Cervical vagal nerve schwannoma induced arrhythmia: a rare case report and literature review.

BACKGROUND: Schwannomas are benign tumors deriving from the sheath of cranial and peripheral nerves. The vagus nerve is comprised of a complex neuro-endocrine-immune network that maintains homeostasis, most tracts of it play a role in parasympathetic activity. We present an example of a rare cervical vagal schwannoma case accompanied by arrhythmia. CASE PRESENTATION: A 35-year-old female patient with a left cervical vagus schwannoma and ventricular arrhythmia underwent schwannoma resection in the operating room. The patient's suppressed heart rate increased after tumor removal, and the cardiac rhythm returned to normal postoperatively. Pathological examination demonstrated the diagnosis of schwannoma. CONCLUSIONS: This case explains the link between the vagus nerve and the cardiovascular system, proving that a damaged cervical vagus nerve can inhibit the heart rate and lead to arrhythmias, and eventually requiring surgical intervention.

Vagal dysfunction and small intestinal bacterial overgrowth: novel pathways to chronic inflammation in HIV.

OBJECTIVE: Chronic inflammation in HIV-infected individuals drives disease progression and the development of comorbidities, despite viral suppression with combined antiretroviral therapy. Here, we sought evidence that vagal dysfunction, which occurs commonly as part of HIV-associated autonomic neuropathy, could exacerbate inflammation through gastrointestinal dysmotility, small intestinal bacterial overgrowth (SIBO), and alterations in patterns of soluble immune mediators. DESIGN: This is a cross-sectional observational study. METHODS: Forty participants on stable combined antiretroviral therapy with gastrointestinal symptoms, and no causes for vagal or gastrointestinal dysfunction other than HIV, underwent autonomic testing, hydrogen/methane breath testing for SIBO, and gastric emptying scintigraphy. A panel of 41 cytokines, high-mobility group box 1, and markers of bacterial translocation (lipopolysaccharide) and monocyte/macrophage activation (sCD14 and sCD163) were tested in plasma. RESULTS: We found that participants with vagal dysfunction had delayed gastric emptying and higher prevalence of SIBO. SIBO was associated with IL-6, but not sCD14; lipopolysaccharide could not be detected in any participant. We also found alteration of cytokine networks in participants with vagal dysfunction, with stronger and more numerous positive correlations between cytokines. In the vagal dysfunction group, high mobility group box 1 was the only soluble mediator displaying strong negative correlations with other cytokines, especially those cytokines that had numerous other strong positive correlations. CONCLUSION: The current study provides evidence that the vagal component of HIV-associated autonomic neuropathy is associated with changes in immune and gastrointestinal function in individuals with well treated HIV. Further study will be needed to understand whether therapies targeted at enhancing vagal function could be of benefit in HIV.

A rare case of bilateral vagus nerve schwanomatosis.

Schwanomatosis is the third most common form of neurofibromatosis. Schwanomatosis affecting the vagus nerve is particularly rare. In this report, we describe an extremely rare case bilateral vagus nerve schwanomatosis in a 45-year-old male patient. The patient initially presented with bilateral neck tumors and hoarseness arising after thoracic surgery. We performed left neck surgery in order to diagnose and resect the remaining tumors followed by laryngeal framework surgery to improve vocal cord closure and symptoms of hoarseness. Voice recovery was successfully achieved after surgery. An appropriate diagnosis and surgical tumor resection followed by phonosurgery improved patient quality of life in this rare case.

Impaired vagus function in rats suppresses bile acid synthesis in the liver by disrupting tight junctions and activating Fxr-Fgf15 signaling in the intestine.

Bile acids (BAs) circulate between the liver and intestine, and regulate the homeostasis of glucose, lipid, and energy. Recent studies demonstrated an essential role of BAs in neurological diseases, suggesting an interaction between BAs and the nervous system. In the present study, we showed that impaired vagus function in rats induced by vagotomy resulted in an increase in bile flow without causing liver injury. The concentrations of unconjugated and glycine-conjugated BAs were increased in both serum and bile of rats after vagotomy, which was due to impaired tight junctions and thus increased passive absorption of BAs in the intestine. Vagotomy markedly suppressed the expression of the rate-limiting BA synthetic enzyme Cyp7a1, which was not due to activation of Fxr-Shp signaling in the liver, but due to activation of Fxr-Fgf15 signaling in the intestine. Furthermore, vagotomy produced a BA profile in the bile favorable for Fxr activation by decreasing tauro-ß-muricholic acid, a natural Fxr antagonist, and increasing glyco-chenodeoxycholic acid, a natural Fxr agonist. In summary, the present study provides the first comprehensive analysis of the critical role of the vagus nerve in regulating BA metabolism and signaling pathway.

