Actualización sobre el síndrome de Eagle y sus complicaciones neurocardiológicas: casos clínicos / An update on Eagle´s syndrome and its neuro-cardiological complications: clinical cases

El síndrome de Eagle o síndrome estilohioideo o sín-drome de la arteria carótida es un trastorno que se origina por la mineralización y elongación del pro-ceso estiloides. Factores traumáticos agudos y cró-nicos, así como otras teorías, han sido propuestos para explicar la etiología y patogenia de esta altera-ción. El conjunto de síntomas puede incluir: dolor fa-ríngeo, odinofagia, disfagia, cefalea, con irradiación a oreja y zona cervical. Si bien existen varias clasifi-caciones, de manera universal se acepta que existen principalmente dos formas de presentación de esta patología: el tipo I o clásico, generalmente asociado a un trauma faríngeo y acompañado de dolor en la zona faríngea y cervical, y el tipo II o carotídeo, que sue-le presentar molestia cervical, cefalea y alteración de la presión arterial, con riesgo de daño de la ac-tividad cardíaca. La identificación de este síndrome suele ser confusa dada la similitud de los síntomas con otras afecciones. El diagnóstico debe realizarse en base a los síntomas y a los estudios por imágenes específicos. El tratamiento puede ser conservador y actuar simplemente sobre los síntomas, o bien, qui-rúrgico. El objetivo del presente trabajo es realizar una revisión actualizada de la literatura sobre el sín-drome de Eagle y presentar tres casos clínicos con distintas manifestaciones (AU)

Eagle's syndrome or styloid syndrome or stylo-carotid artery syndrome is a disease caused by mineralization and elongation of the styloid process. Acute and chronic traumatic factors, along with other hypothesis, have been proposed to explain the aetiology and pathogenesis of this condition. Symptoms can include: pharynx pain, odynophagia, dysphagia, headache, with radiating pain to the ear and neck. Despite there are several classifications, it is universally accepted that this pathology can present in two forms: the type I or classic, generally associated to tonsillar trauma and characterized by pharyngeal and neck pain, and the type II or carotid artery type, which frequently presents with neck pain, headache, blood pressure variation, with risk of damage to cardiac function. Identifying of Eagle's syndrome is often confusing because some symptoms are shared with other pathologies. Diagnosis must be made on the basis of symptoms and imaging studies. Treatment can be conservative, acting only on symptoms, or surgical. The aim of this paper is to provide an updated review of the literature on Eagle syndrome and to present three clinical cases with different manifestations (AU)

Posterior condylar canal dural arteriovenous fistula as a rare cause of glossopharyngeal neuralgia: A case report.

BACKGROUND: The symptoms of dural arteriovenous fistula (dAVF) vary according to the location of the fistula and the pattern of venous drainage. Here, we report the case of a patient with a dAVF-induced glossopharyngeal neuralgia. CASE DESCRIPTION: We report a case of a patient with right glossopharyngeal neuralgia caused by a posterior condylar canal (PCC) dAVF. The glossopharyngeal neuralgia was accompanied by persistent tinnitus and repetitive right side otalgia, as well as ipsilateral shoulder and throat pain, lasting for about 30 s. However, there were no specific findings on otoscopic examination. Cranial magnetic resonance imaging was performed to determine the cause of the symptoms, and a right PCC dAVF was observed. The dAVF was successfully obliterated using transvenous coil embolization. After embolization, the patient's symptoms were completely resolved. CONCLUSION: Although glossopharyngeal neuralgia caused by dAVF is rare, it can present due to intracranial lesions located adjacent to the glossopharyngeal nerve or vagus nerve. Brain MRI is therefore required to identify secondary causes in all patients with glossopharyngeal neuralgia.

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

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

Ameliorating Effect of Microvascular Decompression on Patients with Coexistence of Hemifacial Spasm and Glossopharyngeal Neuralgia: A Retrospective Study.

OBJECTIVE: Microvascular decompression (MVD) has been widely accepted for treating hemifacial spasm (HFS) and glossopharyngeal neuralgia (GN); an effective surgical treatment of coexistent HFS and GN still remains to be determined, however. In this paper we discuss the operative strategy of MVD for patients with coexistent HFS and GN. METHODS: This was a retrospective study. All cases of HFS with or without GN at China-Japan Friendship Hospital from January 2014 to June 2016 have been included. All patients underwent MVD and have been followed up for an average of 1.5 years. RESULTS: A total of 5375 cases of HFS were included, wherein 8 cases coexist with GN. Eight patients had same offending vessel(s) compressing the root entry zone of glossopharyngeal nerve and facial nerve. Posterior inferior cerebellar artery was identified as at least 1 of the offending arteries in all 8 patients. After MVD, spasm ceased in all 8 cases, with 7 cases ceasing immediately and 1 within 2 months. Pain disappeared also in all cases, with 7 cases immediately and 1 case after 4 days. No recurrence or complication was observed during the follow-ups. CONCLUSIONS: HFS combined with ipsilateral GN was rare. MVD could be performed to effectively relieve nerve root compression and associated symptoms for coexistent HFS and GN. Sufficient exposure of root entry zones of both nerves and fully decompression of offending blood vessels and exploratory sequences of different nerve roots are critical points for improving operative effect and reducing complications.

