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OBJECTIVE: The objective of this study was to evaluate the clinical effect and safety of the postauricular infratemporal fossa approach (ITFA) in resecting jugular foramen lesions. METHODS: All 25 patients undergoing microsurgery via postauricular ITFA from March 2015 to May 2023 in the Department of Neurosurgery, Tangdu Hospital, Air Force Military Medical University were included. The clinical and radiological data were retrospectively analyzed. Regular follow-up was carried out. RESULTS: The mean age of all patients was 50.5±8.9 years, and 14 of them were female and 11 were male. Among the cases, lower cranial nerve schwannoma accounted for 60 % (15/25) of all tumors, jugular foramen paraganglioma accounted for 20 % (5/25), and the remaining 20 % included meningioma, chondrosarcoma, plasmacytoma, and salivary gland tumors. Total tumor resection was performed in 18 cases, subtotal tumor resection in 7 cases and partial resection in 1 case. Seven patients underwent gamma knife radiotherapy after surgery. Transient lower cranial nerve dysfunction occurred in 8 patients, and permanent lower cranial nerve dysfunction occurred in 2 patients after surgery. One patient developed facial paralysis, and one patient presented hearing loss. CONCLUSIONS: The postauricular ITFA achieved a relatively high total tumor resection rate and a lower incidence of neurological functional disorders. It is an alternative and suitable surgical approach for resecting jugular foramen lesions. Maximizing the preservation of neurological function is preferred, especially when radical resection cannot be achieved. Stereotactic radiotherapy could be used for residual tumors.

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BACKGROUND AND PURPOSE: Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX-XII palsy, known as Collet-Sicard syndrome (CSS). High-resolution magnetic resonance imaging (HR-MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus. METHODS: We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection. RESULTS: We describe a 68-year-old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right-sided palsy of cranial nerves IX-XII. Three-dimensional fat-saturated black-blood T1-weighted high-resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy. CONCLUSIONS: This case highlights the utility of high-resolution vessel wall imaging and especially fat-saturated T1-weighted black-blood SPACE (sampling perfection with application-optimized contrast using different flip-angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR-MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression.

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Background: Extracranial internal carotid artery (ICA)-dissecting aneurysms (DAs) rarely cause re-entry tears and lower cranial nerve palsies. The therapeutic strategies for these pathologies are not well established. This report presents a case of an extracranial ICA -DA with a re-entry tear that caused lower cranial nerve palsy. Case Description: A 60-year-old man presented with left neck pain, hoarseness, and dysphagia. Physical examination and laryngoscopy determined palsies of the left cranial nerves IX, X, and XII. Digital subtraction angiography (DSA) revealed a DA in the left extracranial ICA, and three-dimensional DSA showed entry and re-entry tears in the intimal flap. Flow-diverting stents (FDSs) were placed on the lesion that covered the entry and re-entry tears because the symptoms did not improve after five weeks of conservative treatment. A post-procedural angiogram indicated flow stagnation in the DA. Symptoms improved remarkably immediately after the procedure, and the aneurysm was almost completely occluded six months later. Conclusion: Herein, an extracranial ICA -DA with a re-entry tear that caused lower cranial nerve palsy did not improve after five weeks of conservative treatment. FDS placement promptly resolved the aneurysm and symptoms. Thus, FDS placement may be an effective treatment option for extracranial ICA-DAs with re-entry tears or lower cranial nerve palsies.

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Introduction Guillain-Barré syndrome (GBS) is an autoimmune disease affecting radicles and peripheral nerves resulting in acute flaccid paralysis. Respiratory failure, autonomic dysfunction, and secondary complications such as pneumonia, and venous thromboembolism are the major causes of death and disability in GBS. Cardiovascular complications play a major role in the prognosis of GBS patients. The aim is to determine the incidence of cardiovascular instability in GBS patients and to see if there are any specific risk groups associated with the development of cardiovascular instability. Methodology This is a retrospective descriptive study conducted in a tertiary care center in South India. Data on 50 consecutive GBS patients were collected from hospital records including case sheets, death summaries, and discharge summaries. Patients with evidence of sepsis, blood loss, heavy alcohol consumption, and chronic liver disease were excluded from the study. Baseline demographic data, symptom onset to admission time, baseline Erasmus Guillain-Barré Syndrome Respiratory Insufficiency Score (EGRIS), and baseline liver function tests were documented. The presence of heart rate and blood pressure fluctuations was noted from the records. Frequency data were calculated from the categorical variables. Analysis of non-parametric variables by chi-square test was done using IBM SPSS Statistics for Windows, Version 25.0 (Released 2017; IBM Corp., Armonk, New York, United States). Results Cardiovascular instability was present in 15 (30%) patients in the study population. It was present in all patients (100%) who require mechanical ventilation. The incidence of cardiovascular instability was higher in patients who had lesser onset to admission times (41.9% vs 10.5%; p=0.019), EGRIS≥4 (40.6% vs 11.1%; p=0.029), and lower cranial nerve involvement (40% vs 6.7%; p=0.018). Conclusion Of patients with GBS, 30% developed cardiovascular instability during their disease course. Patients with lesser onset to admission times, EGRIS ≥4, and those with lower cranial nerve involvement had a greater incidence of cardiovascular instability.

