Computed tomographic features of canine intracranial and jugular foraminal masses involving the combined glossopharyngeal, vagus, and accessory nerve roots.

A chronic cough, gag, or retch is a common presenting clinical complaint in dogs. Those refractory to conservative management frequently undergo further diagnostic tests to investigate the cause, including CT examination of their head, neck, and thorax for detailed morphological assessment of their respiratory and upper gastrointestinal tract. This case series describes five patients with CT characteristics consistent with an intracranial and jugular foraminal mass of the combined glossopharyngeal (IX), vagus (X), and accessory (XI) cranial nerves and secondary features consistent with their paresis. The consistent primary CT characteristics included an intracranial, extra-axial, cerebellomedullary angle, and jugular foraminal soft tissue attenuating, strongly enhancing mass (5/5). Secondary characteristics included smooth widening of the bony jugular foramen (5/5), mild hyperostosis of the petrous temporal bone (3/5), isolated severe atrophy of the ipsilateral sternocephalic, cleidocephalic, and trapezius muscles (5/5), atrophy of the ipsilateral thyroarytenoideus and cricoarytenoideus muscles of the vocal fold (5/5), and an ipsilateral "dropped" shoulder (4/5). Positional variation of the patient in CT under general anesthesia made the "dropped" shoulder of equivocal significance. The reported clinical signs and secondary CT features reflect a unilateral paresis of the combined cranial nerves (IX, X, and XI) and are consistent with jugular foramen syndrome/Vernet's syndrome reported in humans. The authors believe this condition is likely chronically underdiagnosed without CT examination, and this case series should enable earlier CT diagnosis in future cases.

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 Nerve Palsy After Transarterial Embolization of Transverse-Sigmoid Dural Arteriovenous Fistula Using Onyx.

OBJECTIVE: We report a case of a 70-year-old man who developed a transverse-sigmoid dural arteriovenous fistula (TS-DAVF) that was successfully treated by transarterial embolization (TAE) with Onyx. CASE PRESENTATION: The patient presented with sudden and progressive disturbance of consciousness and left hemiparesis. Magnetic resonance imaging (MRI) revealed venous infarction and hemorrhagic changes with brain swelling in the right parietal lobe. Angiography revealed a right TS-DAVF and multiple occlusions with retrograde leptomeningeal venous drainage into the cortical veins. The TS-DAVF was graded as Borden type III and Cognard type IIa+b. Because of its progressive clinical nature and wide distribution of DAVF in the occluded sinus wall, he underwent emergent TAE with liquid embolic materials including n-butyl cyanoacrylate and Onyx under informed consent by his family. Complete obliteration of the TS-DAVF was achieved, leading to a marked amelioration of symptoms, and MRI after treatment confirmed a decrease in the brain swelling. However, he suffered transient dysphagia due to right vagal nerve palsy caused by occlusion of vasa nervorum of ascending pharyngeal artery. He returned home 5 months later with a modified Rankin Scale of 1. CONCLUSIONS: TAE with Onyx appears to be effective for aggressive TS-DAVF with a widely distributed shunt. However, the blood supply to the cranial nerves and potentially dangerous anastomoses between the external-internal carotid artery and vertebral artery should be taken into account to avoid serious complications.

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.

Management of vagal paragangliomas: review of 17 patients.

Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this retrospective study is to review the clinical and genetic findings, surgical treatment, and complications of vagal paragangliomas, as well as to discuss the management options. During the period 1990-2013, 17 patients with vagal paragangliomas were referred to our institution. There were ten patients with isolated tumors, and seven with multicentric paragangliomas. There were nine women and eight men. Mean age of patients was 51.4 years. Five cases had a positive family history of paraganglioma (29.4 %). Germline mutations of SDH genes were found in six of our patients (35.3 %). Many options were considered in the management of vagal paragangliomas. Surgical treatment was performed in 11 young patients (64.7 %) using different approaches: in 4 patients the tumor was resected through a transcervical approach; in 3 through a transcervical-transmandibular approach; in 1 it was resected using a transcervical-transmastoid approach, and in 3 a type A infratemporal fossa approach was performed. In all operated cases, the removal of the tumor led to sacrificing of the vagus nerve. Postoperative hypoglossal nerve deficit was reported in 4 cases (36.3 %). In six elderly patients (35.3 %), we decided to "wait-and-scan" in order to avoid creating greater morbidity than that of the tumor itself. Many factors should be considered in the treatment of vagal paragangliomas: the age and general condition of the patient, the biological behavior of the tumor, tumor size, genetic results, bilaterality, multicentricity, lower cranial nerve function, and of course the potential morbidity of the surgical treatment itself. Rehabilitation and, possibly surgery, are necessary to treat postoperative lower cranial nerve deficits.

Treatment of cervical paragangliomas: is surgery the only way?

