Preoperative Frailty Predicts Worse Outcomes after Microvascular Decompression for Trigeminal Neuralgia, Hemifacial Spasm, and Glossopharyngeal Neuralgia: A Multicenter Analysis of 1,473 Patients from a Prospective Surgical Registry.

INTRODUCTION: Microvascular decompression (MVD) is an efficacious neurosurgical intervention for patients with medically intractable neurovascular compression syndromes. However, MVD may occasionally cause life-threatening or altering complications, particularly in patients unfit for surgical operations. Recent literature suggests a lack of association between chronological age and surgical outcomes for MVD. The Risk Analysis Index (RAI) is a validated frailty tool for surgical populations (both clinical and large database). The present study sought to evaluate the prognostic ability of frailty, as measured by RAI, to predict outcomes for patients undergoing MVD from a large multicenter surgical registry. METHODS: The American College of Surgeons-National Surgical Quality Improvement Program (ACS-NSQIP) database (2011-2020) was queried using diagnosis/procedure codes for patients undergoing MVD procedures for trigeminal neuralgia (n = 1,211), hemifacial spasm (n = 236), or glossopharyngeal neuralgia (n = 26). The relationship between preoperative frailty (measured by RAI and 5-factor modified frailty index [mFI-5]) for primary endpoint of adverse discharge outcome (AD) was analyzed. AD was defined as discharge to a facility which was not home, hospice, or death within 30 days. Discriminatory accuracy for prediction of AD was assessed by computation of C-statistics (with 95% confidence interval) from receiver operating characteristic (ROC) curve analysis. RESULTS: Patients undergoing MVD (N = 1,473) were stratified by RAI frailty bins: 71% with RAI 0-20, 28% with RAI 21-30, and 1.2% with RAI 31+. Compared to RAI score 19 and below, RAI 20 and above had significantly higher rates of postoperative major complications (2.8% vs. 1.1%, p = 0.01), Clavien-Dindo grade IV complications (2.8% vs. 0.7%, p = 0.001), and AD (6.1% vs. 1.0%, p < 0.001). The rate of primary endpoint was 2.4% (N = 36) and was positively associated with increasing frailty tier: 1.5% in 0-20, 5.8% in 21-30, and 11.8% in 31+. RAI score demonstrated excellent discriminatory accuracy for primary endpoint in ROC analysis (C-statistic: 0.77, 95% CI: 0.74-0.79) and demonstrated superior discrimination compared to mFI-5 (C-statistic: 0.64, 95% CI: 0.61-0.66) (DeLong pairwise test, p = 0.003). CONCLUSIONS: This was the first study to link preoperative frailty to worse surgical outcomes after MVD surgery. RAI frailty score predicts AD after MVD with excellent discrimination and holds promise for preoperative counseling and risk stratification of surgical candidates. A risk assessment tool was developed and deployed with a user-friendly calculator: https://nsgyfrailtyoutcomeslab.shinyapps.io/microvascularDecompression.

Glossopharyngeal neuralgia with cardioinhibitory syncope: Is a permanent pacemaker required?

Glossopharyngeal neuralgia is a rare facial pain syndrome, which in more rare cases can be associated with syncope. We present the outcome of a case report that combines this rare association that received medical therapy with anti-epileptic medication and permanent dual chamber pacemaker implantation. In this case, syncope episodes were associated with both vasodepressor and cardioinhibitory reflex syncope types. The patient found relief from syncope, hypotension, and pain after initiation of anti-epileptic therapy. Although a dual chamber pacemaker was implanted, the pacemaker interrogation revealed no requirement for pacing at one-year follow-up. As far as we know, this is the first case that reports pacemaker interrogation during follow-up and, taking into account the absence of pacemaker activation at one-year follow-up, the device was not needed to prevent bradycardia and syncope episodes. This case report supports the current guidelines for pacing in neurocardiogenic syncope, by demonstrating a lack of requirement for pacing in the event of both cardioinhibitory and vasodepressor responses.

Refractory glossopharyngeal neuralgia successfully treated with onabotulinumtoxinA: A case report.

BACKGROUND: Glossopharyngeal neuralgia is a rare but severe and disabling pain condition often caused by vascular compression of the glossopharyngeal nerve. Treatment is similar to that of trigeminal neuralgia, but some patients may be refractory to both medical and surgical approaches. Here we present a case of refractory glossopharyngeal neuralgia that responded well to onabotulinumtoxinA (BTX-A). CASE: We report a case of a 65-year-old man with well-controlled human immunodeficiency virus disease with glossopharyngeal neuralgia symptoms since 2015. He had partial response to medications but was limited by side-effects. He underwent microvascular decompression twice with initial relief both times, but experienced recurrence of attacks 1-3 years after each surgery. He was treated with BTX-A using the chronic migraine PREEMPT protocol (i.e., 31-39 injection sites in head and neck muscles), which led to significant relief of his glossopharyngeal neuralgia pain. CONCLUSIONS: This is the first case to our knowledge of glossopharyngeal neuralgia treated with BTX-A. BTX-A can be an effective treatment for glossopharyngeal neuralgia, even when injections are not administered directly over the sensory distribution of the glossopharyngeal nerve.