Polymyalgia rheumatica and vagal paraganglioma.

INTRODUCTION: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome. CASE REPORT: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica. CONCLUSION: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis.

Extracranial schwannoma in the carotid space: A retrospective review of 91 cases.

BACKGROUND: Schwannomas of the vagus nerve and cervical sympathetic nerve are rare; hence, only limited information exists regarding their diagnosis and clinical management. METHODS: We conducted a retrospective review of the clinical features, imaging studies, and treatment results of patients with schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. RESULTS: Of 91 patients, 91% (n = 83) were preoperatively diagnosed with schwannoma tumors. Using the hyoid bone as an anatomic landmark, the location of the schwannoma of the vagus nerve in the carotid space was significantly different to the location of schwannoma of the sympathetic nerve (p = .003). Although 52 of the 76 patients followed up (68%) had postoperative nerve weaknesses, 13 patients (50%) and 14 patients (53.8%), respectively, fully recovered from schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. CONCLUSION: In the carotid space, schwannomas of the vagus nerve are usually located below the hyoid bone, whereas schwannomas of the sympathetic nerve more commonly arise from the suprahyoid compartment. Accurate preoperative diagnosis and the intracapsular enucleation surgical approach decreased the incidence of postoperative morbidity. © 2016 Wiley Periodicals, Head Neck 39: 42-47, 2017.

Repeat Imaging in Idiopathic Unilateral Vocal Fold Paralysis: Is It Necessary?

OBJECTIVE: Imaging plays a critical role in the evaluation of patients presenting with unilateral vocal fold paresis or paralysis of unknown etiology. In those with idiopathic unilateral vocal fold paralysis (iUVFP), there is no consensus regarding the need or timing of repeat imaging. This study seeks to establish the rate of delayed detection of alternate etiologies for these patients to determine if and when imaging should be repeated. METHODS: Retrospective chart review was conducted identifying patients at our institution with vocal fold movement impairment between 1998 and 2014. Idiopathic paralysis was diagnosed if physical examination, laryngoscopy, and initial imaging excluded a cause. Demographic data, length of follow-up, and the presence of late lesions were noted. Time to detection was plotted using the Kaplan-Meier method. RESULTS: Of 3210 patients reviewed, 207 had a diagnosis of iUVFP. Of these patients, 8 went on to develop alternate diagnoses, including pulmonary disease, skull-base and laryngeal neoplasms, and thyroid malignancy. In Kaplan-Meir analysis, 90% remained "idiopathic" at 5 years of follow-up. The mean time to detection was 27 months. CONCLUSIONS: Patients initially diagnosed with iUVFP may have an occult cause that later becomes evident. We recommend repeat imaging within 2 years after diagnosis, but this is likely unnecessary beyond 5 years.

Vagoglossopharyngeal Neuralgia Occurred Concomitantly with Ipsilateral Hemifacial Spasm and Versive Seizure-Like Movement: A First Case Report.

Vagoglossopharyngeal neuralgia (VGPN) is a very rare condition. VGPN with convulsive like attack is even rarer All of the cases had their head turned to the opposite side of facial pain. Hemifacial spasm occurring concurrently with VGPN has never been reported. Herein, we present the first case of VGPN that had ipsilateral hemifacial spasm and versive seizure-like movement to the same side of facial pain. We reported a 71-year-old man presenting with multiple episodes of intermittent sharp shooting pain arising on the right middle neck, followed by hemifacial spasm on right face. Then the patient became syncope while his head and gaze turned to the same side of the painful neck. Electrocardiography showed sinus arrest. Interictal Electroencephalography was normal. This patient initially responded to pregabalin for two weeks, then the symptoms became worse. Microvascular decompression and carbamazepine resulted in the complete remission of all symptoms after six months of follow-up. We could not explain the pathophysiology of unilateral versive seizure like movement.

Vagoglossopharyngeal neuralgia revealed through predominant digestive vagal manifestations. Case report and literature review.

