Hypertrophic Olivary Degeneration: Neurosurgical Perspective and Literature Review

Background: Hypertrophic olivary degeneration (HOD) occurs because of posterior fossa or brainstem lesions that disrupt the dentato-rubro-olivary tract, well known as the Guillain-Mollaret triangle. Clinical and radiologic hallmarks of this condition are palatal myoclonus and T2 hyperintensity of the inferior olivary complex on magnetic resonance imaging (MRI), respectively. Because symptomatic HOD can complicate the recovery of patients with posterior fossa or brainstem lesions, the purpose of this study is to evaluate clinical and imaging findings of patients with HOD.

Methods: Sixteen patients (8 female and 8 male) with a mean age of 40.7 years, (range, 5-83 years) years were included in this study based on clinical symptoms and MRI findings.

Results: We reviewed the clinical and imaging findings in 16 cases of HOD at our institution. Seven patients (43.7%) had posterior fossa tumors, 6 patients (37.5%) had cavernoma, 2 patients (12.5%) sustained traumatic brain injury, and only 1 patient (6.2%) had cerebellar infarction. Posterior fossa surgery was performed in 13 (81.2%) of these patients. HOD was detected a mean of 7.2 months (range, 0.5-18 months) after surgery or primary neurologic insult. Unilateral HOD was observed in 10 patients (62.5%), while bilateral HOD was observed in only 6 patients (37.5%). Seven patients (43.7%) were asymptomatic for HOD, whereas 5 patients (31.2%) had symptoms attributable to HOD. Two patients died because of primary tumors, although mean follow-up after detection of HOD on MRI was 52.2 months (range, 1-120 months) in the remaining 14 patients. In these cases, no change in clinical symptoms or imaging findings was detected during follow-up.

Conclusions: In this series, posterior fossa tumors and cavernomas were the most common causes of HOD. Although most of the patients with HOD remained asymptomatic, HOD complicated the course of recovery in almost one quarter of the patients included in this study. Neurosurgeons should be aware of HOD, which has characteristic clinical and imaging findings. In addition, HOD can complicate the recovery of patients with disruption to the dentato-rubro-olivary tract.