Imaging of Cranial Neuralgias.

Cranial neuralgia (CN) can cause significant debilitating pain within a nerve dermatome. Accurate diagnosis requires detailed clinical history and examination, understanding of pathophysiology and appropriate neuroimaging to develop an optimal treatment plan. The objective of this article is to review and discuss some of the more common CNs including trigeminal neuralgia and its associated painful neuropathies, occipital neuralgia, and less common glossopharyngeal neuralgia (GPN). The neuroanatomy, pathophysiology, diagnostic imaging, and treatment of each of these pathologies are reviewed with emphasis on the role of CT and MR imaging findings in guiding diagnosis. Although CT is often used to initially identify an underlying cause such as neoplasm, infection, or vascular malformation, MRI is optimal. Clinical history and examination findings along with MRI constructive interference steady state/fast imaging employing steady-state acquisition sequences and MRA of the brain can be used to distinguish between primary and secondary cranial neuropathies and to discern the best treatment option. Pharmacologic and noninvasive therapy is the first-line of treatment of these cranial and cervical neuralgias. If symptoms persist, stereotactic radiosurgery is an option for some patients, although microvascular decompression surgery is the most curative option for both trigeminal and GPN. Refractory occipital neuralgia can be treated with a nerve block, an ablative procedure such as neurectomy or ganglionectomy, or more recently occipital nerve stimulation.

Chiari type I malformation discovered through a glossopharyngeal neuralgia.

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.

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.

A Unique Case of Microvascular Triple Decompression for Combined Simultaneous Trigeminal Neuralgia, Hemifacial Spasm, and Glossopharyngeal Neuralgia Because of the Dolichoectatic Vertebrobasilar System.

BACKGROUND: Trigeminal neuralgia (TN), hemifacial spasm (HFS), and glossopharyngeal neuralgia (GPN) are hyperactive dysfunction syndromes (HDS) commonly caused by microvascular compression of their root entry zone. Cases of combined HDS involving 2 or more of these entities are extremely rare. Although microvascular decompression is the surgical treatment of choice, there are additional techniques that have been described as efficient methods to accomplish vessel transposition. OBJECTIVE: To our knowledge, we present the first reported case of triple simultaneous HDS successfully treated using the clip-sling technique to achieve microvascular decompression. We discuss several technical pearls and pitfalls relevant to the use of the sling suspension technique. METHODS: We report the rare case of a 66-yr-old male with combined simultaneous unilateral right-sided TN, HFS, and GPN because of a dolichoectatic vertebrobasilar system compressing the exit zones of the right trigeminal, facial, and glossopharyngeal nerves and present a literature review of combined HDS and their different surgical treatments. RESULTS: Symptomatic TN, HFS, and GPN have been reported 8 times in the literature with our case being the ninth. A retrosigmoid craniotomy was performed for microvascular decompression of the brainstem with a clip-sling suspension technique augmented with Teflon felt pledgets. The patient had immediate complete relief from TN, HFS, and GPN postoperatively. CONCLUSION: Microvascular decompression using the clip-sling technique via a retrosigmoid approach should be considered as a safe and effective option for transposition and suspension of the offending artery and decompression of the affected nerve roots in cases of combined HDS.

Concurrent Glossopharyngeal Neuralgia and Hemi-Laryngopharyngeal Spasm (HeLPS): A Case Report and a Review of the Literature.

BACKGROUND AND IMPORTANCE: Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION: A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION: This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.

"Ortoglossopelveplastia" e o algoritmo de sua utilização na sequência de Pierre-Robin / "Orthoglossopelveplasty" and the algorithm for its use in the Pierre Robin sequence

