Delayed vagal nerve compressive neuropathy following placement of vagal nerve stimulator: case report.

Vagal neuropathy causing vocal fold palsy is an uncommon complication of vagal nerve stimulator (VNS) placement. It may be associated with intraoperative nerve injury or with device stimulation. Here we present the first case of delayed, compressive vagal neuropathy associated with VNS coil placement which presented with progressive hoarseness and vocal cord paralysis. Coil removal and vagal neurolysis was performed to relieve the compression. Larger 3 mm VNS coils were placed for continuation of therapy. Coils with a larger inner diameter should be employed where possible to prevent this complication. The frequency of VNS-associated vagal nerve compression may warrant further investigation.

Technique Nuances for Functional Preservation of Lower Cranial Nerves during Surgical Management of Ventral Foramen Magnum Meningiomas Via a Dorsal Lateral Approach.

BACKGROUND: This study established novel technique nuances in surgery for ventral foramen magnum meningiomas (vFMMs) via a dorsal lateral approach. METHODS: From July 2012 to July 2019, 37 patients with vFMMs underwent tumor resection surgery and were operated on with a dorsal lateral approach. Two safe zones were selected as the entrance of the surgical corridor. Safe zone I was located between the dural attachment of the first dental ligament (FDL) and the branches of C1; safe zone II lay between the dural attachment of the FDL and the jugular foramen. The tumor was debulked first through safe zone I and then through safe zone II. The tumor was removed through a trajectory from the caudal to cephalad to allow tumor debulking from below and downward delivery, away from the brainstem and lower cranial nerves. RESULTS: Thirty-three patients underwent gross total resection, and 4 patients underwent subtotal resection. Four patients transiently required a nasogastric feeding tube. All patients recovered within 3 months postoperatively. Three patients (8.1%) developed permanent mild hoarseness and dysphagia as a result of postoperative damage of cranial nerves IX and X. One patient underwent tracheotomy. No patient experienced tumor recurrence during the follow-up period. CONCLUSIONS: We established a minimal retraction principle, in which the selection of 2 safe zones as the entrance of the surgical corridor, tumor removal from the inferior to superior direction, and debulking followed by devascularization were the key elements to implement the minimal retraction principle in vFMM surgery.

Cranial Nerve IX and X Weakness: An Unusual Initial Presentation of Myasthenia Gravis.

BACKGROUND Myasthenia gravis (MG) is an autoimmune disease characterized by antibodies binding skeletal muscle acetylcholine receptors (AChR). Rarely does the disease manifest with orolaryngeal symptoms before ocular ones. We present a case of MG that on initial presentation had symptoms of cranial nerves (CN) IX and X weakness, including dysphagia and dysphonia. CASE REPORT A 51-year-old woman with panic attacks presented to the Emergency Department (ED) with complaints of her throat closing, swallowing difficulty, and hoarse voice. Multiple ED visits revealed no etiology. However, she developed stridor, which prompted further evaluation. Laryngoscopy and imaging studies revealed no gross abnormalities; therefore, her symptoms of dysphonia and difficulty breathing were attributed to anxiety. Her hospital course was complicated by a cardiac arrest requiring intubation. ECHO, CTA chest, and MRI brain were unremarkable. Her cardiac arrest was hypothesized as being secondary to laryngeal spasm. During her ICU course, she failed extubation multiple times due to acute respiratory failure. An autoimmune etiology was suspected, prompting a paraneoplastic screen, which revealed elevated levels of AChR antibodies at 124 mmol/L. MG was diagnosed and treatment with plasmapheresis and steroids was initiated. However, complications of thrombocytopenia, anemia, and ARDS ensued, so MG treatment was discontinued. The patient was eventually transferred to a LTACH. Thereafter, at outpatient followup, her MG was treated with mycophenolate and prednisone, which led to significant symptom improvement. CONCLUSIONS MG commonly presents in the third decade with clinical features of ptosis, diplopia, and facial weakness. However, initial and isolated symptoms of dysphagia and dysphonia are rare, leading to missed diagnoses. Our case of a middle-aged woman posed a diagnostic challenge because of her uncommon presentation and comorbidities of panic attacks and obesity. This case highlights the crucial need for a high index of clinical suspicion for MG in any patient presenting with symptoms of CN IX and X weakness.

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.

Vocal cord paralysis due to ectopic parathyroid adenoma and function recovery: a case report and review of the literature.

