J Neurol Surg A Cent Eur Neurosurg 2016; 77(01): 059-062
DOI: 10.1055/s-0035-1566114
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Hypertrophic Olivary Degeneration: A Neurosurgical Point of View

Carlos Henrique Carvalho
1   Department of Neurosurgery, Schwarzwald-Baar Klinik, Academic University Hospital of Freiburg, Villingen, Germany
Hubert Kimmig
2   Department of Neurology, Schwarzwald-Baar Klinik, Academic University Hospital of Freiburg, Villingen, Germany
William Omar Contreras Lopez
3   Department of Functional Neurosurgery, University of São Paulo, São Paulo, Brazil
Manfred lange
4   Department of Neurosurgery, Schwarzwald-Baar Klinik, Academic University Hospital of Freiburg, Villingen, Germany
Reinhard Oeckler
4   Department of Neurosurgery, Schwarzwald-Baar Klinik, Academic University Hospital of Freiburg, Villingen, Germany
› Author Affiliations
Further Information

Publication History

16 November 2014

11 August 2015

Publication Date:
20 November 2015 (online)


Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. HOD typically results from focal lesions interrupting connections from the inferior olive within the dentato-rubro-olivary pathway, a region also known as the triangle of Guillain-Mollaret (TGM) (red nucleus, inferior olivary nucleus, and contralateral dentate nucleus). Clinically, HOD presents classically as palatal tremor and can include dentatorubral tremor and/or ocular myoclonus. The pathologic changes associated with HOD feature radiologic changes with the inferior olivary nucleus appearing larger and increasing its T2-weighted signal intensity on magnetic resonance images. HOD is commonly managed with pharmacotherapy but may require surgical intervention in extreme cases. HOD has been found to develop as a consequence of any injury that disrupts the TGM pathways (e.g., pontine cavernoma).These findings highlight the critical importance of a thorough knowledge of TGM anatomy to avoid secondary HOD. We present a patient who developed HOD secondary to resection of a tectal plate cavernous malformation and review the literature with an emphasis on the current knowledge of this disorder.

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