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A 7-year-old castrated male American Shorthair cat presented with left-side Horner's syndrome and voice change. The overall clinical presentation included dysphagia, intermittent coughing, unilateral miosis, and third eyelid protrusion of the left eye. A topical 1% phenylephrine was applied, and miosis and protrusion of the third eyelid disappeared within 20 min which suggested a post-ganglionic lesion. Laryngoscopy showed left-sided laryngeal paralysis. Computed tomography (CT) identified a mass lesion invading outside of the left tympanic bulla with osteolysis. Endoscopically assisted ventral bulla osteotomy was performed for tumor resection and definitive diagnosis. Middle ear adenocarcinoma was diagnosed based on histopathology. It appears that these neurological signs occurred due to adenocarcinoma in the tympanic bulla, penetrating the jugular foramen and the hypoglossal canal and damaging the cranial nerve IX (glossopharyngeal nerve), X (vagus nerve), XI (accessory nerve), and XII (hypoglossal nerve) and the sympathetic nerve. To the best of our knowledge, this is the first case report of Villaret's syndrome associated with middle ear adenocarcinoma affecting the nerves passing through the jugular foramen and hypoglossal canal in cats.

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The signs of lower cranial nerve palsies are rare and are often caused by tumors. A 49-year-old woman was admitted to our hospital with progressive right-sided atrophy of the tongue, sternocleidomastoid and trapezius, dysarthria, and dysphagia for 3 years. Brain magnetic resonance imaging revealed a circular lesion adjacent to the lower cranial nerves. Cerebral angiography confirmed that the lesion was an unruptured aneurysm in the C1 segment of the right internal carotid artery. After endovascular treatment, the symptoms of this patient had partially improved.

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The author describes a rare case of giant adenoid cystic carcinoma (ACC) mimicking large paraganglioma with lower cranial nerve palsy. A 60-year-old female presented with a progressive increase in postauricular swelling with unilateral hearing loss, facial deviation, difficulty in swallowing, and hoarseness of voice. MRI brain showed highly vascular infiltrating and osteolytic mass suggestive of large glomus jugulare versus sarcoma. It was completely engulfing the jugular foramen and lower cranial nerves with bony erosion of the jugular foramen and occipital condyle. The whole mastoid was filled with the tumor. On digital subtraction angiography the majority of blood supply was from the occipital branch of the external carotid artery and vertebral artery. The patient underwent percutaneous embolization followed by external carotid ligation and resection of the mass. The postoperative course was uneventful. Histopathology was suggestive of mixed ACCs. The patient received radiotherapy. After 1 year of follow up no recurrence or distant metastasis was noted.

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BACKGROUND: Tumors involving the jugular foramen region are challenging for surgical resection. With the development of endoscope in the past decade, surgical approaches assisted by endoscope have been widely emerged in the treatment of skull base tumors. METHODS: Herein, we report a case of jugular foramen schwannoma (Samii type B). Surgical resection was applied via a suboccipital retrosigmoidal craniotomy using surgical microscope assisted by endoscope. Gross total resection was achieved. And the patient recovered without obvious neurological deficits. CONCLUSIONS: Samii type B schwannomas involving the jugular foramen is approachable by endoscope-assisted surgery.

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Inflammatory pseudotumor (IPT) is a non-neoplastic condition of unknown etiology. IPT with lower cranial nerve (CN IX, X, XI, XII) neuropathies is extremely rare. In this study, we systematically reviewed all previously reported cases regarding the management of IPT with lower cranial nerve neuropathies. We searched the PubMed/MEDLINE database for reports related to IPT with lower cranial nerve neuropathies. A total of six papers with 10 cases met the inclusion criteria (mean age 51.6 years, 70% male). The mean follow-up period was 15.4 months (range: 1-60 months). The most frequent treatment was corticosteroids alone (60%), followed by surgery alone (20%), and multimodal treatment (20%). Corticosteroid therapy was associated with an excellent (100%) response rate at 6 months of follow-up, and half of the patients were in complete remission after 9 months. Both cases who received surgery had persistent neurological deficits. Immediate complete remission of neurological symptoms and resolution on imaging after decompression surgery via an endoscopic endonasal approach (EEA) with corticosteroids was demonstrated in our representative case. This review suggests that EEA is a preferred method for diagnosis and treatment, a promising approach associated with favorable outcomes, and a viable first-line treatment for selected cases, followed by multimodal therapy.

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Background: Some studies reported cases of internal carotid artery (ICA) dissection (ICAD) that was treated by carotid artery stenting (CAS). Symptoms of ICAD resulting from the lower cranial nerve palsy are rare and the treatment strategy is not clearly defined. We report a patient with ICAD showing hypoglossal nerve palsy alone that was treated by CAS. Case Description: A 47-year-old man presented with headache, dysphagia, dysarthria, and tongue deviation to the left. He had no history of trauma nor any other significant medical history. Axial T2-CUBE MRI and MRA showed dissection of the left ICA accompanied with a false lumen. These findings indicated that direct compression by the false lumen was the cause of hypoglossal nerve palsy. Although medical treatment was continued, symptoms were not improved. Therefore, CAS was performed to thrombose the false lumen and decompress the hypoglossal nerve. His symptoms gradually improved after CAS and angiography performed at month 6 showed well-dilated ICA and disappearance of false lumen. Conclusion: CAS may be an effective treatment for the lower cranial nerve palsy caused by compression by a false lumen of ICAD.

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BACKGROUND: Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear, and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations. METHODS: A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the third of a three-part series describing non-vestibular schwannomas (IX, X, XI, XII). RESULTS: A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to preoperative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies and follow-up management. CONCLUSION: This article represents the consensually derived opinion of the task force with respect to the treatment of non-vestibular schwannomas. For each of these tumours, the management paradigm is shifting towards the compromise between function preservation and progression free survival.

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INTRODUCTION: A number of neurological complications of COVID-19 have been identified, including cranial nerve paralyses. We present a series of 10 patients with lower cranial nerve involvement after severe COVID-19 infection requiring hospitalization in an intensive care unit. METHODS: We conducted a retrospective, observational study of patients admitted to the post-intensive care unit (p-ICU) of Besançon University Hospital (France) between March 16 and May 22, 2020. We included patients with confirmed COVID-19 and cranial neuropathy at admission to the p-ICU. All these patients were treated by orotracheal intubation, and all but one underwent prone-position ventilation therapy. RESULTS: Of the 88 patients admitted to the p-ICU, 10 patients (11%) presented at least 1 cranial nerve palsy. Of these 10 patients, 9 had a hypoglossal nerve palsy and 8 of these also had a deficit in another cranial nerve. The most frequent association was between hypoglossal and vagal palsies (5 patients). None of the patients developed neurological signs related to a global neuropathy. We found no correlation between the intensity of the motor limb weakness and the occurrence of lower cranial nerve palsies. All but 2 of the patients recovered within less than a month. CONCLUSION: The mechanical compressive hypothesis, linked to the prone-position ventilation therapy, appears to be the major factor. The direct toxicity of SARS-CoV-2 and the context of immune dysfunction induced by the virus may be involved in a multifactorial etiology.

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Patient Palatal lift prostheses (PLPs) are used for dysarthria caused by velopharyngeal incompetence (VPI) and improving hypernasal speech. In this case, we used a PLP with a flexible lift (f-PLP) in a patient with dysphagia associated with VPI due to right-sided cranial nerve injuries after a skull base surgery. We examined its efficacy in swallowing biomechanics and swallowing function using high-resolution manometry (HRM) and videofluoroscopic examination of swallowing (VF). The patient felt that it was easier to swallow with f-PLP. Furthermore, VF indicated that the pharyngeal residue with f-PLP was less than without it. HRM showed that velopharyngeal pressure and intrabolus pressure (IBP) with f-PLP were higher than those without it. Additionally, the upper esophageal sphincter (UES) relaxation time and UES nadir pressure on the patient's healthy left side compared to the right side improved with f-PLP.Discussion We discovered two clinical outcomes. First, the f-PLP ensured velopharyngeal closure and an increase in the hypopharyngeal IBP, which potentially improved the UES opening on the healthy side. Second, the f-PLP improved pharyngeal clearance, and the patient felt that it was easier to swallow with the f-PLP. This implies that an f-PLP potentially exhibits a positive effect on swallowing.Conclusions In this case, the f-PLP contributed to improving the pharyngeal passage of a bolus. We suggest that f-PLPs can be used for patients with dysarthria and those with dysphagia with VPI.

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OBJECTIVE: Surgery for tumors around the jugular foramen has significant risks of dysphagia and vocal cord palsy due to possible damage to the lower cranial nerve functions. For its treatment, long-term tumor control by maximum resection while avoiding permanent neurological damage is required. To accomplish this challenging goal, the authors developed an intraoperative continuous vagus nerve monitoring system and herein report their experience with this novel neuromonitoring method. METHODS: Fifty consecutive patients with tumors around the jugular foramen (34 jugular foramen schwannomas, 11 meningiomas, 3 hypoglossal schwannomas, and 2 others) who underwent microsurgical resection under continuous vagus nerve monitoring within an 11-year period were retrospectively investigated. Evoked vagus nerve electromyograms were continuously monitored by direct 1-Hz stimulation to the nerve throughout the microsurgical procedure. RESULTS: The average resection rate was 96.2%, and no additional surgery was required in any of the patients during the follow-up period (average 65.0 months). Extubation immediately after surgery and oral feeding within 10 days postoperatively were each achieved in 49 patients (98.0%). In 7 patients (14.0%), dysphagia and/or hoarseness were mildly worsened postoperatively at the latest follow-up, but tracheostomy or gastrostomy was not required in any of them. Amplitude preservation ratios on intraoperative vagus nerve electromyograms were significantly smaller in patients with postoperative worsening of dysphagia and/or hoarseness (cutoff value 63%, sensitivity 86%, specificity 79%). CONCLUSIONS: Intraoperative continuous vagus nerve monitoring enables real-time and quantitative assessment of vagus nerve function and is important for avoiding permanent vagus nerve palsy, while helping to achieve sufficient resection of tumors around the jugular foramen.

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BACKGROUND: The findings of a hyperintense sign on T2-weighted imaging (T2-WI) and gadolinium (Gd) contrast enhancement on magnetic resonance imaging (MRI) of the brain stem suggest malignant glioma. However, this pathological condition is probably uncommon, and it may be unknown that a dural arteriovenous fistula (DAVF) can imitate this radiological pattern. In addition, it is extremely rare to be caused by a spinal DAVF. Here, a rare case of spinal DAVF that mimicked malignant glioma of the medulla oblongata is presented. CASE DESCRIPTION: A 56-year-old woman was admitted with a progressive gait disturbance, vertigo, and dysphasia. MRI showed a hyperintense signal in the medulla oblongata on fluid-attenuated inversion recovery (FLAIR) and moderate contrast enhancement on Gd-enhanced MRI. Interestingly, Gd-enhanced MRI demonstrated abnormal dilated veins around the brain stem and cervical spinal cord. Cerebral angiography showed spinal DAVF at the left C4/C5 vertebral foramen fed by the C5 radicular artery. The fistula drained into spinal perimedullary veins and flowed out retrograde at the cortical vein of the posterior cranial fossa. Therefore, surgical disconnection of the spinal DAVF was performed by a posterior approach. The patient's postoperative course was uneventful. Cerebral angiography showed complete disappearance of the DAVF, with marked reductions of the hyperintense sign of the medulla oblongata on FLAIR. CONCLUSION: This important case illustrates MRI findings mimicking brain stem glioma. In cases with the hyperintense sign-on T2-WI associated with contrast enhancement suspicious of brainstem glioma, careful checking for perimedullary abnormal vessels and additional cerebral angiography should be performed.

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OBJECTIVE: We describe the dural relationships and its surgical implications for large lower cranial nerve (CN) neurinomas. The study is based on surgical experience with 14 cases. METHODS: During the period January 2014 to December 2019, 14 consecutive cases with large lower CN neurinomas were surgically treated with the aim of radical tumor resection. RESULTS: There were 9 males and 5 females, ranging in age from 17 to 65 years. All patients were operated in a single stage. The principal surgical observation was that the entire extent of tumor, which included intracranial, jugular fossa, and extracranial components, was within the "dural" confines and the tumor adjoining critical neural and vascular structures was displaced around the dome of the tumor. Radical surgical resection was achieved in all cases. During the follow-up period that ranged from 3 to 71 months (average 32 months), no symptomatic recurrence was observed and no patient needed reoperation. At the time of last clinical follow-up, the lower CN function in all patients was better than at the time of presentation. CONCLUSIONS: The "dural" cover of the lower CN neurinomas forms a strong and reliable plane of compartmentalization and allows safe tumor resection.

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Cerebellopontine (CP) angle tumors are often resected via retrosigmoid craniotomy; however, sometimes cranial nerves (CNs) make their resection more complex. In such cases, the endoscopic transnasal approach can avoid such manipulations as delivering surgical instruments over CNs or peeling off CNs from the tumor, minimizing the risk of postoperative deficits. A 35-year-old man presented with a 37-mm cystic tumor in the right CP angle, and preoperative 3D fusion images revealed that multiple CNs (VII, VIII, and lower CNs) were running on the tumor posteriorly. The endoscopic transnasal approach enabled safe subtotal resection without causing neurological deficits, and the patient underwent stereotactic radiosurgery for the residual schwannoma. The video can be found here: https://youtu.be/xKLwdDsLpWA.

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Objectives Despite being pathologically benign, jugular foramen meningioma (JFM) may be locally aggressive and spread in three compartments. Because of the complex anatomical location, radical removal of JFM usually causes serious morbidity through lower cranial nerve (LCN) deficits. To accomplish long-standing tumor control with good functional outcomes, we report function-preserving multimodal treatment (FMT) for JFM, comprising the combination of intradural tumor removal with the preservation of LCN function and stereotactic radiosurgery (RS) for the residual tumor. Materials This study investigated six JFM patients (five women, one man). Preoperatively, five patients showed no LCN sign. Results All patients underwent function-preserving retrosigmoid intradural tumor removal, and no patient developed new LCN deficit. Three patients underwent RS for the residual tumor at 8 to 12 months after surgery. After RS, all three tumors were controlled. No patients showed tumor recurrence or new LCN deficits in the follow-up period (2 months to 8 years). Conclusion FMT for JFMs can accomplish long-standing tumor control with excellent functional outcomes.

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Objective To evaluate the incidence, prognosis and influencing factors of lower cranial nerve palsy after microvascular decompression (MVD) in patients with hemifacial spasm (HFS). Methods Clinical data of 1033 patients with HFS, admitted to our hospital from May 2014 to August 2016, were retrospectively analyzed. The incidence of lower cranial nerve palsy after procedure and prognosis of these patients were summarized; and influencing factors were analyzed by statistical method. Results In 1033 patients with HFS, 10 patients (0.97％) had lower cranial nerve palsy after procedure: 6 patients suffered hoarseness, 2 patients suffered dysphasia, and 2 patients were with both symptoms; the symptoms in 6 patients completely recovered within one month, 2 patients within one-3 months, and 2 patients within 3-6 months. No permanent low cranial nerve palsy occurred. There were no statistical significances in age, gender, clinical course, sides, or the types of offending vessels between the lower cranial nerve palsy patients (n=10) and the non-palsy patients (n=1023)(P>0.05). Conclusion Lower cranial nerve palsy is a rare complication after MVD, and type of responsible vessels is not a influencing factor in this complication

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Objective Gamma Knife stereotactic radiosurgery (GK-SRS) is a preferred treatment option for tumors of the jugular foramen. We hypothesized that GK-SRS toxicity is higher for lower cranial nerve schwannomas than for glomus jugulare tumors despite anatomically similar locations. Methods We performed a retrospective review of all patients who received GK-SRS for glomus jugulare tumors and lower cranial nerve schwannomas at our institution between 2006 and 2014. Because of small sample sizes, Fisher's exact tests and logistic regression techniques were employed using SPSS. Result We identified 20 glomus jugulare tumors and 6 lower cranial nerve schwannoma patients with a median follow-up of 17 months. Median marginal dose was 16 Gy (range 13-18 Gy) and 12.5 Gy (range 12-14 Gy), respectively. All except one patient had tumor control at last follow-up visit. No worsening of pre-existing neurological deficits was observed. There were seven patients who developed any new neurological deficit after GK-SRS, four from the glomus group, and three from the schwannoma group (20 and 50% of each group, respectively). Only two of seven patients had permanent new neurological deficits. Both of them were in the schwannoma group. Univariate analysis showed that only a diagnosis of schwannoma had a greater risk of permanent new cranial nerve complication after GK-SRS compared with diagnosis of glomus jugulare ( p = 0.046). Conclusion Although the marginal dose for glomus jugulare is greater, our study suggests that the risk of a new permanent neurological deficit after GK-SRS was higher in the schwannoma group compared with the glomus group.