Ectopic parathyroid adenomas (PAs) can occur in numerous locations and are thought to be the cause of a significant portion of failed primary surgery for hyperparathyroidism. PA is a rare cause of hoarseness, which may be harbingers of a malignant process. Here, we describe an unusual case of an ectopic PA in the carotid sheath presenting as unilateral vocal cord paralysis (VCP). A 49-year-old lady presented with a 1-week history of hoarseness, irritating cough and shortness of breath. Fibreoptic laryngoscopy revealed left VCP. Ultrasound and computed tomography of the neck demonstrated a mass in the carotid sheath. Laboratory investigations revealed hypercalcemia (3.10 mmol/L), hypophosphatemia (0.81 mmol/L) and elevated intact parathyroid hormone (iPTH) level (381.6 pg/mL), despite of a negative 99mTc-sestamibi scan. After more rigorous tests, the ectopic tumor adjacent to the left vagus nerve was successfully resected, with subsequent histopathological confirmation of PA. The patient eventually got a normal iPTH level and serum calcium postoperatively, and regular voice function was also regained 4 months after surgery. This case emphasizes the importance of broad differential diagnosis and thorough workup. Although most patients with PA present with hypercalcemia, this disease entity also need to be considered in the differentials of neck masses and VCP.

Magnetic Resonance Imaging Features of Collet-Sicard Syndrome Associated With Glomus Jugulare Paraganglioma.

Collet-Sicard syndrome is an unusual disorder. The authors here demonstrated the Magnetic resonance (MR) imaging findings of the Collet-Sicard syndrome associated with glomus jugulare tumor. Neoplastic or non-neoplastic lesion of skull base can cause Collet-Sicard syndrome. MR imaging can be used successfully to demonstrate the etiology of this syndrome.

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.

A 68-year-old with cranial nerve neuropathies and a troponin rise.

In this case study, we summarise the inpatient investigations and management of a 68-year-old woman with Takotsubo cardiomyopathy secondary to a Varicella zoster encephalitis and the difficulties inherent with making this diagnosis. She presented with evolving cranial nerve neuropathies, which started with a vagal nerve mononeuritis and eventually included left-sided sensorineural hearing loss and a facial nerve palsy. These symptoms were concomitant with a variety of cardiac abnormalities, including fast atrial fibrillation and electrocardiographic changes. We summarise some of the current understanding of Takotsubo cardiomyopathy and the criteria for its diagnosis. Although left ventricular apical ballooning has been described in association with severe infections and states of high stress, we have not seen it reported in association with a Varicella zoster encephalitis.

Tapia's syndrome: pathogenetic mechanisms, diagnostic management, and proper treatment: a case series.

BACKGROUND: Tapia's syndrome is an uncommon disease described in 1904 by Antonio Garcia Tapia, a Spanish otolaryngologist. It is characterized by concomitant paralysis of the hypoglossal (XIIth) and pneumogastric (Xth) nerves. Only 69 cases have been described in the literature. Typically, the reported patients presented with a history of orotracheal intubation. Common symptoms are dysphonia, tongue deviation toward the affected side, lingual motility disturbance, and swallowing difficulty. CASE PRESENTATION: In the report, we describe three cases of Tapia's syndrome in three Caucasian patients who underwent surgery with general anesthesia. Two of these patients underwent neck abscess drainage, and the third had an open reduction of a shoulder fracture. The clinical symptoms of Tapia's syndrome appeared after extubation. All three of our patients recovered their lost function at 3 months after diagnosis. CONCLUSIONS: We underline the importance of performing airway endoscopy and a specific program of swallowing rehabilitation for the proper management of Tapia's syndrome.

One-day high-fat diet induces inflammation in the nodose ganglion and hypothalamus of mice.

A high-fat diet (HFD) induces inflammation in systemic organs including the hypothalamus, resulting in obesity and diabetes. The vagus nerve connects the visceral organs and central nervous system, and the gastric-derived orexigenic peptide ghrelin transmits its starvation signals to the hypothalamus via the vagal afferent nerve. Here we investigated the inflammatory response in vagal afferent neurons and the hypothalamus in mice following one day of HFD feeding. This treatment increased the number of macrophages/microglia in the nodose ganglion and hypothalamus. Furthermore, one-day HFD induced expression of Toll-like receptor 4 in the goblet cells of the colon and upregulated mRNA expressions of the proinflammatory biomarkers Emr1, Iba1, Il6, and Tnfα in the nodose ganglion and hypothalamus. Both subcutaneous administration of ghrelin and celiac vagotomy reduced HFD-induced inflammation in these tissues. HFD intake triggered inflammatory responses in the gut, nodose ganglion, and subsequently in the hypothalamus within 24 h. These findings suggest that the vagal afferent nerve may transfer gut-derived inflammatory signals to the hypothalamus via the nodose ganglion, and that ghrelin may protect against HFD-induced inflammation.

Impaired vagus-mediated immunosuppression in microsomal prostaglandin E synthase-1 deficient mice.