PURPOSE: To analyze the results after surgery or stereotactic radiotherapy (SRT) in the treatment of cervical paragangliomas. Against this background, the decision-making algorithm used in the treatment of carotid body tumors (CBTs) and vagal paragangliomas (VPs) was reevaluated relative to the existing literature on the topic. MATERIALS AND METHODS: Retrospective study between 2000 and 2012. A total of 27 CBTs and nine VPs in 32 patients were treated. Shamblin class I: 59.3% (n=16); class II: 29.6% (n=8); class III: 11.1% (n=3). Treatment modalities were surgery, radiotherapy, or observation. The end points for analysis were long-term tumor control and integrity of the cranial nerves. RESULTS: 21 CBTs and seven VPs underwent surgery; SRT was performed in three CBTs and two VPs. Three CBTs were clinically observed. Permanent nerve paresis followed after surgery for CBTs in five patients (20%) and in all patients with VPs. No impaired cranial nerve function resulted after SRT. The median follow-up period was 4.7 years. The tumor control rate after therapy for CBTs and VPs was 100%. One CBT that received clinical observation showed slow tumor progression. CONCLUSIONS: A surgical procedure should be regarded as the treatment of choice in patients with small CBTs. In larger CBTs, particularly in elderly patients with unimpaired cranial nerves, radical surgery should be regarded critically. As surgery for VPs caused regularly impairment of cranial nerves with functional disturbances of various degrees a comprehensive consultation with the patient is mandatory and nonsurgical strategies should be discussed.

[Synchronous schwannoma of the vagus nerve and the cervical sympathetic chain]. / Synchrones Schwannom des N. vagus und des zervikalen sympathischen Grenzstrangs.

Schwannomas are rare neural sheath tumors which are generally benign. Up to 45% of all schwannomas originate in the head and neck region. In the parapharyngeal space (PPS) they may arise from any of the lower cranial nerves IX, X, XI and XII or from the cervical sympathetic chain. We report a unique case of a synchronous schwannoma of the vagal nerve and the cervical sympathetic chain in a patient without neurofibromatosis.

[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 report of giant vagal schwannoma in the right neck and literature review].

Vagal nerve-derived schwannomas are rare, especially those with huge tumors. This paper reports a case of giant tumor in the right neck. The patient was misdiagnosed as pleomorphic adenoma before operation, and was diagnosed as schwannoma during operation. The clinical manifestations, diagnosis, differential diagnosis, treatment and prognosis are discussed in this paper based on the history and related literature.

Vocal cord paralysis after percutaneous embolization of a vagal paraganglioma--the role of intraoperative nerve monitoring.

The value of preoperative embolization is well established in the management of paragangliomas. Percutaneous direct intratumoral embolization has been described as a safe and effective technique to achieve devascularization of these tumors, thereby reducing intraoperative blood loss. However, the present article presents a case in which a patient developed vocal cord palsy after percutaneous embolization of a vagal paraganglioma. In view of the present findings, the possibility of intraoperative nerve monitoring needs to be explored to ensure safety of this procedure.

Vagus nerve schwannoma in the middle mediastinum.

Intrathoracic neurogenic tumors are generally located in the posterior mediastinum. They usually arise from an intercostal nerve or a sympathetic chain. Tumors originating from the vagus nerve in the middle mediastinum are extremely rare. This report describes a patient with a huge intrathoracic schwannoma of the vagus nerve in the middle mediastinum and reviews the literature.

Neurosarcoidosis affecting the vagus nerve.

OBJECTIVES: Our objective was to review patients who presented to our medical center with a diagnosis of neurosarcoidosis affecting the vagus nerve and to present symptoms, progression, treatments, and outcome. METHODS: We performed a chart review of patients who presented to our medical center in the past 10 years with a diagnosis of neurosarcoidosis specifically affecting cranial nerve X. RESULTS: A chart review of 53 patients revealed only 4 with findings suggestive of vagal neurosarcoidosis. All were male and had a mean age of 50 years (range, 42 to 57 years) at presentation of symptoms. Two of the 4 patients presented initially with cough, 1 had recurrent syncope, and another presented with left facial pain. Vagus nerve involvement included vocal fold paresis or paralysis in all 4 patients, 2 of whom reported coughing with exposure to various odors and 2 of whom were found to have a unilateral palatal weakness. All but 1 had positive findings on magnetic resonance imaging of the head. CONCLUSIONS: Neurosarcoidosis involving the vagus nerve is a rare finding, but should be considered in the differential diagnosis of vocal fold paresis or paralysis.

Utility of the plasma pancreatic polypeptide response to modified sham feeding in diabetic gastroenteropathy and non-ulcer dyspepsia.

