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.

[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.

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.

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.

Intraoperative mapping and monitoring of sensory vagal fibers during vagal schwannoma resection.

Vagal schwannomas are rare, benign tumors. Intermittent intraoperative neuromonitoring via selective stimulation of splayed motor fibers running on the schwannoma surface to elicit a compound muscle action potential has been previously reported as a method of preserving vagal motor fibers. In this case report, vagal sensory fibers are mapped and continuously monitored intraoperatively during high vagus schwannoma resection using the laryngeal adductor reflex (LAR). Mapping of nerve fibers on the schwannoma surface enabled identification of sensory fibers. Continuous LAR monitoring during schwannoma subcapsular microsurgical dissection enabled sensory (and motor) vagal fibers to be monitored in real time with excellent postoperative functional outcomes. Laryngoscope, 129:E434-E436, 2019.

Unilateral vagus nerve lesion revealing clival meningocele.

INTRODUCTION: Cranial nerve lesions can be secondary to a space-occupying lesion of the skull base compressing adjacent nerves. CASE REPORT: We report the case of an 84-year-old man, who presented with rapid and concomitant onset of dysphagia and ipsilateral recurrent laryngeal nerve paralysis, suggesting an isolated lesion of the vagus nerve. MRI revealed a diagnosis of previously unknown clival meningocele. DISCUSSION: Unilateral vagus nerve paralysis constitutes an exceptional mode of presentation of meningocele. Only a few isolated cases of clival meningocele have been reported to date, with no cranial nerve repercussions. The symptomatic management adopted in this case allowed rapid improvement of the patient's disorders.

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.

Diagnosis and management of pediatric cervical vagal schwannoma.

Schwannomas arising from the vagus nerve are extremely rare in children, with only 15 cases reported in the world literature. We describe a pediatric case of cervical vagal nerve schwannoma successfully treated with cranial nerve-sparing surgery. Our patient presented extensive mass in the right side of the neck with ipsilateral Horner's syndrome. Her first sign, anisocoria, was diagnosed at the age of 1.5 y, making her the youngest vagal schwannoma case ever reported. Using an ultrasonic surgical aspirator and nerve monitoring, a multidisciplinary team successfully removed the mass with no recurrence after 2 years of follow-up.

Vagus nerve schwannoma in the right upper mediastinum.

A 35-year-old woman was admitted to our hospital with an abnormal shadow on her chest roentgenogram. Computed tomography showed that a tumor was located in the right upper mediastinum. Resection of the tumor by video-assisted thoracoscopic surgery was performed. Operative findings determined that the tumor originated from the right vagus nerve and was diagnosed as schwannoma by pathological examination.

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.

[Synchronous unilateral vagal paraganglioma and carotid body tumor:one case report].

One case of synchronous unilateral vagal paraganglioma and carotid body tumor was reported. The preoperative medical imaging revealed no obvious abnormalities at the carotid bifurcation. In addition to the bigger vagal paragaglioma，a smaller carotid body tumor was discovered during operation.

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.

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.

Endocrine tumors associated with the vagus nerve.

The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.