Glossopharyngeal Neuralgia Treatment Outcomes After Nerve Section, Microvascular Decompression, or Stereotactic Radiosurgery: A Systematic Review and Meta-Analysis.

BACKGROUND: Glossopharyngeal neuralgia (GPN) is a rare neuralgic pain syndrome amenable to neurosurgical treatments, including nerve section (NS), microvascular decompression (MVD), and stereotactic radiosurgery (SRS). However, thorough comparisons of the modalities have not been performed to date. The objective of the present study was to compare the pain and complication outcomes after these approaches to GPN. METHODS: Searches of 7 electronic databases from inception to June 2018 were conducted following the appropriate guidelines. The incidence rates (IRs) of short-term (≤3 months) and long-term (≥12 months) pain relief and complications were extracted and analyzed using a meta-analysis. Meta-regression was used to assess for heterogeneity. RESULTS: A total of 792 GPN cases managed by NS, MVD, or SRS were described by 6, 11, and 6 studies, reporting outcomes for 282 (36%), 446 (56%), and 67 (8%) cases. The short-term pain relief rate was highest after NS postoperatively (IR, 94%; 95% confidence interval [CI], 88%-98%) and lowest after SRS at 3 months postoperatively (IR, 80%; 95% CI, 68%-96%). The postoperative complication rate was greatest after MVD (IR, 26%; 95% CI, 16%-38%) and lowest after SRS (IR, 0%; 95% CI, 0%-4%). The long-term pain relief rate was greatest after NS (IR, 96%; 95% CI, 91%-99%) and lowest after SRS (IR, 82%; 95% CI, 67%-94%). Statistically significant differences between the approaches were found for each outcome. CONCLUSION: Neurosurgical treatment of GPN is frequently performed by 1 of 3 modalities with unique outcomes profiles. NS might provide the most favorable treatment response, with respect to short- and long-term pain relief and postoperative outcomes.

Trigeminal Neuralgia, Glossopharyngeal Neuralgia, and Myofascial Pain Dysfunction Syndrome: An Update.

Neuropathic pain is a common phenomenon that affects millions of people worldwide. Maxillofacial structures consist of various tissues that receive frequent stimulation during food digestion. The unique functions (masticatory process and facial expression) of the maxillofacial structure require the exquisite organization of both the peripheral and central nervous systems. Neuralgia is painful paroxysmal disorder of the head-neck region characterized by some commonly shared features such as the unilateral pain, transience and recurrence of attacks, and superficial and shock-like pain at a trigger point. These types of pain can be experienced after nerve injury or as a part of diseases that affect peripheral and central nerve function, or they can be psychological. Since the trigeminal and glossopharyngeal nerves innervate the oral structure, trigeminal and glossopharyngeal neuralgia are the most common syndromes following myofascial pain dysfunction syndrome. Nevertheless, misdiagnoses are common. The aim of this review is to discuss the currently available diagnostic procedures and treatment options for trigeminal neuralgia, glossopharyngeal neuralgia, and myofascial pain dysfunction syndrome.

Intraoperative neurophysiological monitoring of microvascular decompression for glossopharyngeal neuralgia.

PURPOSE: To evaluate if adding cranial nerves (CNs) V and VI to standard intraoperative neurophysiological monitoring (IONM) of microvascular decompressions for glossopharyngeal neuralgia improve its efficacy. METHODS: We reviewed all patients who received a microvascular decompression for glossopharyngeal neuralgia at our institution between January 2008 and August 2012. All received upper extremity somatosensory evoked potentials, brainstem auditory evoked potentials, and free-running electromyography of muscles innervated by ipsilateral CNs VII, IX, and X. The sample was divided into 12 patients who received additional monitoring of CNs V and VI and 15 who did not. RESULTS: No difference on neurotonic activity presence was found on CN V (standard IONM: 0% versus additional CNs IONM: 8.33%; p = 0.423), CN VI (never present on the additional CN patients), CN VII (standard IONM: 73.33% versus additional CNs IONM: 66.64%; p = 0.973), CN IX (standard IONM: 40.0% versus additional CNs IONM: 25.0%; p = 0.683), or CN X (standard IONM: 46.67% versus additional CNs IONM: 33.33%; p = 0.701) between groups. Additionally, no differences of brainstem auditory evoked potentials wave V's delay, and amplitude at the end of the decompression, or closing of the case were found between groups. CONCLUSIONS: Monitoring free-running electromyography of additional CNs V and VI does not improve the efficacy of IONM of microvascular decompressions for glossopharyngeal neuralgia.

