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      Triggered episodic vestibular syndrome and transient loss of consciousness due to a retrostyloidal vagal schwannoma: a case report

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          Abstract

          Background

          Various conditions may trigger episodic vertigo or dizziness, with positional changes being the most frequently identified condition. In this study, we describe a rare case of triggered episodic vestibular syndrome (EVS) accompanied by transient loss of consciousness (TLOC) linked to retrostyloidal vagal schwannoma.

          Case description

          A 27-year woman with known vestibular migraine presented with a 19-month history of nausea, dysphagia, and odynophagia triggered by swallowing food and followed by recurrent TLOC. These symptoms occurred independently of her body position, resulting in a weight loss of 10 kg within 1 year and in an inability to work. An extensive cardiologic diagnostic work-up undertaken before she presented to the neurologic department was normal. On the fiberoptic endoscopic evaluation of swallowing, she showed a decreased sensitivity, a slight bulging of the right lateral pharyngeal wall, and a pathological pharyngeal squeeze maneuver without any further functional deficits. Quantitative vestibular testing revealed an intact peripheral-vestibular function, and electroencephalography was read as normal. On the brain MRI, a 16 x 15 x 12 mm lesion in the right retrostyloidal space suspicious of a vagal schwannoma was detected. Radiosurgery was preferred over surgical resection, as resection of tumors in the retrostyloid space bears the risk of intraoperative complications and may result in significant morbidity. A single radiosurgical procedure (stereotactic CyberKnife radiosurgery, 1 x 13Gy) accompanied by oral steroids was performed. On follow-up, a cessation of (pre)syncopes was noted 6 months after treatment. Only residual infrequent episodes of mild nausea were triggered by swallowing solid food remained. Brain MRI after 6 months demonstrated no progression of the lesion. In contrast, migraine headaches associated with dizziness remained frequent.

          Discussion

          Distinguishing triggered and spontaneous EVS is important, and identifying specific triggers by structured history-taking is essential. Episodes being elicited by swallowing solid foods and accompanied by (near) TLOC should initiate a thorough search for vagal schwannoma, as symptoms are often disabling, and targeted treatment is available. In the case presented here, cessation of (pre)syncopes and significant reduction of nausea triggered by swallowing was noted with a 6-month delay, illustrating the advantages (no surgical complications) and disadvantages (delayed treatment response) of first-line radiotherapy in vagal schwannoma treatment.

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          Most cited references24

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          Classification of vestibular symptoms: towards an international classification of vestibular disorders.

          Alexandre Bisdorff, Michael von Brevern, Thomas Lempert … (2009)
          Article 0 comments Cited 130 times – based on 0 reviews     Review now
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            Jugular and vagal paragangliomas: Systematic study of management with surgery and radiotherapy.

            Carlos Suarez, Juan Rodrigo, Carsten C Bödeker … (2013)
            The definitive treatment for head and neck paraganglioma (PG) is surgical excision. Unfortunately, surgery, particularly of vagal paraganglioma (VPG; "glomus vagale") and foramen jugulare ("glomus jugulare") tumors, may be complicated by injuries to the lower cranial nerves, a high price to pay for treatment for a benign tumor. Alternatively these tumors may be followed without treatment, or irradiated. The purpose of this review was to compare the existing evidence concerning the efficacy and safety of surgery, external beam radiotherapy (EBRT), and stereotactic radiosurgery (SRS), for jugular paragangliomas (JPGs) and VPGs.
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              PET/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma

              Ingo Janssen, Clara Chen, Corina M Millo … (2016)
              Pheochromocytomas/paragangliomas (PPGLs) and their metastases are tumors that predominantly express somatostatin receptor 2 (SSR2). (68)Ga-DOTA(0)-Tyr(3)-octreotate ((68)Ga-DOTATATE) is a PET radiopharmaceutical with both high and selective affinity for SSRs. The purpose of this study was to evaluate the utility of (68)Ga-DOTATATE in comparison with other specific and nonspecific radiopharmaceuticals recommended in the current guidelines for the localization of metastatic sporadic PPGL by PET/CT.
              Article 0 comments Cited 37 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Journal
                Journal ID (nlm-ta): Front Neurol
                Journal ID (iso-abbrev): Front Neurol
                Journal ID (publisher-id): Front. Neurol.
                Title: Frontiers in Neurology
                Publisher: Frontiers Media S.A.
                ISSN (Electronic): 1664-2295
                Publication date (Electronic): 26 June 2023
                Publication date Collection: 2023
                Volume: 14
                Electronic Location Identifier: 1222697
                Affiliations
                [1] 1Neurology, Cantonal Hospital of Baden , Baden, Switzerland
                [2] 2Department of Radiation Oncology, Bern University Hospital, University of Bern , Bern, Switzerland
                [3] 3Neurosurgery, Bern University Hospital , Bern, Switzerland
                [4] 4Faculty of Medicine, University of Zurich , Zurich, Switzerland
                Author notes

                Edited by: Michael Strupp, Ludwig Maximilian University of Munich, Germany

                Reviewed by: Nicolina Goldschagg, Ludwig Maximilian University of Munich, Germany; Bryan Kevin Ward, Johns Hopkins University, United States

                *Correspondence: Alexander Andrea Tarnutzer alexander.tarnutzer@ 123456ksb.ch
                Article
                DOI: 10.3389/fneur.2023.1222697
                PMC ID: 10330762
                SO-VID: 2a26a1bf-f8e5-4ee5-9bd3-e31723941d2e
                Copyright © Copyright © 2023 Spiegelberg, Ermiş, Raabe and Tarnutzer.
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                Date received : 15 May 2023
                Date accepted : 31 May 2023
                Page count
                Figures: 2, Tables: 0, Equations: 0, References: 24, Pages: 6, Words: 3857
                Categories
                Subject: Neurology
                Subject: Case Report
                Custom metadata
                section-at-acceptance Neuro-Otology

                ScienceOpen disciplines: Neurology
                Keywords: case report,dysphagia,radiotherapy,transient loss of consciousness,dizziness
                Data availability:
                ScienceOpen disciplines: Neurology
                Keywords: case report, dysphagia, radiotherapy, transient loss of consciousness, dizziness

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