Unilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature

doi:10.1016/j.wneu.2017.10.061

World Neurosurgery

Volume 110, February 2018, Pages 294-300

https://doi.org/10.1016/j.wneu.2017.10.061 Get rights and content

Background

Hypertrophic olivary degeneration (HOD) is a rare phenomenon in the dento-rubro-olivary pathway caused by lesion or disruption of the fibers of the Guillain-Mollaret triangle. Hemorrhage of pontine and midbrain cavernous angiomas can rarely lead to HOD portending neurologic deterioration and possible concomitant life-threatening complications; for this reason, it may define a poignant consideration in planning intervention.

Case Description

The patient was a 57-year-old woman with known midbrain-pontine cavernous angioma. For several years, the lesion had been stable, as shown by imaging follow-up, until 10 months before the patient presented with falls, dysarthria, and headache. Imaging showed some decrease in size as well as blood product around the cavernous angioma, suggesting interim period hemorrhage and interval development of HOD.

Conclusions

The literature regarding imaging recommendations for stable cavernous angioma in the midbrain-pontine junction is reviewed. The implication of HOD for patient outcome is discussed and a comment is made on how the development of HOD may affect management of the cavernous angioma.

Introduction

Hypertrophic olivary degeneration (HOD) is a rare entity caused by a lesion in the dento-rubro-olivary tracts or Guillain-Mollaret triangle. It has been noted as a result of resection of midbrain-pontine cavernous angiomas and suggested in case reports as a consequence of interval bleed in midbrain-pontine cavernous angiomas. Herein, we present a case of an adult with multiple known stable cavernous angiomas, including one located in the midbrain with extension into the pons. The patient presented in the interval between imaging follow-up with new-onset falls, dysarthria, and headache and was found to have interval hemorrhage of the midbrain-pontine cavernous angioma with development of HOD. Our purpose is to describe the case, describe the pathophysiology of HOD, review the literature on the development of HOD in the setting of midbrain-pontine cavernous angiomas, and comment on the recommendations for follow-up on these lesions using development of HOD as a measure of progression of these entities.

Section snippets

Clinical History

A 57-year-old woman with past medical history significant for multiple known cavernous angiomas in the frontal and parietal lobes and midbrain as well as hypertension, chronic kidney disease, and ruptured and clipped intracranial aneurysm, presented to the emergency department with worsening gait, imbalance, multiple falls, headache, blurred vision, and dysarthria. The patient also previously had an occipital approach ventriculoperitoneal shunt placed for obstructive hydrocephalus.

Imaging Findings

Initial

Pathogenesis

HOD is a rare phenomenon in the dento-rubro-olivary pathway, first described by Oppenheim in 1887.¹ It occurs as a consequence of a lesion or disruption in the dento-rubro-olivary pathway, which was defined by Guillain and Mollaret. Thus, it is referred to as the Guillain-Mollaret triangle, as shown in Figure 3.² The triangle is formed by the parvocellular red nucleus, the inferior olivary nucleus (ION), and contralateral dentate nucleus. The parvocellular red nucleus receives input from the

Conclusions

This case shows neurologic deterioration after interval bleed of the known midbrain-pontine junction cavernous angioma with interval development of HOD. Appearance of HOD has been reported in the literature after resection of midbrain-pontine cavernous angiomas. HOD has also been seen in several reports of midbrain-pontine cavernous angiomas before resection at the time of hemorrhage. This finding may represent a compelling benchmark for intervention given that it 1) is a possible complication

Acknowledgments

The authors are very appreciative of Dr. Daniele Rigamonti for his thorough review and expert insight of this article.