Idiopathic cranial polyneuropathy with unilateral IX and X and contralateral XI nerve palsy in a 4-year-old boy.

A 4-year-old boy presented with a sudden onset of nasal escape of fluids, nasal speech, and difficulty placing his left arm through a sleeve. Neurologic examination indicated a unilateral cranial IX and X and contralateral XI nerve palsy that was considered idiopathic. Palsy of cranial nerves IX, X, and XI is rare in childhood, and few reports have described this condition. Our patient received prednisolone for 1 week and demonstrated complete recovery within several weeks. We suggest that aggressive therapy is unnecessary for patients with idiopathic cranial polyneuropathy. The pathogenesis of this condition may involve an immunologic mechanism.

Syncopal storm caused by glossopharyngeal neuralgia.

We report a rare case presenting with up to 50 episodes of syncopes per day in a 54-year-old man with the ultimate diagnosis of idiopathic glossopharyngeal neuralgia. All episodes were started with a severe pain sensation in the right side of the throat followed by asystole and then very slow ventricular escape beats. The patient was successfully treated with the combination of carbamazepine, gabapentin, and dual-chamber pacemaker implantation.

Paroxysmal neuralgia in pediatric population--a diagnostic dilemma for physicians and dental practioners.

Paroxysmal neuralgia is relatively uncommon in children. Neuropathic orofacial pain is a challenge for the clinician, as no obvious dental pathology exists either clinically or radiographically. Dentist and physician should be able to recognize the characteristics of neuropathic pain so as to correctly diagnose these conditions hence avoid unnecessary dental intervention. This article reviews the conditions with paroxysmal neuralgia in children and available treatment strategies.

The effect of degenerated neuron density of petrosal ganglion on the development of blood pressure variabilities after subarachnoid hemorrhage in a rabbit model: an experimental study.

AIM: The aim of this study was to determine the relationship between ischemic neurodegeneration, of the petrosal ganglion of the glossopharyngeal nerve, and BP fluctuations, after subarachnoid hemorrhage (SAH). MATERIAL AND METHODS: Twenty-four rabbits had their blood pressure and heart rhythms studied daily over 20 days. Then, the histopathology of the petrosal ganglion was examined in all animals. Normal and apoptotic neuron density of the petrosal ganglion and blood pressure values were compared statistically. RESULTS: Mean total volume of the petrosal ganglia was calculated as 0.9 ± 0.34/mm3. BP level of control group was 96.1 ± 2.1 mmHg; 116.5 ± 4 mmHg of mild hypertension (HT) group and 128.1 ± 3.6mmHg in the severe HT group. When the groups were compared to each other they were significantly different. The level of normal-apoptotic neuron in control group was 11,240 ± 802/mm³ -40 ± 6.3/mm³; 9730 ± 148.7/mm³ - 1560 ± 256.2/mm³ in the mild HT group and 6870 ± 378.8/mm³-4240 ± 628.2/mm³ in the severe HT group. When the groups were compared to each other there was significantly difference. CONCLUSION: Blood pressure variability observed in this study may be explained by ischemic neurodegeneration of petrosal ganglia caused by SAH. The results of this study suggest that petrosal ganglion ischemia has potential implications for the development of hypertension. These findings suggest that new treatment strategies should be considered for the treatment of SAH.

Cranial nerve vascular compression syndromes of the trigeminal, facial and vago-glossopharyngeal nerves: comparative anatomical study of the central myelin portion and transitional zone; correlations with incidences of corresponding hyperactive dysfunctional syndromes.