BACKGROUND: The relationship between cardiovascular and gastrointestinal (ie, plasma pancreatic polypeptide [PP] response to modified sham feeding [MSF]) indices of vagal function is unclear. Hyperglycemia inhibits PP secretion via vagally mediated mechanisms. Our aims were to (a) compare the PP response, (b) its relationship with glycemia, and (c) the relationship between PP response to MSF, gastric emptying (GE) of solids, and symptoms during GE study in healthy controls, patients with diabetic gastroenteropathy (DM), and non-ulcer dyspepsia (NUD). METHODS: In 24 healthy controls, 40 DM, and 40 NUD patients, we measured plasma PP concentrations during MSF, cardiovagal functions, GE, and symptoms during a GE study. KEY RESULTS: Baseline PP concentrations were higher in DM than in controls and NUD (P = .01), and in type 2 than in type 1 DM patients (P < .01). The PP increment during MSF was normal (≥20 pg/mL) in 70% of controls, 54% of DM, and 47% of NUD patients. Overall, the PP response and cardiovagal tests were concordant (P = .01). Among patients with a reduced PP increment with MSF, 7/10 of T1DM and 1/7 of T2DM patients had moderate or severe cardiovagal dysfunctions (P < .05). The PP response to MSF was not associated with GE. CONCLUSIONS & INFERENCES: Up to 30% of healthy controls have a reduced PP increment during MSF, limiting the utility of this test to detect vagal injury. The PP response is more useful when it is normal than abnormal. A reduced PP response is more likely to be associated with cardiovagal dysfunctions in T1DM than in T2DM.

Multiple paragangliomas: a case report.

BACKGROUND: Carotid and vagal paragangliomas (CPGLs and VPGLs) are rare neoplasms that arise from the paraganglia located at the bifurcation of carotid arteries and vagal trunk, respectively. Both tumors can occur jointly as multiple paragangliomas accounting for approximately 10 to 20% of all head and neck paragangliomas. However, molecular and genetic mechanisms underlying the pathogenesis of multiple paragangliomas remain elusive. CASE PRESENTATION: We report a case of multiple paragangliomas in a patient, manifesting as bilateral CPGL and unilateral VPGL. Tumors were revealed via computed tomography and ultrasound study and were resected in two subsequent surgeries. Both CPGLs and VPGL were subjected to immunostaining for succinate dehydrogenase (SDH) subunits and exome analysis. A likely pathogenic germline variant in the SDHD gene was indicated, while likely pathogenic somatic variants differed among the tumors. CONCLUSIONS: The identified germline variant in the SDHD gene seems to be a driver in the development of multiple paragangliomas. However, different spectra of somatic variants identified in each tumor indicate individual molecular mechanisms underlying their pathogenesis.

Selective vagal denervation of the sinus and atrioventricular nodes, guided by vagal reflexes induced by high frequency stimulation, to treat refractory neurally mediated syncope.

A 15-year-old female patient presented with frequent episodes of vasovagal syncope refractory to non-pharmacological and pharmacological measures. Two tilt-table tests performed before and after conventional therapy were positive and reproduced the patient's clinical symptoms. Selective vagal denervation, guided by HFS, was performed. Six radiofrequency pulses were applied on the left and right sides of the interatrial septum, abolishing vagal responses at these locations. Basal sinus node and Wenckebach cycle lengths changed significantly following ablation. A tilt test performed after denervation was negative and revealed autonomic tone modification. The patient reported significant improvement in quality of life and remained asymptomatic for 9 months after denervation. After this period, three episodes of NMS occurred during a 4-month interval and a tilt test performed 11 months after the procedure demonstrated vagal activity recovery.

Prediction of early secondary complications in patients with spontaneous subarachnoid hemorrhage based on accelerated sympathovagal ratios.

PURPOSE: The development of secondary complications following spontaneous subarachnoid hemorrhage (SAH) largely depends on sympathetic overexcitation. The roles of vagal activities, however, are poorly defined. Because both components of the autonomic nervous system can be explored in the frequency domain of heart rate variability (HRV), the present study aimed to determine the dynamic evolution of autonomic activities and to identify patients at high risk for complications following hemorrhage. METHODS: Thirty patients with SAH were enrolled in our study. Those who suffered from symptomatic vasospasm, cerebral infarction, neurogenic pulmonary edema, or early mortality within 1 week of ictus were categorized into the complication group. Spectral analysis of HRV explored three important indices of sympathetic and vagal modulations: low-frequency (LF), high-frequency (HF), and LF/HF ratios. Patterns of HRV dynamics within the first 3 days were compared between complication and non-complication groups. The group trends, estimated by the slopes of HRV changes, were determined for further univariate and multivariate analysis. RESULTS: Our study showed that daily HRV in the complication group exhibited an approximately 2.7-fold increase of sympathovagal ratio (denoted by LF/HF). This resulted from reciprocal changes of sympathoexcitation (LF) and vagal withdrawal (HF). Multivariate analysis revealed that LF/HF slope, an indicator of the trend of sympathovagal change, was an independent variable significantly associated with the development of complications. CONCLUSIONS: This study confirmed that during early SAH period, patients with and without complications presented different patterns of sympathovagal changes. LF/HF slope during the first 3 days was a significant predictor of secondary complications after SAH.

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.