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      Upbeat vertical nystagmus after brain stem cavernoma resection: a rare case of nucleus intercalatus/nucleus of roller injury

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          Abstract

          Introduction

          CNS cavernomas are a type of raspberry-shaped vascular malformations that are typically asymptomatic, but can result in haemorrhage, neurological injury, and seizures. Here, we present a rare case of a brainstem cavernoma that was surgically resected whereafter an upbeat nystagmus presented postoperatively.

          Case report

          A 42-year old man presented with sudden-onset nausea, vomiting, vertigo, blurred vision, marked imbalance and difficulty swallowing. Neurological evaluation showed bilateral ataxia, generalized hyperreflexia with left-sided predominance, predominantly horizontal gaze evoked nystagmus on right and left gaze, slight left labial asymmetry, uvula deviation to the right, and tongue deviation to the left. MRI demonstrated a 13-mm cavernoma with haemorrhage and oedema in the medulla oblongata. Surgery was performed via a minimal-invasive, midline approach. Complete excision was confirmed on postoperative MRI. The patient recovered well and became almost neurologically intact. However, he complained of mainly vertical oscillopsia. The videonystagmography revealed a new-onset spontaneous upbeat nystagmus in all gaze directions, not suppressed by fixation. An injury of the rarely described intercalatus nucleus/nucleus of Roller is thought to be the cause.

          Conclusion

          Upbeat nystagmus can be related to several lesions of the brainstem, including the medial longitudinal fasciculus, the pons, and the dorsal medulla. To our knowledge, this is the first case of an iatrogenic lesion of the nucleus intercalatus/nucleus of Roller resulting in an upbeat vertical nystagmus. For neurologists, it is important to be aware of the function of this nucleus for assessment of clinical manifestations due to lesions within this region.

          Electronic supplementary material

          The online version of this article (10.1007/s00415-020-09891-4) contains supplementary material, which is available to authorized users.

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          Most cited references18

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          Vertical nystagmus: clinical facts and hypotheses.

          The pathophysiology of spontaneous upbeat (UBN) and downbeat (DBN) nystagmus is reviewed in the light of several instructive clinical findings and experimental data. UBN due to pontine lesions could result from damage to the ventral tegmental tract (VTT), originating in the superior vestibular nucleus (SVN), coursing through the ventral pons and transmitting excitatory upward vestibular signals to the third nerve nucleus. A VTT lesion probably leads to relative hypoactivity of the drive to the motoneurons of the elevator muscles with, consequently, an imbalance between the downward and upward systems, resulting in a downward slow phase. The results observed in internuclear ophthalmoplegia suggest that the medial longitudinal fasciculus (MLF) is involved in the transmission of both upward and downward vestibular signals. Since no clinical cases of DBN due to focal brainstem damage have been reported, it may be assumed that the transmission of downward vestibular signals depends only upon the MLF, whereas that of upward vestibular signals involves both the MLF and the VTT. The main focal lesions resulting in DBN affect the cerebellar flocculus and/or paraflocculus. Apparently, this structure tonically inhibits the SVN and its excitatory efferent tract (i.e. the VTT) but not the downward vestibular system. Therefore, a floccular lesion could result in a disinhibition of the SVN-VTT pathway with, consequently, relative hyperactivity of the drive to the motoneurons of the elevator muscles, resulting in an upward slow phase. UBN also results from lesions affecting the caudal medulla. An area in this region could form part of a feedback loop involved in upward gaze-holding, originating in a collateral branch of the VTT and comprising the caudal medulla, the flocculus and the SVN, successively. Therefore, it is suggested that the main types of spontaneous vertical nystagmus due to focal central lesions result from a primary dysfunction of the SVN-VTT pathway, which becomes hypoactive after pontine or caudal medullary lesions, thereby eliciting UBN, and hyperactive after floccular lesions, thereby eliciting DBN. Lastly, since gravity influences UBN and DBN and may facilitate the downward vestibular system and restrain the upward vestibular system, it is hypothesized that the excitatory SVN-VTT pathway, along with its specific floccular inhibition, has developed to counteract the gravity pull. This anatomical hyperdevelopment is apparently associated with a physiological upward velocity bias, since the gain of all upward slow eye movements is greater than that of downward slow eye movements in normal human subjects and in monkeys.
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            Brainstem Cavernous Malformations