Vagoglossopharyngeal neuralgia is a rare pathology whose atypical forms, dominated by syncopal manifestations, are still rarer. Although the territory of the vagus nerve involves, beyond the cardiovascular system, the respiratory and the digestive systems, there is no report in literature of atypical forms other than syncopal. Therefore, the authors were prompted to report the case of a patient whose vagoglossopharyngeal neuralgia was predominantly revealed by digestive symptoms. A 58-year-old patient presented with stereotypical severe digestive disturbances including nausea, vomiting and diarrhoea. High definition cranial MRI showed a neurovascular conflict between the posterior inferior cerebellar artery and the IXth and Xth nerves, on the right side. A microsurgical decompression was carried out which confirmed the vascular compression and successful transposition of the artery. One year after the surgery, the patient was free from all painful and digestive symptoms. A survey of the literature did not find any reference to digestive symptoms together with the neuralgia; only a syncopal type of cardiac symptoms related to the parasympathetic nervous system were described. The hypothesis was that the revealing digestive symptoms are linked to a similar parasympathetic mechanism, implying the visceral component of the Xth cranial nerve.

Case Report of Lewis and Sumner Syndrome with Bilateral Vagus Nerves Paralysis for 16 Years.

This report describes a patient with dysphonia for 16 years in combination with asymmetric and progressive decrease in sense and power of both upper and lower extremities for the past 3 years. Electrophysiological study revealed asymmetric conduction block and abnormal sensory action potential in 4 limbs. The vagus nerves palsy and abnormal electrodiagnosis of the limbs led us to diagnose the disease as Lewis and Sumner syndrome, also called multifocal acquired demyelinating sensory and motor neuropathy diagnosis, which improved by corticosteroid consumption to some extent. This case is uncommon by its long time presentation and progression. To the best of the authors' knowledge, this is the first report of simultaneous bilateral vagus nerve palsy in combination with upper and lower limbs' demyelinating neuropathy. In conclusion, persistent dysphonia can be a part of the presentation of demyelinating neuropathy.

Superiority of resection over enucleation for schwannomas of the cervical vagus nerve: A retrospective cohort study of 22 consecutive patients.

INTRODUCTION: Schwannoma of the cervical vagus nerve is rare. Treatment options include intracapsular enucleation and en bloc resection. The purpose of this study was to compare the outcomes of enucleation and resection in terms of postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence, quality-adjusted life-year (QALY) and vocal handicap index (VHI). METHODS: Twentytwo consecutive patients were divided into two groups. Patients in group A (n = 9) underwent intracapsular enucleation, whereas patients in Group B (n = 13) underwent en bloc resection. Main endpoints of the study were postoperative mortality and morbidity, freedom from vocal cord palsy, freedom from local recurrence and quality of life. The quality of life after surgery was assessed according to the quality-adjusted life-year (QALY) EQ-5D-5L methodology, and calculation of the voice handicap index (VHI). RESULTS: Postoperative mortality was nil. Morbidity included 1 wound dehiscence in group A and 2 transitory dysphagias in group B. Freedom from vocal cord palsy was 22% in group A and zero in group B (p = 0.15). Operation-specific local recurrence rate was 33% (3/9 patients) in group A and nil in group B (0/23 patients) (p = 0.05). QALYs was 0.55 in group A and 0.54 in group B (p = 1.0). VHI was 23.77 in group A and 26.15 in group B (p = 1.00). CONCLUSION: Resection is superior to enucleation in terms of freedom from local recurrence. Functional results are comparable for both techniques.

Ganglioneuromas involving the hypoglossal nerve and the vagus nerve in a child: Surgical difficulties.

Ganglioneuromas are benign tumors that arise from the Schwann cells of the autonomic nervous system. They are usually seen in the posterior mediastinum and the paraspinal retroperitoneum in relation to the sympathetic chain. In the head and neck, they are usually related to the cervical sympathetic ganglia or to the ganglion nodosum of the vagus nerve or the hypoglossal nerve. We describe what we believe is the first reported case of multiple ganglioneuromas of the parapharyngeal space in which two separate cranial nerves were involved. The patient was a 10-year-old girl who presented with a 2-year history of a painless and slowly progressive swelling on the left side of her neck and a 1-year history hoarseness. She had no history of relevant trauma or surgery. Intraoperatively, we found two tumors in the left parapharyngeal space-one that had arisen from the hypoglossal nerve and the other from the vagus nerve. Both ganglioneuromas were surgically removed, but the affected nerves had to be sacrificed. Postoperatively, the patient exhibited hypoglossal nerve and vocal fold palsy, but she was asymptomatic. In addition to the case description, we discuss the difficulties we faced during surgical excision.