Introdução: Muitos pacientes portadores de sequência de Pierre Robin (micrognatia, glossoptose e obstrução de via aérea) apresentam o músculo genioglosso alterado, encurtado e retrátil, que impede a protração lingual, mantendo a parte anterior da língua verticalizada e seu volume deslocado em direção posterior. Isso pode corroborar para obstrução supraglótica, dificuldade alimentar e inversão das forças de estímulo do crescimento do corpo mandibular. Métodos: Estudo retrospectivo de pacientes com Pierre Robin tratados entre 2012 e 2017 pela equipe, com descrição da "ortoglossopelveplastia", que propõe uma modificação na glossopexia, soltando o genioglosso anômalo da sua inserção, liberando a língua para elevar seu terço anterior e avançar o volume de sua base, sendo auxiliada por ponto de tração da base lingual à sínfise mandibular. Apresentamos um algoritmo de tratamento proposto que prioriza a necessidade desta cirurgia, associada ou não à distração mandibular, de acordo com a gravidade da dificuldade respiratória e/ou alimentar. Resultados: São apresentados 12 casos de obstrução da orofaringe atendidos de 2012 a 2017, discutem-se suas prioridades, a ortoglossopelveplastia e se aplica o algoritmo proposto. Conclusão: A reorganização anatômica da musculatura em uma posição anteriorizada correta proporciona protração e funcionalidade à língua, com desobstrução da via aérea na orofaringe, melhora da função alimentar e do desenvolvimento mandibular, com baixa morbidade cirúrgica e poucas complicações.

Introduction: Several patients with the Pierre Robin sequence (micrognathia, glossoptosis, and airway obstruction) have an altered, shortened, and retractable genioglossus muscle that prevents protraction of the tongue and keeps the anterior part of the tongue vertical and its volume posteriorly displaced. This can contribute to supraglottic obstruction, feeding difficulty, and inversion of the growth stimulation forces of the mandibular body. Methods: A retrospective study of patients with the Pierre Robin sequence treated between 2012 and 2017 with "orthoglossopelveplasty," which includes modification of glossopexy, releasing the anomalous genioglossus of its insertion and releasing the tongue to raise its anterior third and advance the volume of its base using a traction suture of the tongue base to the mandible symphysis. We present a treatment algorithm that prioritizes the need for surgery associated, or not, with mandibular distraction in accordance with respiratory and/or feeding difficulty severity. Results: Twelve cases of oropharyngeal obstruction treated from 2012 to 2017 are presented, their priorities and orthoglossopleoplasty are discussed, and the proposed algorithm is applied. Conclusion: Anatomical reorganization of the musculature in a correct anterior position provides protraction and functionality to the tongue, clears the airway in the oropharynx, and improves the feeding function and mandibular development, with low surgical morbidity rates and few complications.

[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.

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.

Vagoglossopharyngeal neuralgia revealed through predominant digestive vagal manifestations. Case report and literature review.

Vagoglossopharyngeal neuralgia is a rare pathology whose atypical forms, dominated by syncopal manifestations, are still rarer. Although the territory of the vagus nerve involves, beyond the cardiovascular system, the respiratory and the digestive systems, there is no report in literature of atypical forms other than syncopal. Therefore, the authors were prompted to report the case of a patient whose vagoglossopharyngeal neuralgia was predominantly revealed by digestive symptoms. A 58-year-old patient presented with stereotypical severe digestive disturbances including nausea, vomiting and diarrhoea. High definition cranial MRI showed a neurovascular conflict between the posterior inferior cerebellar artery and the IXth and Xth nerves, on the right side. A microsurgical decompression was carried out which confirmed the vascular compression and successful transposition of the artery. One year after the surgery, the patient was free from all painful and digestive symptoms. A survey of the literature did not find any reference to digestive symptoms together with the neuralgia; only a syncopal type of cardiac symptoms related to the parasympathetic nervous system were described. The hypothesis was that the revealing digestive symptoms are linked to a similar parasympathetic mechanism, implying the visceral component of the Xth cranial nerve.

Microvascular decompression in patients with coexistent trigeminal neuralgia, hemifacial spasm and glossopharyngeal neuralgia.

BACKGROUND: Trigeminal neuralgia(TN), hemifacial spasm (HFS) and glossopharyngeal neuralgia (GPN) were referred to hyperactive dysfunction syndromes (HDSs) of the cranial nerves. These symptoms may occur synchronously or metachronously, but the combination of three diseases is extremely rare. METHODS: From 2007 through 2013, six patients with coexistent GPN-HFS-TN were treated in our department. The combined symptoms occurred on the same side in three and on both sides in three. These patients underwent nine microvascular decompression (MVD) procedures in total. The clinical data including operative findings were respectively analyzed, and the etiological factors as well as treatment strategies were discussed. RESULTS: Intraoperatively, in all the cases a small posterior fossa was found, which was crowded with cranial nerve roots and cerbellar vesels. Postoperatively, spasm was stopped immediately in four and within 3 months in two; the symptom of TN disappeared immediately in four and within 2 weeks in two; the symptom of GPN was relieved immediately in four and improved with medication in two. During the up to 77 months' follow-up, no changes, recurrence or any dysfunctions of cranial nerves were observed in any of the patients. CONCLUSIONS: The combination of HFS-TN-GPN is extremely rare and is often associated with a looped VBA and a smaller posterior fossa. However, MVD is still a good choice for treatment. To achieve a safe and effective outcome, dissection of the caudal cranial nerves and proximal transposition of the vertebral artery before decompression of the affected nerve roots are strongly recommended.

