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.

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.

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.

Jugular and vagal paragangliomas: Systematic study of management with surgery and radiotherapy.

BACKGROUND: 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. METHODS: Relevant articles were reviewed using strict criteria for systematic searches. Forty-one surgical studies met the criteria which included 1310 patients. Twenty articles including 461 patients treated with EBRT, and 14 radiosurgery studies comprising 261 patients were also evaluated. Results were compared between treatment modalities using analysis of variance (ANOVA) tests. RESULTS: A total of 1084 patients with JPGs and 226 VPGs were treated with different surgical procedures. Long-term control of the disease was achieved in 78.2% and 93.3% of patients, respectively. A total of 715 patients with JPG were treated with radiotherapy: 461 with EBRT and 254 with SRS. Control of the disease with both methods was obtained in 89.1% and 93.7% of the patients, respectively. The treatment outcomes of a JPG treated with surgery or radiotherapy were compared. Tumor control failure, major complication rates, and the number of cranial nerve palsies after treatment were significantly higher in surgical than in radiotherapy series. The results of SRS and EBRT in JPGs were compared and no significant differences were observed in tumor control. Because only 1 article reported on the treatment of 10 VPGs with radiotherapy, no comparisons with surgery could be made. Nevertheless, the vagus nerve was functionally preserved in only 11 of 254 surgically treated patients (4.3%). CONCLUSION: There is evidence that EBRT and SRS offer a similar chance of tumor control with lower risks of morbidity compared with surgery in patients with JPGs. Although the evidence is based on retrospective studies, these results suggest that surgery should be considered only for selected cases, but the decision should be individual for every patient.

[Schwannoma of the vagus nerve: diagnostic strategy and therapeutic approach]. / Schwannome médiastinal bénin du nerf vague: stratégies diagnostique et thérapeutique.

INTRODUCTION: Scwhannomas are uncommon neurogenic tumors of the mediastinum. Most of them are located in the posterior mediastinum usually in the paralateral gutters. We report the case of an uncommon localisation of such a tumour appended to the right vagus nerve in the middle mediastinum. CASE REPORT: The patient was 50 years old and complained of thoracic pain, shortness of breath and dysphagia. CT scanning of thorax and abdomen revealed a heterogeneous mass in the middle mediastinum, which was not visible on plain radiography. Further investigation included transoesophageal ultrasound and PET scan. The diagnosis was confirmed by histopathology after mini video-assisted thoracotomy. The schwannoma was entirely removed without any post-surgical complications. CONCLUSION: We report a novel case of schwannoma arising from the vagus nerve and review the diagnostic strategies. PET scanning has poor sensitivity and specificity and does not therefore contribute to confirm the diagnosis which depends on exploring the mediastimun surgically.

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.

Malignant vagal paraganglioma: report of a case treated with embolization and surgery.

Approximately 20 cases of malignant vagal paragangliomas (MVP)have been reported in English literature. Malignancy is based on the presence of metastases. A careful preoperative evaluation is necessary to detect multicentricity and/or significant production of catecholamines. A new case of MVP treated with embolization and surgery is presented and the literature discussed. It is concluded, that preoperative embolization followed by radical surgical resection is a rational treatment of patients with unilateral MVP.

[Cranial nerve vascular compression syndromes and neurogenic hypertension]. / Syndromy vaskulární komprese mozkových nervu a neurogenní hypertenze.

The presented paper is a review of syndromes which till recently had an obscure cause and still have attributes such as "primary" or "essential". Their common denominator is vascular compression of the roots of the appropriate cephalic nerves. The syndromes include trigeminal neuralgia, glossopharyngeal neuralgia, hemifacial spasm, some forms of tinnitus and vertigo, exceptionally toricollis. Vascular compression of the ventrolateral oblongata on the left leads to neurogenic hypertension. The author discusses the state of knowledge on the aetiology of syndromes, the clinical and laboratory research of which contributed some findings on the physiology and pathophysiology of the mentioned conditions. At the same time the authors present their own experience with the therapeutic method of syndromes--microvascular decompression.

Four faces of baroreflex failure: hypertensive crisis, volatile hypertension, orthostatic tachycardia, and malignant vagotonia.

BACKGROUND: The baroreflex normally serves to buffer blood pressure against excessive rise or fall. Baroreflex failure occurs when afferent baroreceptive nerves or their central connections become impaired. In baroreflex failure, there is loss of buffering ability, and wide excursions of pressure and heart rate occur. Such excursions may derive from endogenous factors such as stress or drowsiness, which result in quite high and quite low pressures, respectively. They may also derive from exogenous factors such as drugs or environmental influences. METHODS AND RESULTS: Impairment of the baroreflex may produce an unusually broad spectrum of clinical presentations; with acute baroreflex failure, a hypertensive crisis is the most common presentation. Over succeeding days to weeks, or in the absence of an acute event, volatile hypertension with periods of hypotension occurs and may continue for many years, usually with some attenuation of pressor surges and greater prominence of depressor valleys during long-term follow-up. With incomplete loss of baroreflex afferents, a mild syndrome of orthostatic tachycardia or orthostatic intolerance may appear. Finally, if the baroreflex failure occurs without concomitant destruction of the parasympathetic efferent vagal fibers, a resting state may lead to malignant vagotonia with severe bradycardia and hypotension and episodes of sinus arrest. CONCLUSIONS: Although baroreflex failure is not the most common cause of the above conditions, correct differentiation from other cardiovascular disorders is important, because therapy of baroreflex failure requires specific strategies, which may lead to successful control.

A case of trigeminal-vagal neuralgia relieved by peripheral self-stimulation.

A case of simultaneous trigeminal and vagoglossopharyngeal neuralgia is described. No microvascular compression was seen at the trigeminal complex while the origin of vagoglossopharyngeal pain could have been due either to Chiari malformation or microvascular compression of the IX-X nerve complex. Decompressive surgery was ineffective. The patient could completely block his facial painful fits by strongly pinching the anterior axillary fold. This case militates against peripheral theories of facial neuralgias, including microvascular compression and ganglion ignition focus theories, and supports a central origin thereof.

Paroxysmal vagal overactivity, apparent life-threatening event and sudden infant death.

The possibility of reversible cardiac asystoly due to paroxysmal vagal overactivity (VO) has been well studied, first in adults, then in children, and finally in breath-holding spells. Few studies deal with infants and the incidence of VO among preterm, apparent life-threatening event (ALTE) and sudden infant death syndrome (SIDS). In this review, we summarize data acquired during the past 20 years leading to the diagnosis of VO in infants. We describe the clinical aspects of VO in infants and young children such as Holter-ECG and oculocardiac reflex criteria. The familial aspect of VO and its possible link with SIDS (a new risk factor?) are discussed. Finally, whether or not to treat infants with symptomatic VO is discussed.