[Vagal Nerve Schwannoma Complicated with Bloody Pleural Effusion；Report of a Case].

A 51-year-old male who had received hemodialysis twice a week was referred to our hospital for a further examination of bloody pleural effusion in the right chest. He has been suffering from a fever and cough for 2 months. Chest computed tomography and magnetic resonance imaging revealed a pleural effusion in the right pleural cavity and posterior mediastinal tumor in paravertebral lesion. Chest drainage was performed, and cytological diagnosis did not show malignant findings. To make a definite diagnosis and treatment, surgical resection was carried out. During surgery, posterior mediastinal tumor originated from vagal nerve, and a schwannoma was diagnosed by frozen section. After resection, postoperative course was uneventful, and bloody pleural effusion disappeared.

Massive hemoptysis: a rare presentation of posterior mediastinal, giant, benign vagal schwannoma.

Schwannomas are rare neurogenic tumor originating from Schwann cells of the nerve sheath, most frequently encountered type of posterior mediastinal tumors. In most cases, schwannomas are benign, malignant and multiple schwannomas are rare. Histopathologically, the tumor is composed of fascicles of spindle cells, which are strongly positive for S-100 proteins. Surgical resection is a treatment of choice, and prognosis is excellent. Here, we report a case of posterior mediastinal schwannoma in a 20- years old male patient who complained of right-sided back pain and two episodes of massive hemoptysis of recent onset. Contrast enhanced computed tomography (CECT) and magnetic resonance imaging of the chest showed a well circumscribed, heterogeneous mass in the posterior mediastinum, compressing the right lower lobe with widening of intervertebral foramens. CT-guided trucut biopsy revealed spindle cell neoplasm. On immunohistochemistry, tumor cells expressed strong positivity for S-100 protein. Final diagnosis was schwannoma, probably originating from the right vagus nerve. Surgical resection of the encapsulated tumor resulted in the successful recovery, without any recurrence over next one year follow up.

Cough after laryngeal herpes zoster: a new aspect of post-herpetic sensory disturbance.

OBJECTIVE: Although neurogenic cough is increasingly recognised, its pathophysiology remains obscure. We describe two cases of chronic cough following laryngeal herpes zoster, a rarely described manifestation of varicella-zoster virus reactivation, and suggest that this may be analogous to post-herpetic neuralgia. The same mechanisms may cause both phenomena. CASE REPORTS: We describe two cases of chronic cough persisting for more than three months following an acute attack of laryngeal herpes zoster. CONCLUSION: Neuronal damage by varicella-zoster virus results in irritable nociceptors and deafferentation, mechanisms known to cause post-herpetic neuralgia. When the vagus nerve is affected, as in laryngeal herpes zoster, the result may be a chronic cough. Similar damage may underlie chronic neurogenic cough in other contexts.

Chronic fatigue syndrome from vagus nerve infection: a psychoneuroimmunological hypothesis.

Chronic fatigue syndrome (CFS) is an often-debilitating condition of unknown origin. There is a general consensus among CFS researchers that the symptoms seem to reflect an ongoing immune response, perhaps due to viral infection. Thus, most CFS research has focused upon trying to uncover that putative immune system dysfunction or specific pathogenic agent. However, no single causative agent has been found. In this speculative article, I describe a new hypothesis for the etiology of CFS: infection of the vagus nerve. When immune cells of otherwise healthy individuals detect any peripheral infection, they release proinflammatory cytokines. Chemoreceptors of the sensory vagus nerve detect these localized proinflammatory cytokines, and send a signal to the brain to initiate sickness behavior. Sickness behavior is an involuntary response that includes fatigue, fever, myalgia, depression, and other symptoms that overlap with CFS. The vagus nerve infection hypothesis of CFS contends that CFS symptoms are a pathologically exaggerated version of normal sickness behavior that can occur when sensory vagal ganglia or paraganglia are themselves infected with any virus or bacteria. Drawing upon relevant findings from the neuropathic pain literature, I explain how pathogen-activated glial cells can bombard the sensory vagus nerve with proinflammatory cytokines and other neuroexcitatory substances, initiating an exaggerated and intractable sickness behavior signal. According to this hypothesis, any pathogenic infection of the vagus nerve can cause CFS, which resolves the ongoing controversy about finding a single pathogen. The vagus nerve infection hypothesis offers testable hypotheses for researchers, animal models, and specific treatment strategies.