The cholinergic anti-inflammatory pathway controls innate immune responses and inflammation. The prostaglandin (PG) system is involved in several neuro-processes and associated with inflammatory activation of cells in vagal nuclei. Here we aimed to investigate the potential role of PG in cholinergic neuro-regulation. The effect of vagus nerve stimulation (VNS) has been evaluated in microsomal prostaglandin E synthase-1 (mPGES-1) knockout (-/-) and wild-type (+/+) mice regarding cytokine and PG levels after lipopolysaccharides (LPS) challenge. As expected, VNS decreased the release of pro-inflammatory cytokines both in serum and spleen extracts of mPGES-1 (+/+)animals. However, the immune suppressive effect of VNS was completely abolished in mPGES-1 (-/-) mice. The PG content was not affected by VNS in the spleen of mPGES-1 (+/+) and mPGES-1 (-/-) mice but interestingly, acetylcholine (ACh) release in spleen induced by VNS confirmed an intact cholinergic pathway in mPGES-1 (+/+) whereas no VNS-induced ACh release was found in mPGES-1 (-/-) animals. Our data show that mPGES-1 and consequently PGE2 are crucial in the cholinergic anti-inflammatory pathway. Moreover, the mechanisms involved do not affect PG content in the spleen, but lack of mPGES-1 was found to strongly affect cholinergic mechanisms in the inflamed spleen. These findings illustrate previously unrecognized associations between the cholinergic and prostaglandin systems, and may be of importance for further development of therapeutic strategies directed at modulation of the inflammatory reflex, and immunosuppression in chronic inflammatory diseases.

Cervical osteophyte resulting in compression of the jugular foramen: Case report.

Jugular foramen syndrome is a condition characterized by unilateral paresis of cranial nerves IX, X, and XI in the setting of extrinsic compression. Here, the authors describe the case of a giant cervical osteophyte resulting in compression of the jugular foramen. A 74-year-old man who presented with progressive dysphagia and dysarthria was found to have right-sided tongue deviation, left palatal droop, and hypophonia. His dysphagia had progressed to the point that he had lost 25 kg over a 4-month period, necessitating a gastrostomy to maintain adequate nutrition. He underwent extensive workup for his dysphagia with several normal radiographic studies. Ultimately, CT scanning and postcontrast MRI revealed a posterior osteophyte arising from the C1-2 joint space and projecting into the right jugular foramen. This resulted in a jugular foramen syndrome in addition to delayed filling of the patient's right internal jugular vein distal to the osteophyte. Although rare, a posterior cervical osteophyte should be considered in cases of jugular foramen syndrome.

Nasal CPAP and preterm bradycardia: cause or cure.

Nasal continuous positive airway pressure (nCPAP) is widely used for the treatment of respiratory distress syndrome and apnoea of prematurity. Complications related to fixation devices have been well documented. We report a clinically well preterm baby suffering intermittent, profound episodes of bradycardia without any prior associated apnoea or desaturation. We believe these episodes were due to the oculocardiac reflex related to orbital compression from the continuous positive airway pressure (CPAP) fixation straps. Bradycardia was replicated by gentle ocular compression and the episodes resolved after repositioning the CPAP straps. Vagal overstimulation has previously been reported in preterm babies but we believe this to be the first case in which pressure from CPAP strapping has been reported to trigger bradycardia. However, we suspect that similar cases could easily go unrecognised. Careful positioning of CPAP securing straps may prevent accidental vagal overstimulation contributing to episodic bradycardia.

Isolated vagus nerve paralysis associated with internal carotid artery dissection.

Dysphagia and hoarseness caused by laryngopharyngeal paralysis associated with internal carotid artery (ICA) dissection is rare. We reported a case which recovered spontaneously. A 57-year old man visited our hospital complaining of dysphagia and hoarseness lasting for two weeks. Paralysis of right vocal fold and rotational movement of the posterior pharyngeal wall toward the left side during swallowing were observed. Magnetic resonance imaging was performed under diagnosis of isolated right vagus nerve paralysis, and dissection of the right ICA was revealed. He was treated conservatively, and both of laryngopharyngeal movement and the ICA dissection were improved completely. There is a possibility that laryngeal paralysis caused by ICA dissection has been misdiagnosed as an idiopathic paralysis.

Carotid artery pseudoaneurysm after orthognathic surgery causing lower cranial nerve palsies: endovascular repair.