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Holmes tremor in a monocentric series of resected brainstem cavernomas
2021, Neurochirurgie
Citation Excerpt :
HOD is considered to be associated to the late occurrence of HT, the delay between brainstem lesion and HT varies in the literature but generally arise at around four weeks to two years. This may be associated to trans-synaptic degeneration, secondary to disruption in the dento-rubro-olivary pathway referred to as the Guillain-Mollaret triangle [29,36–38]. For other authors the latent period before occurrence of HT is induced by the plasticity of the affected systems, particularly the nigrostrial system [29].
Several scientific papers report clinical symptoms, indications, complications and outcomes of brainstem cavernous malformation (BSCM) surgery without reporting on the occurrence of postoperative Holmes tremor (HT). Our purpose is to report our experience with HT in a monocentric series of resected brainstem cavernomas.
We reviewed all the BSCM surgical records between 2002 and 2018 at Saint-Luc University Hospital's Department of Neurosurgery, Brussels and selected patients developing HT postoperatively. Patients’ demographics, symptoms, pre- and postoperative imaging, recurrence and complications were analysed. A PubMed literature review was performed to compare our results with those in the existing literature.
In a total series of 18 resected BSCM, 5 patients: 1 male and 4 females, with a median age of 51 years (range 29–59 years), developed HT. The median preoperative mRS score was 2 (range 1–4). GTR was achieved in all patients without surgery-related death. BSCM were located in the mesencephalon in 4 patients (80%) who developed HT. Tremor was noticed between ten days and one year after surgery. One patient saw significant improvements to the point of stopping treatment. The median follow-up period was 2 years (range 1–14 years). At the last follow-up, 40% of our patients showed a worse mRS score, 40% stayed unchanged, and 20% improved.
We are reporting an original single-center series of patients suffering from HT after BSCM surgery. The risk for HT after surgery is significant for midbrain BSCM. A spontaneous favorable evolution is possible.
Nombreux articles scientifiques décrivent les symptômes, les indications chirurgicales, les complications et les résultats postopératoires des cavernomes du tronc cérébral sans rapporter l’apparition de tremblement de Holmes. Notre objectif est de rapporter ici notre expérience avec le tremblement de Holmes dans une série monocentrique de cavernomes du tronc cérébral opérés.
Nous avons revu les dossiers de tous les patients opérés d’un cavernome du tronc cérébral dans le service de neurochirurgie des cliniques universitaires Saint-Luc, Bruxelles et avons sélectionné tous les patients qui ont présenté un tremblement de Holmes dans la période postopératoire. L’âge et le sexe des patients, leurs symptômes, leurs imageries pré- et postopératoires, les récidives et les complications postopératoires ont été analysées. Une revue de la littérature existante sur la plateforme PubMed a été réalisée afin de comparer nos résultats avec ceux déjà publiés.
Dans une série de 18 cavernomes du tronc cérébral opérés, 5 patients : 1 homme et 4 femmes, avec un âge médian de 51 ans (allant de 29 à 59 ans), ont présenté un tremblement de Holmes dans les suites d’une chirurgie pour un cavernome du tronc cérébral. Le score mRS préopératoire médian était 2 (score de 1 à 4). Une résection macroscopiquement complète a été obtenue chez tous les patients sans décès liés à la chirurgie. Quatre (80 %) des cinq patients ayant développé un tremblement de Holmes présentaient un cavernome situé dans le mésencéphale. Un patient a présenté une amélioration significative de son tremblement au point de pouvoir arrêter son traitement. Le suivi postopératoire médian a été de 2 ans (allant de 1 à 2 ans). Au dernier contact, 40 % des patients présentaient une aggravation de leur clinique par rapport à la période préopératoire, 40 % étaient stables et 20 % présentaient une évolution favorable.
Nous décrivons une série monocentrique originale de patients souffrant d’un tremblement de Holmes après l’exérèse d’un cavernome du tronc cérébral. Le risque de développer cette complication est significatif après résection d’un cavernome mésencéphalique. Une évolution spontanément favorable est possible.
Hypertrophic olivary degeneration: A comprehensive review focusing on etiology
2019, Brain Research
Citation Excerpt :
The condition of HOD was first reported by the German physiologist Oppenheim in 1887; who describe an enlargement of the ION in his postmortem study (Oppenheim, 1887; Martins et al., 2016a,b). However, it was not until 1931 that French physicians Guillain and Mollaret described the anatomy of this condition, who described HOD in a patient with oculopalatal myoclonus and a lesion on the ION (Guillain, 1931; Rosenblum et al., 2018), providing a better understanding of the disease process. This degeneration is unique; in that, it causes hypertrophy of the ION rather than atrophy.
Hypertrophic olivary degeneration (HOD) is a rare form of trans-synaptic 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 (DROP), a region also known as the Guillain-Mollaret triangle (GMT) (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. Oppenheim first described the enlargement of the ION in his post-mortem study. Since then, a limited number of cases have been reported in the literatures. Thus, we intended to describe the definition, pathophysiology, clinical features, radiological features, diagnosis, and current management of HOD. We provide a comprehensive review of HOD focusing on etiology. The present review may lead to a better understanding of HOD clinical characteristic with the goal of contributing to existing knowledge of this rare disease.
An acute clinical presentation associated with hypertrophic olivary degeneration
2019, Canadian Journal of Neurological Sciences
Atlas of Nervous System Vascular Malformations: A Systematic Review
2022, Life
Hypertrophic olivary degeneration: a description of four cases of and a literature analysis
2022, Quantitative Imaging in Medicine and Surgery
A brief history of cerebral cavernous malformations: A personal perspective
2021, Vessel Plus

