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.

Avellis syndrome with ipsilateral prosopalgia, glossopharyngeal neuralgia, and central post-stroke pain: A case report and literature review.

RATIONALE: Avellis syndrome is a rare bulbar syndrome. The main lesions may involve nucleus ambiguus and the lateral spinothalamic tract. The typical reported clinical manifestations are hoarseness, dysphagia, pain, and temperature disturbance of contralateral body. The manifestations, however, may vary. We aim to report new manifestations of Avellis syndrome in this report. PATIENT CONCERNS: A 47-year-old Chinese peasant woman who felt sudden dizziness, nausea when she was doing the laundry was referred to our department from other hospital. She vomited the stomach contents once and complained numbness of the left trunk and limbs as well as coughing while drinking. The patient presented with palatopharyngeal paralysis, Horner syndrome, and diminished pain as well as temperature sensation in the contralateral face, trunk, and limbs. She also presented with ipsilateral prosopalgia, glossopharyngeal neuralgia, and central poststroke pain. DIAGNOSES: T2-weighted MRI images demonstrated a high-signal intensity lesion in the right medulla oblongata which indicated a banded infarction site. The patient was diagnosed with medulla oblongata infarction, Avellis syndrome, Horner syndrome, dysphagia, hemiparesthesia, ipsilateral prosopalgia, glossopharyngeal neuralgia, and central poststroke pain. INTERVENTIONS: The patient was administrated aspirin to prevent the aggregation of platelet and rosuvastatin tablets to regulate lipids as well as to stabilize vascular plaque. She was injected with butylphthalide sodium chloride to improve nerve nutritional status and carbamazepine was prescribed to deal with prosopalgia and glossopharyngeal neuralgia. Gabapentin and pregabalin were administrated to deal with the central poststroke pain. OUTCOMES: The symptoms of prosopalgia as well as glossopharyngeal neuralgia were gone, and dizziness, dysphagia, and Horner syndrome were significantly alleviated when she was discharged from the hospital while the interventions showed little effect on central poststroke pain. LESSONS: We reported a case of Avellis syndrome who manifested as the typical reported manifestations. The patient, what's more, presented with ipsilateral trigeminal, glossopharyngeal neuralgia, and central poststroke pain which were described for the first time. It is of great significance for clinicians to recognize the typical as well as other manifestations which helps to make a clear diagnosis.

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.

GLOSSOPHARYNGEAL NERVE AS A SOURCE OF OROFACIAL PAIN - DIAGNOSTIC AND THERAPEUTIC CHALLENGES.

Chronic neuropathic orofacial pain along with physical suffering can cause emotional, psychological and social difficulties, which significantly affects the quality of life of patients. Pain in the area of glossopharyngeal nerve innervation, especially chronic neuropathic, is relatively rare, but is significant because of the great suffering it causes to sufferers. It can be life threatening, due to the cardiac arrhythmia, syncope or convulsions it can cause. Drug treatment is often of limited effectiveness and can be fraught with side effects. It is necessary to look for the etiology of the underlying disease, and if possible, to take adequate causal treatment. This review article discusses the etiology, clinical features, differential diagnosis, and treatment modalities of neuropathic pain in the area of glossopharyngeal nerve innervation.

A case of syncopal convulsions triggered by glossopharyngeal neuralgia.

Syncopal convulsions and epileptic seizures are clinically hard to distinguish and differ in terms of treatment approaches. It is important to consider the cardiac arrhythmias that impair cerebral perfusion in the differential diagnosis of antiepileptic treatment-resistant convulsions. Here we offer a 72 year old male patient glossopharengial neuralgia after swallowing associated with recurrent episodes of syncopal convulsions. The patient was successfully treated with temporary pacemaker and carbamazepine. This phenomenon is noteworthy in terms of both asystole triggered by glossopharengial neuralgia and syncopal convulsions which are rare in the differential diagnosis of epileptic seizures.

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.

Gradual aggravation of idiopathic glossopharyngeal neuralgia due to chronic tonsillitis: A case report.

RATIONALE: Glossopharyngeal neuralgia (GPN) is a rare type of neuralgia. Depending on the cause, it is classified as idiopathic GPN and secondary GPN. Secondary GPN can also be caused by a mass effect or inflammation of the tonsils, the innervation area of glossopharyngeal nerve. PATIENT CONCERNS: The patient was diagnosed idiopathic GPN 8 years ago. The patient had intermittent pain, but the pain was well controlled. From 5 months ago, the pain gradually worsened, the patient complained severe pain which impaired chewing and swallowing function. DIAGNOSES: Idiopathic GPN, secondary GPN, chronic tonsillitis INTERVENTIONS:: Percutaneous glossopharyngeal nerve block, glossopharyngeal neurolysis, and pulsed radiofrequency neuromodulation of glossopharyngeal nerve were performed. After the diagnosis of tonsillitis, antibiotic therapy was performed OUTCOMES:: Two weeks after the antibiotic treatment, the pain decreased simultaneously with the patient's tonsillitis recovery. LESSONS: In a Patient who had already been diagnosed with idiopathic GPN, both pain control and its cause should be considered when the pain is aggravated.

Neuropathic Orofacial Pain.

Neuropathic pain of the orofacial region can cause much distress in individuals presenting with this condition. It may be easily mistaken for dental pain, and hence many individuals may undergo unnecessary dental work. Knowledge of the types of neuropathic orofacial pain may assist in timely diagnosis and improvement of a patient's quality of life.

[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.