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.

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.

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.

[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.

Subdiaphragmatic vagotomy increases the sensitivity of lumbar Aδ primary afferent neurons along with voltage-dependent potassium channels in rats.

Subdiaphragmatic vagal dysfunction causes chronic pain. To verify whether this chronic pain is accompanied by enhanced peripheral nociceptive sensitivity, we evaluated primary afferent neuronal excitability in subdiaphragmatic vagotomized (SDV) rats. SDV rats showed a decrease in the electrical stimuli-induced hind limb-flexion threshold at 250 Hz, but showed no similar effect at 5 or 2000 Hz, which indicated that lumbar primary afferent Aδ sensitivity was enhanced in SDV rats. The whole-cell patch-clamp technique also revealed the hyper-excitability of acutely dissociated medium-sized lumbar dorsal root ganglion (DRG) neurons isolated from SDV rats. The contribution of changes in voltage-dependent potassium (Kv) channels was assessed, and transient A-type K(+) (I(A) ) current density was apparently decreased. Moreover, Kv4.3 immunoreactivity in medium-sized DRG neurons was significantly reduced in SDV rats compared to sham. These results indicate that SDV causes hyper-excitability of lumbar primary Aδ afferent neurons, which may be induced along with suppressing I(A) currents via the decreased expression of Kv4.3. Thus, peripheral Aδ neuroplasticity may contribute to the chronic lower limb pain caused by SDV.

Schwannoma originating from lower cranial nerves: report of 4 cases.

Four cases of schwannoma originating from the lower cranial nerves are presented. Case 1 is a schwannoma of the vagus nerve in the parapharyngeal space. The operation was performed by the transcervical approach. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia happened transiently after the operation. Case 2 is a schwannoma in the jugular foramen. The operation was performed by the infralabyrinthine approach. Although only the intracapsular tumor was enucleated, facial palsy, hoarseness, dysphagia and paresis of the deltoid muscle occurred transiently after the operation. The patient's hearing had also slightly deteriorated. Case 3 is a dumbbell-typed schwannoma originating from the hypoglossal nerve. The hypoglossal canal was markedly enlarged by the tumor. As the hypoglossal nerves were embedded in the tumor, the tumor around the hypoglossal nerves was not resected. The tumor was significantly enlarged for a while after stereotactic irradiation. Case 4 is an intracranial cystic schwannoma originating from the IXth or Xth cranial nerves. The tumor was resected through the cerebello-medullary fissure. The tumor capsule attached to the brain stem was not removed. Hoarseness and dysphagia happened transiently after the operation. Cranial nerve palsy readily occurs after the removal of the schwannoma originating from the lower cranial nerves. Mechanical injury caused by retraction, extension and compression of the nerve and heat injury during the drilling of the petrous bone should be cautiously avoided.

Pre-operative cerebellar mutism secondary to vagus nerve schwannoma.

Cerebellar mutism, also known as 'posterior fossa syndrome,' is an uncommon condition often reported after posterior fossa tumour resection in the paediatric population. It is infrequently associated with other intrinsic cerebellar pathologies. We hereby report a rare case of pre-operative cerebellar mutism associated with an extrinsic posterior fossa lesion - vagus nerve schwannoma.

Lesion of the nucleus solitarius leads to impaired laryngeal sensation in bulbar palsy patients.

BACKGROUND: In order to clarify the laryngeal sensation of bulbar palsy patients, we studied the relationship between laryngopharyngeal sensation and brainstem lesion in patients with dysphagia caused by bulbar palsy. METHODS: Fifteen patients with lateral medullary infarction and dysphagia were included in this study. We performed laryngeal sensory test using the flexible laryngoscope and probes method previously developed by Yaguchi et al. The test sites included the right and left tip of the laryngeal surface of the epiglottis and bilateral arytenoid regions. Lesion sites were identified by magnetic resonance imaging and classified horizontally according to Kim's classification. We also used the anatomical atlas Cytoarchitecture of the Human Brain Stem to determine whether the lesions included the nucleus solitarius and nucleus ambiguus. RESULTS: Eight cases had normal sensation and 7 cases had decreased sensation of the affected side of the epiglottis and arytenoid region. The lesions of decreased laryngeal sensation group were classified horizontally as large type or dorsal type and included the nucleus solitarius. Decreased laryngeal sensation was significantly correlated with lesions that included the nucleus solitarius (Fisher exact test; P = .026). CONCLUSIONS: This study clarifies that patients with dysphagia caused by bulbar palsy may present with laryngeal sensory impairment of the affected side and laryngopharyngeal movement disorder. The important finding is that damage to both the nucleus solitarius and ambiguus cause dysphagia accompanied by decreased laryngeal sensation and that the lesions are relatively extensive and affect the middle level of the dorsal medulla.

[Middle mediastinal schwannoma originating from the intrathoracic vagal nerve with difficulty in swallowing; report of a case].