PURPOSE: Reported complications following Le Fort osteotomies are rare but can include epistaxis from disruptions or pseudo-aneurysms of the maxillary artery or its distal branches the descending palatine and sphenopalatine arteries, aseptic necrosis of the maxilla, ophthalmic injuries including blindness, ophthalmoplegia, and keratitis sicca, and arteriovenous fistulas or false aneurysms of the carotid arteries (external and/or internal). The mechanism of injury to neurovascular structures can be the result of direct or indirect trauma, such as injuries from surgical instruments, traction injuries during manipulation of the osteotomized bone segments or during inadvertent manipulations of the head and neck, or from fractures extending to the base of the skull, orbit, or pterygopalatine fossa associated with the pterygomandibular dysjunction or maxillary downfracture. CASE REPORT: An 18 year-old male with facial bone dysplasia, apertognathia, maxillary hypoplasia and mandibular hyperplasia was treated with maxillary Le Fort I osteotomy with internal fixation and elastic intermaxillary fixation. Following surgery, the patient developed palsies of the vagus and accessory nerves manifesting as dysphagia, cough, vocal cord paralysis and trapezius muscle atrophy. Cross sectional imaging revealed a small, laterally pointing pseudoaneurysm of the high cervical internal carotid artery (ICA) at the skull base, exerting pulsatile mass effect on adjacent lower cranial nerves. The patient was treated with carotid artery stent reconstruction and pseudoaneurysm coil obliteration, and kept on dual antiplatelet therapy for two months. Partial recovery from cranial nerve palsies was observed within a year. CONCLUSION: A small, broad-based, laterally-pointing ICA pseudoaneurysm at the exit of the carotid canal without surrounding hematoma was clearly demonstrated on CTA, which visualization was difficult on MRA due to considerable metallic artifact from surgical hardware. Angiography exquisitely demonstrated the pseudoaneurysm, which was fully repaired with the combination of stenting and coil obliteration, allowing total preservation of the ICA.

Persistent cough associated with osteophyte formation and vagus nerve impingement following cervical spinal surgery: case report.

Persistent cough due to irritation of the vagus nerve by osteophytes resulting from cervical spinal surgery is a very rare condition. The authors report the case of a 49-year-old woman who presented with a persistent cough subsequent to cervical spinal surgery. One year after the initial operation, the patient underwent surgery to free the larynx from the prevertebral fascia and cut the pharyngeal plexus, but her symptoms persisted. In order to control the cough, she used a soft cervical collar with padding inserted in the left side so that the larynx would be pushed to the right, a solution she discovered on her own. Without the collar, she coughed uncontrollably. A CT scan was performed and showed an osteophyte that had developed at the level of the prosthesis. Based on these findings, the authors hypothesized that the cough was caused by vagus nerve irritation due to the osteophyte. The osteophyte was resected and the vagus nerve was moved to a position anterior to the carotid artery and was isolated by means of an autogenous tensor fascia lata graft. The patient's symptom disappeared immediately after the surgery. At the most recent follow-up visit, 18 months after surgery, the patient was symptom free and was pursuing regular daily activities without using a cervical collar.

Cranial nerve IX and X impairment after a sports-related Jefferson (C1) fracture in a 16-year-old male: a case report.

BACKGROUND: Jefferson (C1) fractures are rare cervical spine injuries that usually do not result in cranial nerve (CN) impairment. However, case reports of Collet-Sicard syndrome (impairment of CNs IX-XII) and impairment of CNs IX, X, and XII have been reported. All reported cases involved adult patients in high-impact collisions, such as motor vehicle accidents or falls. To our knowledge, a Jefferson fracture with selective CN impairment due to a low-energy, sports-related injury in a pediatric patient has not been reported. METHODS: Chart and radiographic data for a single case were reviewed and reported in a retrospective study approved by the Institutional Review Board of the participating hospital. RESULTS: A 16-year-old male was diagnosed with a Jefferson fracture after a head-to-chest football collision. On computed tomography, the distance between the atlas transverse process and styloid process of the skull was 5 mm right and 10 mm left. Before halo fixation, the patient had vague complaints of dysphagia. These complaints worsened which led to the diagnosis of CN IX and X impairment and placement of a feeding tube. The fracture healed uneventfully, the dysphagia symptoms resolved, and the halo fixation and feeding tube were removed. The patient returned to all activities, but was instructed to avoid participation in contact sports. CONCLUSIONS: This was the first report of selective CN impairment in a pediatric patient with a Jefferson fracture resulting from a low-impact sports-related injury. Careful monitoring of the patient complaints led to appropriate treatment. Further studies into the spatial relationship between the transverse process of the atlas in relation to the styloid process of the skull may be warranted. LEVEL OF EVIDENCE: Level V, case report.

[A case of Churg-Strauss syndrome presenting with lower cranial neuropathy].

We reported a 60 year-old man with Churg-Strauss syndrome (CSS). Three months later, he presented with dysarthria, dysphagia and severe headache. We detected glossopharyngeal and vagal nerve palsy, and made a diagnosis of cranial nerve involvement comorbid with CSS. Intravenous administration of methypredonisolone was effective for alleviating clinical signs and symptoms. Two months later, he complained of headache and facial numbness, but symptoms improved with an escalating dose of prednisolon. As compared to previously reported cases, our case was characteristic because of involvement of lower cranial nerve with CSS, which has been reported previously in only one case.