesions that affect the afferent pathways to the olive result in hypertrophic olivary degeneration (HOD). Therefore lesions resulting in HOD of the right ION would involve the right RN, the right CTT, the left DN, or the left SCP. Lesions affecting efferent pathways to the olive (inferior cerebellar peduncle lesions) are less likely to cause HOD.

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