A 41-year-old male complaining of difficulty in swallowing was referred to our hospital. Chest computed tomography( CT) demonstrated 34×25×36 mm tumors in the subcarinal region. Gadolinium( Gd)-diethylenetriamine pentaacetic acid( DTPA) enhanced magnetic resonance imaging (MRI) showed the tumor with the target appearance sign, i.e., signal intensity of the mass was low on T1-weighted MRI, and the center of the mass was enhanced by Gd-DTPA. A neurogenic tumor was suspected on radiological findings. Resection of the tumor by video-assisted thoracoscopic surgery was performed. The tumor was found to originate from the left vagus nerve by operative findings and was diagnosed as schwannoma by pathological examination.

A case of spontaneous pneumothorax presenting with high-degree atrioventricular block.

The myocardial electrical conduction is significantly influenced by the sympathetic and parasympathetic nervous systems. Hypervagotonia refers to increased vagal tone leading to sinus bradycardia, sinus pause, or any degree of atrioventricular (AV) block. There are many clinical, electrocardiographic, and electrophysiologic clues that help make the diagnosis of hypervagotonia as the cause of bradyarrhythmias. We describe a case of spontaneous pneumothorax presenting with symptomatic high-degree AV block and subsequent electrophysiologic diagnosis of hypervagotonia. The patient had complete resolution of his bradycardia and AV block after chest tube placement. This case is intended to alert cardiologists to this rare presentation of spontaneous pneumothorax.

Vagal paraganglioma: imaging versus intraoperative findings.

A 59-year-old woman presented with a mass on the jugulo-digastric level. Inspection of the oral cavity showed medial bulging of the left pharyngeal wall and tonsil. Transcutaneous ultrasonography showed a vascular tumor of the parapharyngeal space with complete encirclement of the internal carotid artery. Magnetic resonance angiography and common carotid angiography showed the bloody support from the external carotid artery of that tumor. During the surgical procedure, we identified the tumor, who arises from the vagus nerve and who had multiple fine arterial branches from the internal carotid artery until the skull base. We performed complete resection of the parapharyngeal tumor, by transcervical approach, with sacrificed the vagus nerve. The histopathological report showed a vagal paraganglioma. After one year is no sign of recurrence.

Medialization thyroplasty with a customized silicone implant: clinical experience.

The authors implemented medialization thyroplasty with a customized silicone implant in a total of 43 operations (36 patients) in 1999-2003. In 5 of these patients, the medialization thyroplasty was combined with cricothyroid subluxation (3 cases) or adduction of arytenoid cartilage (3 cases). One patient received medialization thyroplasty, cricothyroid subluxation and adduction of arytenoid cartilage. Postoperatively 36 patients reported substantial reduction of their complaints, 5 patients found their voice improved and only 2 patients (5.6%) stated that their voice had not changed. The subjective evaluation was consistent with the findings of laryngoscopy and the preoperative and postoperative phonation parameters (maximum phonation time, maximum sound pressure level, jitter and shimmer). Average maximum phonation time was 6.5 s before surgery and 12.5 s after surgery. Maximum vocal sound pressure level was, on average, about 4 dB higher after surgery. Jitter was reduced from 5.3 to 3.7% and shimmer from 32.3 to 18.6%. The differences between presurgical and postsurgical parameters in our study were all statistically significant, indicating voice improvement. Medialization thyroplasty with a silicone implant was proven to be a successful and safe surgical method for the treatment of vocal fold paralysis.

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.

Maternal vagal tone change in response to methadone is associated with neonatal abstinence syndrome severity in exposed neonates.

OBJECTIVE: Though methadone pharmacotherapy is the treatment of choice for opiate-dependence during pregnancy in the USA, most methadone-exposed neonates develop neonatal abstinence syndrome (NAS). NAS expression is widely variable among methadone-exposed neonates and only a subset requires pharmacotherapy. This study explores the potential predictors of NAS severity, including aspects of maternal substance use and methadone maintenance histories, concomitant exposure to other licit substances, and individual differences in intrinsic maternal or infant factors that may affect the infant's vulnerability to NAS expression. METHODS: Fifty methadone-maintained pregnant women attending a comprehensive substance abuse treatment facility, received electrocardiogram monitoring at 36 weeks of gestation at the times of trough and peak maternal methadone levels. Vagal tone, an estimate of the magnitude of an individual's respiratory sinus arrhythmia and an indicator of autonomic control, was derived. RESULTS: NAS expression was unrelated to maternal substance abuse history, methadone maintenance history, or psychotropic medication exposure. Male infants displayed more profound NAS symptoms and received more pharmacotherapy to treat NAS (all p < 0.05). NAS expression was related to maternal vagal reactivity; both suppression and activation of maternal vagal tone in response to methadone administration were positively and significantly associated with NAS symptomatology (F (2,44) = 4.15, p < 0.05) and treatment (F (2,44) = 3.39, p < 0.05). Infants of vagal non-responder mothers showed substantially lower NAS expression. CONCLUSIONS: NAS severity is associated with maternal vagal tone change in response to methadone administration.

A carotid artery dissection presenting with dysphagia due to a dilation of upper oesophagus.