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: Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

^∗: Present address: BWH Neurosurgery.

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Case ReportUnilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature

Background

Case Description

Conclusions

Introduction

Section snippets

Clinical History

Imaging Findings

Pathogenesis

Conclusions

Acknowledgments

Parkinsonism Relat Disord

J Clin Neurosci

Surg Neurol

World Neurosurg

Hypertrophic olivary degeneration after surgical removal of cavernous malformations of the brain stem: report of four cases and review of the literature

Acta Neurochir (Wien)

Hypertrophic olivary degeneration: MR imaging and pathologic findings

Radiology

Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings

AJNR Am J Neuroradiol

The dentato-olivary pathway. Somatotopic relationship between the dentate nucleus and the contralateral inferior olive

Arch Neurol

Hypertrophic olivary degeneration: The forgotten triangle of Guillain and Mollaret

Neurol India

The pathophysiological basis for hypertrophic olivary degeneration (HOD) following brainstem insult

JSM Neurosurg Spine

La oliva bulbar: su estructura, función y patología

Rev Neuro-Psiquat

MR imaging of presumed olivary hypertrophy in palatal myoclonus

AJNR Am J Neuroradiol

Cerebellar MR changes in patients with olivary hypertrophic degeneration

AJNR Am J Neuroradiol

Enlargement of the inferior olivary nucleus in association with lesions of the central tegmental tract or dentate nucleus

Brain

Bilateral hypertrophic olivary degeneration

Ann Indian Acad Neurol

Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome

Br J Radiol

Frequency of bilateral hypertrophic olivary degeneration in a large retrospective cohort

J Neuroimaging

Symptomatic and essential palatal tremor. 1. Clinical, physiological and MRI analysis

Brain

Midbrain tremor and € hypertrophic olivary degeneration after pontine hemorrhage

Mov Disord

A rare presentation of hypertrophic olivary degeneration secondary to primary central nervous system lymphoma

JAMA Neurol

Hypertrophic olivary degeneration and Holmes' tremor secondary to bleeding of cavernous malformation in the midbrain

Tremor Other Hyperkinet Mov (N Y)

Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy

Brain

Hypertrophic olivary degeneration following pontine haemorrhage: hypertensive crisis or cavernous haemangioma bleeding?

J Neurol Neurosurg Psychiatry

Essential palatal myoclonus following dental surgery: a case report

J Med Case Rep

Case Report
Unilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature