Neuralgia del trigémino y del glosofaríngeo concomitantes, asíncronas y refractarias. Buena respuesta al abordaje quirúrgico en un tiempo / Concomitant, asynchronous and refractory trigeminal and glossopharyngeal neuralgia. Good response to surgical approach in one time

Introducción: La cirugía de descompresión microvascular (DMV) es el tratamiento de elección de una neuralgia craneal refractaria secundaria a compresión vascular. Las neuralgias simultáneas de dos pares craneales son extremadamente infrecuentes. Describimos un caso con neuralgia concomitante del trigémino (NT) y glosofaríngeo (NG) refractarias y secundarias a cruce neurovascular abordadas quirúrgicamente en un mismo tiempo. Caso clínico: Mujer de 65 años con NT derecho (inicialmente en V2-V3) desde 2004 con control regular con carboxamidas. Diecisiete años después empeoraron los paroxismos en V2-V3, aparecieron también en V1 y en el territorio del nervio glosofaríngeo derecho (oído derecho y fosa amigdalar al hablar y deglutir). La resonancia magnética cerebral mostró contacto arterial significativo entre arteria cerebelosa superior (ACS) con origen del V par craneal derecho y de la arteria cerebelosa antero-inferior (AICA) con el origen de pares craneales bajos derechos. Se realizó DMV de ambos pares craneales en un mismo tiempo quirúrgico mediante craniectomía retrosigmoidea, y se procedió a la liberación del V par craneal, en contacto íntimo con la ACS, y del IX par craneal en contacto con la AICA derecha, para lo que se puso teflón entre ellas. La paciente tuvo una resolución inmediata de los paroxismos trigeminales y una mejoría considerable en intensidad y frecuencia de los glosofaríngeos. A los 2 años de la intervención continúa la desescalada de tratamiento neuromodulador con buena respuesta.ConclusiónLa DMV en la NT y NG simultáneas es factible y puede ofrecer un buen resultado posquirúrgico. (AU)

INTRODUCTION: Microvascular decompression (MVD) surgery is the first choice treatment for refractory cranial neuralgia secondary to vascular compression. Simultaneous neuralgia of two cranial nerves is extremely rare. We describe a case of concomitant refractory trigeminal (TN) and glossopharyngeal (GN) neuralgia secondary to neurovascular crossover, treated surgically at the same time. CASE REPORT: 65-year-old woman with right TN (initially V2-V3) since 2004 with regular control with carboxamides. Seventeen years later, paroxysms worsened in V2-V3, also appearing in V1 and in the territory of the right glossopharyngeal nerve (right ear and tonsillar fossa when speaking and swallowing). Cerebral MRI showed significant arterial contact between the superior cerebellar artery (SCA) with the origin of the right V cranial nerve and the antero-inferior cerebellar artery (AICA) with the origin of the right lower CCNN. MVD of both cranial nerves was performed at the same surgical time by means of retrosigmoid craniectomy, releasing the V cranial nerve, in intimate contact with the SCA, and the IX cranial nerve in contact with the right AICA, interposing teflon between them. The patient had an immediate resolution of the trigeminal paroxysms and a dramatic improvement in intensity and frequency of glossopharyngeal paroxysms. Two years after the intervention, de-escalation of neuromodulator treatment continues with good response. CONCLUSION. MVD in simultaneous TN and GN is feasible and can offer a good post-surgical outcome. (AU)

Glossopharyngeal Neuralgia Characterized by Otalgia: A Retrospective Study.

Glossopharyngeal neuralgia (GPN) is an uncommon facial pain syndrome and is characterized by paroxysms of excruciating pain in the distributions of the auricular and pharyngeal branches of cranial nerves IX and X. Glossopharyngeal neuralgia characterized by otalgia alone is rare. Herein, the authors analyzed 2 patients with GPN with otalgia as the main clinical manifestation. The clinical features and prognosis of this rare group of patients with GPN were discussed. They both presented with paroxysmal pain in the external auditory meatus and preoperative magnetic resonance imaging suggested the vertebral artery were closely related to the glossopharyngeal nerves. In both patients, compression of the glossopharyngeal nerve was confirmed during microvascular decompression, and the symptoms were relieved immediately after surgery. At 11 to 15 months follow-up, there was no recurrence of pain. A variety of reasons can cause otalgia. The possibility of GPN is a clinical concern in patients with otalgia as the main complaint. The authors think the involvement of the glossopharyngeal nerve fibers in the tympanic plexus via Jacobson nerve may provide an important anatomic basis for GPN with predominant otalgia. Surface anesthesia test of the pharynx and preoperative magnetic resonance imaging is helpful for diagnosis. Microvascular decompression is effective in the treatment of GPN with predominant otalgia.

Malformación de Chiari tipo i descubierta a través de una neuralgia glosofaríngea / Chiari type i malformation discovered through a glossopharyngeal neuralgia

La neuralgia glosofaríngea es una enfermedad rara cuyo tratamiento inicial es farmacológico. Cuando la terapia médica no es eficaz, se encuentran disponibles diferentes opciones quirúrgicas, incluida la radiocirugía estereotáctica, la descompresión microvascular o la sección de nervios. Se presenta el caso de una mujer de 26 años con neuralgia glosofaríngea y malformación de Chiari. Este tipo raro de neuralgia a veces se asocia con una anomalía de la unión cráneo-cervical. Se realizó una expansión de fosa posterior con duraplastia y descompresión microvascular. La paciente mostró una completa desaparición del dolor, sin necesidad de llevar a cabo la resección de las amígdalas cerebelosas (AU)

Glossopharyngeal neuralgia is a rare disease whose initial treatment is pharmacological. When medical therapy is not effective, different surgical options are available including stereotactic radiosurgery, microvascular decompression or nerve section. It is reported a case of a 26-year-old female with glossopharyngeal neuralgia and Chiari malformation. This rare type of neuralgia sometimes is associated with an abnormality of the cranio-cervical junction. It was performed a posterior fossa expansion with duraplasty and microvascular decompression. The patient showed a complete disappearance of the pain, with no need of tonsil resection (AU)

Imaging manifestations on sequential magnetic resonance imaging in pharyngolaryngeal involvement by varicella zoster virus.

Clarifying temporal changes in magnetic resonance imaging (MRI) offers a good chance to understand the pathology of neural lesions; however, such information is scarce in varicella zoster virus (VZV) neuropathies for the glossopharyngeal and vagus nerves. Here, we present the changes in sequential MR images of such a pathology over a period of 12 months from symptom onset.A 27-year-old woman with difficulty in swallowing and hoarseness due to a palatal palsy and arytenoid fixation on the left presented 2 days after onset. MRI revealed a lesion which largely filled the left jugular foramen on T2-weighted images (T2-WI) with high diffusion-weighted imaging (DWI) signals, which has never been previously described, on the 3rd day after onset. The DWI signals were highest on day 3, then deteriorated over 2 months until the signal was only detectable at the intracranial level, but not in the jugular foramen. The glossopharyngeal nerve had returned to normal by 2 months.The time course of the glossopharyngeal and vagus nerve swelling detected on T2-WI suggests that nerve swelling reduces over several months, even though the paralytic symptoms persist. Furthermore, the high DWI signal suggests that nerve swelling was caused by edematous swelling of the nerve fibers, rather than fiber disruption with water displacement in the extracellular space. These findings may provide good clues to speculate on the dynamically changing pathology of VZV neuropathies of the glossopharyngeal and vagus nerves.

Repeat Gamma Knife Radiosurgery for Recurrent Glossopharyngeal Neuralgia: A Systematic Review and Our Initial Experience.

BACKGROUND: Glossopharyngeal neuralgia (GPN) is a rare facial pain syndrome with debilitating symptoms. For medication-resistant GPN, stereotactic radiosurgery (SRS) is an emerging treatment option with a promising role; however, recurrence rates after SRS are fairly high. We present a patient who underwent repeat SRS for recurrent GPN and subsequently maintained over 3 years of complete pain relief. For the first time, we present a systematic review of repeat SRS for recurrent GPN. SUMMARY: Twelve cases of repeat SRS for GPN have previously been reported in the literature (13 studies including ours). Among patients with follow-up, initial pain relief was achieved in 83% (n = 10) of cases a median of 5 weeks after repeat SRS; 2 patients failed to obtain any pain relief. A favorable pain response (BNI I-IIIb) was achieved in 67 and 58% of cases at 6 and 12 months, respectively. All 13 were targeted to the glossopharyngeal meatus. Three patients (23%) experienced adverse radiation effects. Five patients (50%) experienced recurrence a median of 14 months after repeat SRS. Two patients (17%) required additional surgical intervention. At the final follow-up, 75% (n = 9) of the patients had a favorable pain outcome. Key Messages: Repeat SRS may be a viable alternative to open surgery for the treatment of recurrent GPN, albeit with an increased risk of adverse radiation effects. Though limited by a small cohort of patients, the best predictors of an effective second treatment may be a response to initial SRS for >5 months, a maximum dose >75 Gy, and a target at the glossopharyngeal meatus. Larger prospective studies are needed to better define its role.

Microvascular Decompression Alone without Rhizotomy Is an Effective Way of Treating Glossopharyngeal Neuralgia: Clinical Analysis of 46 Cases.

BACKGROUND: Microvascular decompression (MVD) has been the right choice for glossopharyngeal neuralgia (GPN) patients. However, whether glossopharyngeal/vagal nerve root rhizotomy should be combined with MVD is still controversial. OBJECTIVE: To evaluate whether glossopharyngeal/vagal nerve root rhizotomy during MVD is necessary for the treatment of GPN. METHODS: We performed a retrospective study of 46 GPN patients who underwent MVD surgery alone in our hospital, and their patient demographics, clinical presentations, and intraoperative findings are shown. The immediate and long-term follow-up outcomes were investigated to show the treatment's efficiency and safety; the outcome was also compared with our previous study. The relevant literature was reviewed to show complications for GPN patients undergoing glossopharyngeal/vagal nerve root rhizotomy with MVD. RESULTS: The most common offending vessel was the posterior inferior cerebellar artery (60.9%). 100% of the patients were pain-free (score of I on the Barrow Neurological Institute pain intensity [BNI-P] scale) immediately after MVD surgery, while 1 patient relapsed with occasional pain 12 months after the operation (score of III on the BNI-P scale). Poor wound healing and hearing loss were found in 1 case each. No complications related to the glossopharyngeal nerve/vagal nerve were reported. Some surgical techniques, such as thorough exploration of the CN IX-X rootlets, full freeing from arachnoid adhesions, and usage of a moist gelatin sponge, can improve the success rate of the operation. CONCLUSIONS: MVD alone without rhizotomy is an effective and safe method for patients with GPN.

Efficacy Comparison of Microvascular Decompression and Rhizotomy in the Treatment of Glossopharyngeal Neuralgia: A Retrospective Analysis of 37 Cases.

AIM: To evaluate and compare the long-term efficacy and safety of microvascular decompression (MVD) and glossopharyngeal nerve roots rhizotomy (GNR) in the treatment of glossopharyngeal neuralgia (GN). MATERIAL AND METHODS: The data of 37 patients with glossopharyngeal neuralgia undergoing MVD alone and MVD + GNR from July 2004 to March 2017 were analyzed retrospectively. RESULTS: Efficacy: In the 22 cases of the MVD group, 19 cases were cured and 3 cases were improved. In the 15 cases of the MVD + GNR group, 14 cases were cured and 1 case improved. There was no significant difference between the two groups (chi-square test, p > 0.05). COMPLICATIONS: Postoperative complications in the MVD group: 2 patients had short-term hoarseness and drinking cough, 1 case had cerebrospinal fluid leakage, 1 case had intracranial infection, and 1 case had ipsilateral hearing loss, with no deaths. Postoperative complications in the MVD + GNR group: permanent hoarseness in 2 cases, short-term drinking cough and hoarseness in 4 cases, ipsilateral facial paralysis in 1 case, and cerebrospinal fluid leakage in 1 case, with no intracranial infection or death. The incidence of postoperative hoarseness and drinking cough in the MVD + GNR group was higher than that in the MVD group (chi-square test, p < 0.05). CONCLUSION: MVD alone was a safe and effective method for the treatment of primary glossopharyngeal neuralgia. The efficacy was equivalent to MVD + GNR, and there was a lower incidence of hoarseness and drinking cough.

CT-guided percutaneous radiofrequency thermocoagulation for glossopharyngeal neuralgia: A retrospective clinical study of 117 cases.

OBJECTIVE: Glossopharyngeal neuralgia (GPN) is a rare disorder of the ninth cranial nerve. Percutaneous radiofrequency thermocoagulation (PRT) is an established treatment for neuropathic pain. Since PRT was first applied with GPN, only a few studies have provided detailed reports on its clinical outcomes and complications, and the number of cases was small. The aim of this study was to investigate the effects, incidence rates, and severity of adverse events of computed tomography (CT)-guided PRT in 117 patients with GPN. PATIENTS AND METHODS: A total of 117 patients with idiopathic GPN underwent CT-guided PRT from July 2004 to December 2016. A retrospective review of medical records was performed to investigate baseline characteristics and immediate outcomes after operation. Long-term outcomes were obtained via telephone interviews. Patients were followed up at 3 months, 6 months, and thereafter, every year after operation. According to Barrow Neurological Institute (BNI) pain scale, the effects of this treatment were categorized into 5 levels. Adverse events, frequencies, severity, and recovery times of complications were recorded. RESULTS: Patients who were classified into BNI class I and BNI class II experienced excellent pain relief. Ninety-six patients (82.1%) achieved "excellent" pain relief immediately after treatment. The mean follow-up period was 73.6 months (range, 13-150). With regard to long-term outcomes, the percentage of patients who experienced "excellent" pain relief was 75.9% at 1 year, 63.0% at 3 years, 54.0% at 5 years, 44.2% at 10 years, and 39.3% at 12.5 years. Complications, which included dysphagia, lingual numbness, pharynx and larynx numbness, hoarseness, and abnormal sense of taste, were graded 1 as defined by the Landriel Ibanez classification, and all complications disappeared within 12.9 ± 5.1 weeks. CONCLUSION: This study indicates that PRT is a minimally invasive procedure that leads to minor complications and is proven to have immediate and long-term effectiveness for managing GPN. It is especially suitable for patients with contraindication to surgery and patients who require recurrent treatment. We provide a detailed report of the adverse events experienced by GPN patients who underwent PRT.

Neuralgia glosofaríngea asociada a síncopes y secundaria a carcinoma de cuello / Glossopharyngeal neuralgia associated with syncopes and secondary to neck carcinoma

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MRI Findings and Outcomes in Patients Undergoing Microvascular Decompression for Glossopharyngeal Neuralgia.

BACKGROUND AND PURPOSE: Glossopharyngeal neuralgia causes extreme paroxysmal pain in the posterior pharynx, tonsillar region, base of tongue, or deep ear, that is, the distribution of the glossopharyngeal nerve. Some cases of glossopharyngeal neuralgia are associated with neurovascular conflict, usually by the posterior inferior cerebellar artery. Such symptomatic compression occurs only in proximal, centrally myelinated portions of the glossopharyngeal nerve near the brainstem. Microvascular decompression provides effective and durable pain relief for properly selected patients with medically refractory glossopharyngeal neuralgia. The purpose of this study is to describe a tailored MRI evaluation of neurovascular conflict in glossopharyngeal neuralgia to improve candidate selection for microvascular decompression. METHODS: Our team developed a glossopharyngeal neuralgia imaging and evaluation protocol including a grading system for neurovascular conflict of the glossopharyngeal nerve and applied it to evaluate candidates for microvascular decompression. RESULTS: Our team grades neurovascular conflict as "contact" (vessel touching nerve without intervening cerebrospinal fluid) versus "deformation" (deviation or distortion of nerve from its normal course by the offending vessel). MRIs of patients with glossopharyngeal neuralgia demonstrate proximal neurovascular conflict. Postoperative MRI demonstrates separation of the glossopharyngeal nerve from the offending vessel. CONCLUSION: A tailored glossopharyngeal neuralgia imaging evaluation protocol is presented. We believe this approach has helped improve microvascular decompression outcomes and reduce unnecessary procedures at our institution. Further research may elucidate whether clinical and imaging features, including neurovascular conflict severity, predict surgical outcome for glossopharyngeal neuralgia.

Endoscopically Assisted Targeted Keyhole Retrosigmoid Approaches for Microvascular Decompression: Quantitative Anatomic Study.

OBJECTIVE: We describe and quantitatively assess minimally invasive keyhole retrosigmoid approaches targeted to the upper, middle, and lower cranial nerve (CN) complexes of the cerebellopontine angle (CPA). METHODS: Anatomic dissections were performed on 10 sides of 5 fixed, silicone-injected cadaver heads. Surgical views through various trajectories were assessed in endoscopic videos and 3-dimensional (3D) interactive virtual reality microscope views. Surgical freedom and angles of attack to the proximal and distal areas of CN complexes of the CPA were compared among upper and lower keyholes and conventional retrosigmoid craniotomy using neuronavigation. RESULTS: Compared with keyholes, the conventional approach had superior surgical freedom to most areas except for the distal CN V, the root of CN VII, and the root of CN IX, where differences were not significant. The conventional retrosigmoid approach provided a larger horizontal angle of attack than either the upper or lower keyholes for all selected areas; however, the vertical angles of attack were not different. Splitting the petrosal fissure resulted in a significant increase in the vertical angle of attack to the root zones of CNs V and VII but not to the distal areas of these nerves or CN IX. Illustrative cases of endoscope-assisted keyhole retrosigmoid approaches for the treatment of trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia are presented. CONCLUSIONS: Targeted keyhole retrosigmoid approaches require detailed understanding of the 3D anatomy of the CPA to create appropriate locations of corridors, including skin incisions and keyholes. Endoscope assistance complements the standard microsurgical technique by maximizing the visualization and identification of the delicate neurovascular structures.

Surgical treatment of bilateral glossopharyngeal neuralgia.

Glossopharyngeal neuralgia (GPN) is a condition characterised by sudden, severe pain in the distribution of the glossopharyngeal nerve. It can be triggered by talking, yawning, coughing and swallowing. Classically, patients experience a unilateral lancinating and excruciating pain described as electrical shock-like pain in the areas around the ear, tongue, or the mandibular angle. Uncommon manifestations include cardiac arrhythmias and syncope during pain episodes. Surgery is indicated in refractory cases. Bilateral GPN is rare, and definitive surgical treatment for bilateral GPN has not yet been reported. In this case report, a young woman with bilateral GPN who underwent staged surgery bilaterally is described. She did not develop life-threatening cardiac abnormalities postoperatively.

Novel Technical Variations and Increased Adhesive Strength in the "Birdlime" Transposition Technique for Microvascular Decompression.

BACKGROUND: Vessel transposition is the standard procedure in microvascular decompression (MVD), which necessitates more complex and difficult surgical techniques, including suturing in the deep operative field. We recently reported the usefulness of TachoSil tissue sealing sheets soaked with fibrin glue for vessel transposition in MVD, known as the "birdlime" technique. Here we describe various technical variations and improved adhesive strength of this material for safe and optimal vessel transposition without complications. METHODS: We retrospectively reviewed the operative findings of 31 consecutive patients who underwent MVD with the transposition procedure using TachoSil tissue sealing sheet soaked with fibrin glue between January 2012 and June 2017. The adhesive strength of TachoSil soaked with fibrin glue was measured using porcine dura mater. RESULTS: A single piece of this material was required in 13 patients, a single piece for multiple lesions was required in 5 patients, multiple pieces for a long segment were required in 12 patients, mainly with the offending vertebral artery in 9 patients, stacked pieces in 2 patients, and direct adhesion of vessels in 6 patients. All patients except 1 had total remission of symptoms immediately after the procedure. There were no severecomplications, recurrences, adhesions, or cranial nerve dysfunction in any patient. TachoSil soaked with fibrin glue showed significantly higher adhesive tensile strength compared with fibrin glue or Teflon felt with fibrin glue. CONCLUSIONS: Modifications of the birdlime technique and reinforcement of the adhesive strength provide an easy and adjustable way to perform MVD safely and effectively to reduce the risk of intraoperative neurovascular injury.

[A Case of Coexistent Glossopharyngeal Neuralgia and Hemifacial Spasm Successfully Treated with Transposition of the Vertebral Artery].

A case of coexistent glossopharyngeal neuralgia and hemifacial spasm was treated by transposition of the vertebral artery. A 60-year-old man was referred to our hospital due to pain in the left posterior part of the tongue that was difficult to control with oral medication at a local hospital. The diagnosis was left glossopharyngeal neuralgia based on the symptoms, imaging findings, and lidocaine test results. Moreover, the patient had left hemifacial spasm. Microvascular decompression was performed, which confirmed that the vertebral artery was compressing the lower cranial nerve and the posterior inferior cerebellar artery was compressing the root exit zone of the facial nerve. The vertebral artery and posterior inferior cerebellar artery were transposed using TachoSil®. After the surgery, both glossopharyngeal neuralgia and hemifacial spasm disappeared, and the patient was discharged.

Microvascular Decompression for Glossopharyngeal Neuralgia: Long-Term Follow-Up.

OBJECTIVE: To examine operative findings and outcome of microvascular decompression (MVD) for glossopharyngeal neuralgia (GPN). This research displayed the long-term outcomes of a large series of 35 cases with GPN treated with MVD. METHODS: From January 2004 to June 2006, 35 consecutive patients were diagnosed with GPN. All of them underwent MVD. Demographic data, clinical presentation, operative findings, clinical results, operative complications were reviewed. RESULTS: A total of 33 patients (94.3%) experienced complete pain relief immediately after MVD. Long-term follow-up was available for 30 of these 35 patients, and 28 of these 30 patients continued to be pain-free. There was no long-term operative morbidity in all cases. One patient had a cerebrospinal fluid leak and 1 case presented with delayed facial palsy. CONCLUSIONS: Classic GPN is usually caused by pulsatile neurovascular compression of the glossopharyngeal and vagus rootlets. MVD is a safe, effective, and durable operation for GPN.

Developmental abnormalities of the craniocervical junction resulting in Collet-Sicard syndrome.

BACKGROUND CONTEXT: Collet-Sicard syndrome describes the paralysis of cranial nerves IX-XII and is the most frequently reported neurologic complication associated with Jefferson fractures. As the lateral mass of the atlas is displaced laterally toward the styloid process and the stylohyoid ligament, the lateral mass impinges on cranial nerves IX-XII. However, Collet-Sicard syndrome in association with other anomalies of the atlas has rarely been reported. PURPOSE: The aim of this study was to report an unusual case of Collet-Sicard syndrome as a result of developmental abnormalities of the craniocervical junction. STUDY DESIGN/SETTING: This is a case report of a single patient. METHODS: Chart and radiographic data were reviewed and reported. RESULTS: We report a 70-year-old man who developed hoarseness, dysarthria, and dysphagia from developmental abnormalities of the craniocervical junction including a congenital occiput-C1-C3 fusion and hypoplastic dens. On computed tomography, the distance between the left transverse process of the atlas and the left styloid process of the skull was 3 mm. CONCLUSION: In suspected Collet-Sicard syndrome, developmental abnormalities of the craniocervical junction should be considered in the differential diagnosis.