            Once considered inoperable lesions in inviolable territory, brainstem cavernous malformations (BSCM) are now surgically curable with acceptable operative morbidity. Recommending surgery is a difficult decision that would be facilitated by a grading system designed specifically for BSCMs that predicted surgical outcomes.
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              Cerebral cavernomas and seizures: a retrospective study on 163 patients who underwent pure lesionectomy.

              The objective was to evaluate the outcome of microsurgical "pure" lesionectomy in patients with supratentorial cavernous angiomas presenting with seizures. For this retrospective study 163 patients with cavernoma-related epileptic seizures were selected. They all underwent surgery in a single institution between 1988 and 2003. A microsurgical frame/frameless guided minimally invasive transulcal "pure" lesionectomy was performed. The haemosiderin stained gliotic brain parenchyma that was usually found surrounding the lesion was not removed. Among the 99 patients with epilepsy and longer clinical history, 68 (68.7%) were found completely to be seizure-free, 10 (10.1%) presented sporadic and less frequent seizures and 17 (17.1%) remained unchanged. Sixty-three out of 64 (98.4%) patients who experienced only single or sporadic seizures were found to be completely seizure-free after surgery. Five patients were lost at follow-up (mean 48 months, range 0.5-14 years). Long-term morbidity was 1.8%. Mortality was null. No haemorrhagic episodes were observed during follow-up. Pure lesionectomy prevents bleeding and development of epilepsy in patients that receive early surgery after the epileptic onset. In most of the epileptic patients with a good concordance between the electroclinical data and the location of the angioma, good results can be achieved by this kind of surgery so that more invasive and costly studies to find and remove the epileptogenic cerebral parenchyma seem justified only after lesionectomy fails.
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                Author and article information

                Contributors
                Torstein.Meling@hcuge.ch
                Journal
                J Neurol
                J. Neurol
                Journal of Neurology
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                0340-5354
                1432-1459
                26 May 2020
                26 May 2020
                2020
                : 267
                : 10
                : 2865-2870
                Affiliations
                [1 ]Department of Neurosurgery, Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil 4, 1205 Geneva, Switzerland
                [2 ]GRID grid.8591.5, ISNI 0000 0001 2322 4988, Faculty of Medicine, , University of Geneva, ; Geneva, Switzerland
                [3 ]GRID grid.150338.c, ISNI 0000 0001 0721 9812, Division of Otorhinolaryngology Head and Neck Surgery, , Geneva University Hospitals, ; Geneva, Switzerland
                [4 ]GRID grid.150338.c, ISNI 0000 0001 0721 9812, Department of Diagnostic Neuroradiology, , Geneva University Hospitals, ; Geneva, Switzerland
                [5 ]GRID grid.12366.30, ISNI 0000 0001 2182 6141, UMR 1253, iBrain, , Université de Tours, Inserm, ; Tours, France
                [6 ]GRID grid.411167.4, ISNI 0000 0004 1765 1600, CHRU de Tours, ; Tours, France
                [7 ]GRID grid.5510.1, ISNI 0000 0004 1936 8921, Institute of Clinical Medicine, Faculty of Medicine, , University of Oslo, ; Oslo, Norway
                Author information
                http://orcid.org/0000-0001-6595-0873
                Article
                9891
                10.1007/s00415-020-09891-4
                7501124
                32458196
                ee00d44a-2ffe-4012-993b-010e319a4cf5
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 25 February 2020
                : 2 May 2020
                : 5 May 2020
                Categories
                Original Communication
                Custom metadata
                © Springer-Verlag GmbH Germany, part of Springer Nature 2020

                Neurology
                cavernoma,nucleus intercalatus,nystagmus,complication
                Neurology
                cavernoma, nucleus intercalatus, nystagmus, complication

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