Carotid and vertebral artery dissection is one of the major causes of ischemic stroke in young patients and diagnosing it remains a challenge for the clinician due to its variable clinical presentation. We report the case of a 57-years old man admitted to the emergency department complaining of dysphagia and a hoarse voice. Physical examination revealed a left deviation of his tong and some weakness of the left side of his soft palate. A gastroscopy and a cervical tomodensitometry revealed the presence of an important dilation of his upper oesophagus. A cerebral magnetic resonance was performed, showing en excentric high signal intensity on the left carotid artery, and the MR angiography confirmed the presence of an acute dissection on the left internal carotid artery. Our patient suffered of a left carotid artery dissection presenting with ipsilateral Xth and XIIth nerve palsies. His main symptom was a dysphagia due to a dilation of his upper oesophagus; the pneumogastric nerve being responsible for the parasympathic innervation of the upper muscles of the oesophagus.

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.

Adenoviral gene transfer of BDNF and GDNF synergistically prevent motoneuron loss in the nucleus ambiguus.

We have previously shown that neuroprotective effects of an adenoviral glial cell line-derived neurotrophic factor (GDNF) gene transfer on the lesioned adult rat motoneurons in the nucleus ambiguus. In the present study, we examined neuroprotective effects of adenoviral gene transfer of brain-derived neurotrophic factor (BDNF) or/and GDNF to motoneurons in nucleus ambiguus using an adult rat vagal nerve avulsion model. The animals avulsed and inoculated with adenoviral vectors encoding BDNF (AxCAmBDNFME) or/and GDNF (AxCAhGDNF) showed immunolabeling for BDNF or/and GDNF in the nucleus ambiguus on the treated side, respectively, and expression of virus-induced BDNF or/and GDNF mRNA transcripts in the brainstem tissue that contained the nucleus ambiguus of the treated side. The treatment with AxCAhGDNF or AxCAmBDNFME significantly prevented the loss of vagal motoneurons in comparison to the control; the protective effect of AxCAmBDNFME was greater than that of AxCAhGDNF. The combined treatment with AxCAmBDNFME and AxCAhGDNF acted synergistically and significantly larger number of vagal motoneurons was preserved as compared to either AxCAmBDNFME treatment or AxCAhGDNF treatment. The treatment with AxCAmBDNFME or/and AxCAhGDNF after avulsion also suppressed the activity of nitric oxide synthase in lesioned motoneurons in the nucleus ambiguus. These results indicate that adenovirus-mediated BDNF and GDNF gene transfer may prevent the degeneration of motoneurons in humans after either vagal nerve injury or recurrent laryngeal nerve injury.

Effectiveness of amitriptyline versus cough suppressants in the treatment of chronic cough resulting from postviral vagal neuropathy.

OBJECTIVE: The objective of this prospective, randomized, controlled study (N = 28) was to evaluate the effectiveness of amitriptyline versus cough suppressants in the treatment of chronic cough resulting from postviral vagal neuropathy. METHODS: Patients were selected based on a clinical history consistent with postviral vagal neuropathy and a history of an antecedent upper respiratory tract infection. All patients had been tried on antireflux medication (proton pump inhibitors) and had a negative chest x-ray before presentation. All were nonsmokers without a history of asthma. Patients on angiotensin-converting enzyme inhibitors were excluded from the study. All patients completed a pretreatment, validated cough-specific quality-of-life (QOL) survey. Patients were randomized by chart numbers to either 10 mg amitriptyline at bedtime or 10 to 100 mg/5 mL, 10 mL codeine/guaifenesin every 6 hours standing dose while awake. Both groups were instructed to complete 10 days of therapy and then asked to subjectively rate the reduction in the frequency and severity of their cough by 100%, 75%, 50%, 25%, or 0% as well as completing the posttreatment cough QOL questionnaire. Those patients experiencing a 75% to 100% reduction were recorded as having a complete response, 25% to 50% a partial response, and 0% as having no response. Final results and the cough QOL survey were recorded and used for statistical analysis. RESULTS: A majority of patients in the amitriptyline group achieved a complete response on the initial dose of 10 mg. None of the codeine/guaifenesin group achieved a complete response. The data were analyzed using a logistic regression model, and amitriptyline was found to be a highly significant predictor of a greater than 50% response when compared with codeine/guaifenesin (P = .0007). The same data were analyzed using a proportional odds model and similar results were noted. CONCLUSIONS: Chronic cough can have a profound impact on the psychosocial function of patients. The most common causes of a persisting cough in the absence of infection or chronic smoking are laryngopharyngeal reflux, asthma, particularly the cough variant, allergy, rhinosinusitis, bronchitis, and medications, in particular angiotensin-converting enzyme inhibitors. Currently, there are few effective treatments for cough with an acceptable therapeutic ratio and more selective drugs with a more favorable side effect profile are needed. This is this first prospective, randomized, controlled study comparing the effectiveness of amitriptyline versus codeine/guaifenesin for select cases of chronic cough resulting from suspected postviral vagal neuropathy.