OBJECTIVE: The aim of this study was to evaluate the anatomy of the central myelin portion and the central myelin-peripheral myelin transitional zone of the trigeminal, facial, glossopharyngeal and vagus nerves from fresh cadavers. The aim was also to investigate the relationship between the length and volume of the central myelin portion of these nerves with the incidences of the corresponding cranial dysfunctional syndromes caused by their compression to provide some more insights for a better understanding of mechanisms. METHODS: The trigeminal, facial, glossopharyngeal and vagus nerves from six fresh cadavers were examined. The length of these nerves from the brainstem to the foramen that they exit were measured. Longitudinal sections were stained and photographed to make measurements. The diameters of the nerves where they exit/enter from/to brainstem, the diameters where the transitional zone begins, the distances to the most distal part of transitional zone from brainstem and depths of the transitional zones were measured. Most importantly, the volume of the central myelin portion of the nerves was calculated. Correlation between length and volume of the central myelin portion of these nerves and the incidences of the corresponding hyperactive dysfunctional syndromes as reported in the literature were studied. RESULTS: The distance of the most distal part of the transitional zone from the brainstem was 4.19 ± 0.81 mm for the trigeminal nerve, 2.86 ± 1.19 mm for the facial nerve, 1.51 ± 0.39 mm for the glossopharyngeal nerve, and 1.63 ± 1.15 mm for the vagus nerve. The volume of central myelin portion was 24.54 ± 9.82 mm(3) in trigeminal nerve; 4.43 ± 2.55 mm(3) in facial nerve; 1.55 ± 1.08 mm(3) in glossopharyngeal nerve; 2.56 ± 1.32 mm(3) in vagus nerve. Correlations (p < 0.001) have been found between the length or volume of central myelin portions of the trigeminal, facial, glossopharyngeal and vagus nerves and incidences of the corresponding diseases. CONCLUSION: At present it is rather well-established that primary trigeminal neuralgia, hemifacial spasm and vago-glossopharyngeal neuralgia have as one of the main causes a vascular compression. The strong correlations found between the lengths and volumes of the central myelin portions of the nerves and the incidences of the corresponding diseases is a plea for the role played by this anatomical region in the mechanism of these diseases.

Glossopharyngeal neuralgia treated with gamma knife radiosurgery.

BACKGROUND: Although gamma knife radiosurgery is an established treatment option for trigeminal neuralgia, its role in the management of glossopharyngeal neuralgia is unclear. We report a case of glosspharyngeal neuralgia treated effectively with gamma knife radiosurgery, review the literature, and discuss the rationale supporting dose and target selection. CASE DESCRIPTION: A 47-year-old woman presented with persistent lancinating pain to the left throat, which was refractory to medical therapy. She declined a microvascular decompression and instead chose stereotactic radiosurgery. Gamma knife radiosurgery to the glossopharyngeal nerve at the glossopharyngeal meatus was used, and a maximum dose 80 Gy was delivered. She was pain-free off medications 1 month after the procedure and remains pain-free 11 months. There were no adverse neurologic effects attributable to the procedure. CONCLUSIONS: This clinical response provides encouraging evidence for the treatment of glossopharyngeal neuralgia with stereotactic radiosurgery and is consistent with previous reports. Further investigation is needed to define the role of stereotactic radiosurgery in the management of glossopharyngeal neuralgia.

Neuralgia of the glossopharyngeal and vagal nerves: long-term outcome following surgical treatment and literature review.

This study describes our experience in the surgical treatment of neuralgia of the glossopharyngeal and vagal nerves. Over the last 19 years, 21 patients underwent surgery. Their case notes were reviewed to obtain demographic information, clinical presentation, surgical findings and early results. All patients were then contacted by telephone for long-term results and complications. Independent analysis of results was carried out by a Neurology team. Ten patients had microvascular decompression (MVD). Four patients had MVD and nerve section. In the remaining seven patients, the glossopharyngeal and first two rootlets of the vagal nerve were sectioned. Nineteen (90%) of 21 patients experienced complete relief of pain immediately after surgery. The remaining patients reported an improvement in their symptoms. There were no mortalities. Four patients experienced short-term complications, which resolved. Two patients were left with a persistent hoarse voice. At follow-up (mean duration of 4 years), there was no recurrence in symptoms. In our experience, surgery is safe and effective for the treatment of vago-glossopharyngeal neuralgia.

Microvascular decompression for glossopharyngeal neuralgia through the transcondylar fossa (supracondylar transjugular tubercle) approach.

OBJECTIVE: Our surgical results were reviewed to clarify the cause of glossopharyngeal neuralgia (GPN) and the effects of the microvascular decompression (MVD) procedure. METHODS: Fourteen cases of idiopathic GPN were operated on through the transcondylar fossa (supracondylar transjugular tubercle) approach. Their clinical data and operative records were retrospectively reviewed. RESULTS: In every case, vascular compression on the glossopharyngeal nerve was found and MVD was performed without any major complications. In 13 of the 14 cases the neuralgia completely disappeared postoperatively. Recurrence of pain was found in 1 case. Offending vessels were the posterior inferior cerebellar artery (PICA) in 10 cases, the anterior inferior cerebellar artery (AICA) in 2 cases, and both arteries in 2 cases. In 10 of the 14 cases, the high-origin PICA formed an upward loop between the glossopharyngeal and vagus nerves, compressing the glossopharyngeal nerve upward. In those cases, the PICA was transposed and fixed to the dura mater by the stitched sling retraction technique, and MVD was very effective. CONCLUSION: The offending artery was the PICA in most cases. MVD is expected to be very effective, especially when the radiological images show the following 3 findings: 1) high-origin PICA, 2) the PICA making an upward loop, and 3) the PICA coursing the supraolivary fossette. The transcondylar fossa approach is suitable for transposing the PICA by the stitched sling retraction technique, and provides sufficient surgical results.