[A patient with vertebral artery dissection who initially suffered from pharyngeal pain].

We have encountered a case of a patient with bilateral vertebral artery (VA) dissection who suffered from severe pharyngeal pain. A 61-year-old man, who initially visited a nearby hospital for investigation of mild headache, was pointed out to have a left VA aneurysm. The next morning, severe pharyngeal pain on the right side suddenly occurred. The cause was unknown even when consultation was made to an otolaryngology and oral and maxillofacial surgeon. Sore throat improved in two days. On the 12th day after pharyngeal pain, the patient entered our hospital with a diagnosis of VA dissection. Imaging studies indicated severe stenosis of the right VA and an aneurysm of the left VA which confirmed the diagnosis of bilateral VA dissection. After severe stenosis of the right VA improved a little, the trapping of the left VA aneurysm was performed with bypass surgery of the occipital artery to the posterior inferior cerebellar artery. Although there have been reports of glossopharyngeal neuralgia due to compression of dissective aneurysms of VA, no report exists in terms of a sore throat due to VA dissection without glossopharyngeal neuralgia. This symptom was considered to be involved in the referred pain.

Giant dumbbell-shaped intra- and extracranial nerve schwannoma in a child presenting with glossopharyngeal neuralgia syncope syndrome: a case report and review of the literature.

Lower cranial nerve schwannomas are benign tumors of the neurolemmocytes of the cranial nerves. Among children, cranial nerve schwannomas are extremely rare and are predominantly associated with neurofibromatosis (NF) type 2. The purpose of the current case report is to describe a unique giant extra- and intracranial foramen jugular schwannoma in a young boy with lower cranial nerve deficits and glossopharyngeal neuralgia syncope syndrome and to review the pertinent literature. In the current case report, we illustrate the course of disease in a 14-year-old boy with a 4-month history of recurrent syncope and a big bulge on the left side of his neck. Audiometry showed deafness of the left ear. Magnetic resonance imaging (MRI) demonstrated a giant unilateral dumbbell-shaped intra- and extracranial foramen jugular schwannoma with a volume of 156 cm3 causing severe brain stem compression and obstructive hydrocephalus. The tumor was removed completely in a two-step surgery. The tumor was confirmed during surgery to originate from the glossopharyngeal nerve. The histological examination revealed the characteristic features of a schwannoma. The MRI 3 months after the second surgery confirmed complete tumor removal. The genetic examination for NF was negative. Review of literature showed that dumbbell-shaped lower cranial nerve schwannomas in the childhood population are rare.

Jugular foramen schwannoma presenting with glossopharyngeal neuralgia syncope syndrome.

INTRODUCTION: Jugular foramen schwannomas are rare skull base tumours which typically have a variable clinical presentation. Glossopharyngeal syncope syndrome is an unusual clinical presentation; in the following case report, it was the sole presentation of an extracranial jugular foramen tumour. METHODS: The presentation of a patient with glossopharyngeal neuralgia syncope syndrome is reviewed and the pathophysiology, clinical features and treatment discussed. RESULTS: A 45-year-old woman presented with unilateral throat pain, bradycardia and hypotension leading to episodes of impaired consciousness when lying on her left side or turning her head to the left. Imaging detected a left-sided extracranial jugular foramen schwannoma. The tumour was excised, and the patient had no more syncopal attacks. CONCLUSION: Glossopharyngeal neuralgia syncope syndrome can be the sole presentation of a jugular foramen schwannoma. Although this syndrome may be treated with anti-dysrhythmic drugs, cardiac pacing or nerve section, in the presented patient excision of the jugular foramen schwannoma was successful in preventing further episodes of syncope.

[Vagoglossopharyngeal neuralgia treated with vascular decompression]. / Vagoglossofaryngeusneuralgi behandlet med mikrovaskulaer dekompression.

This case report describes a 63-year-old man referred with right-sided glossopharyngeal neuralgia. Magnetic resonance imaging (MRI) demonstrated asymmetry of the PICA vessels. A microvascular decompression was performed and complete remission of symptoms followed. After 14 months the neuralgia recurred, this time accompanied by sinus bradycardia/sinus arrest during attacks causing discomfort and syncopes. An MRI now demonstrated an arterial loop at the site of the vagoglossopharyngeal complex leaving the brainstem. After another microvascular decompression, complete remission of symptoms was observed.