Pediatric intracranial lower cranial nerve schwannoma unassociated with neurofibromatosis type 2: case report and review of the literature.

INTRODUCTION: Pediatric schwannomas are rare, and most of them are associated with neurofibromatosis type 2 (NF2) and usually located in the vestibular nerve. Herein, we present the first pediatric case of intracranial schwannoma derived from the IX/X nerve complex unrelated to NF2. CASE REPORT: The patient was a 9-year-old boy who presented with a 3-month history of headache and nausea. There was no family history of NF2. Imaging studies revealed a cystic lesion with enhanced wall in the left cerebellomedullary fissure. During the operation, the IX/X nerve complex was strongly adhered to the tumor at the jugular foramen. The tumor was totally excised, and the postoperative MRI demonstrated no residual tumor. Histopathological diagnosis was schwannoma. Genetic analysis revealed no mutation associated with NF2 and schwannomatosis. CONCLUSION: We reported the first case of pediatric lower cranial nerve schwannoma which was not associated with NF2. The schwannoma should be included as differential diagnosis of pediatric posterior fossa tumors.

Cofosis unilateral asociada a un neurinoma del glosofaríngeo / Unilateral deafness associated with a glossopharyngeal neurinoma

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Revisión morfológica, clínica y quirúrgica sobre el nervio glosofaríngeo / Morphological, clinical and surgical review on the glosopharyngeal nerve

En 1910, Weisenberg describió por primera vez la neuralgia del nervio glosofaríngeo (NGF), en un joven con diagnóstico de neuralgia del trigémino y sometido a múltiples intervenciones quirúrigcas, llegando a la sección del ganglio del trigémino. Algunas investigaciones han abordado la relación entre el nervio glosofaríngeo y las estructuras circundantes, desde su origen en el tronco encefálico y su trayecto a través de la base de cráneo. La examinación imagenológica de la interface entre la base de cráneo y los tejidos blandos de la región es compleja, ya que esas estructuras en la base del cráneo presentan características radiológicas de gran penetrancia y además pueden ser fácilmente dañadas o alteradas durante la disección anatómica o la cirugía. Si bien la NGF es un sindrome facial infrecuente, constituye una enfermedad neurológica de importancia por el gran sufrimiento que ocasiona, y en algunos casos, por los serios problemas que pueden amenazar la vida del paciente, debido a la posibilidad de aparición de sincopes y arritmias cardíacas. El objetivo de este trabajo consiste en realizar una revisión biobliográfica de la morfología del nervio glosofaríngeo, sus relaciones vasculares, y la importancia de su conocimiento en la clínica y la cirugía.

In 1910, Weisenberg first described nephropathy of the glossopharyngeal nerve (NGF) in a young man diagnosed with trigeminal neuralgia and undergoing multiple surgical interventions, reaching the trigeminal ganglion section. Some research has addressed the relationship between the glossopharyngeal nerve and the surrounding structures, from its origin in the brainstem and its path through the skull base. Imaging of the interface between the skull base and the soft tissues of the region is complex, since these structures at the base of the skull exhibit radiological characteristics of great penetrance and can be easily damaged or altered during anatomical dissection or surgery. Although NGF is an uncommon facial syndrome, it is a major neurological disease due to the great suffering it causes, and in some cases, serious problems that may threaten the patient's life, due to the possibility of syncopes and arrhythmias Cardiac disorders. The objective of this work is to perform a biobliographic review of the glossopharyngeal nerve morphology, its vascular relationships, and the importance of its knowledge in clinical and surgery.

Glossopharyngeal neuralgia and radiosurgery.

OBJECT: Glossopharyngeal neuralgia is difficult to treat. On the basis of results obtained by using Gamma Knife surgery (GKS) to treat trigeminal neuralgia, the authors have used GKS to treat glossopharyngeal neuralgia in a series of patients since 2007. Their objectives with this study were to demonstrate the usefulness and safety of GKS for treating glossopharyngeal neuralgia and to describe a simple treatment method. METHODS: From 2007 through 2013, the authors treated glossopharyngeal neuralgia in 5 patients (4 women and 1 man), who ranged in age from 36 to 74 years. One patient had previously undergone treatment for trigeminal neuralgia at the Ruber International Hospital, Department of Functional Neurosurgery and Gamma Knife Radiosurgery. For all patients, before GKS, medical management did not control the pain. Three patients had previously undergone surgery (2 microvascular decompression and 1 rhizotomy) without improvement. For the GKS procedure, the nerve was localized by MRI and CT under stereotactic conditions and the target was located at the level of the glossopharyngeal meatus of the jugular foramen. For 1 patient, a maximum dose of 80 Gy was administrated with a 4-mm collimator, and for the others, the maximum dose was 90 Gy. The nerves located near the glossopharyngeal nerve received between 63 and 10 Gy, and the brainstem received less than 10 Gy. The mean follow-up time was 43 months (range 14-83 months). RESULTS: All patients improved within 3-6 months after undergoing GKS. All 5 are without pain; 3 patients take no medication, but the other 2 patients continue to take medication. No neurological deficits after GKS were observed. CONCLUSIONS: GKS is useful and safe for treating glossopharyngeal neuralgia, even for patients who have previously undergone surgery. GKS should be considered as the initial therapy for glossopharyngeal neuralgia.

Exposure of wide cerebellomedullary cisterns for vascular lesion surgeries in cerebellomedullary cisterns: opening of unilateral cerebellomedullary fissures combined with lateral foramen magnum approach.

OBJECTIVE: To clarify microsurgical anatomic features of the cerebellomedullary fissure (CMF), the natural cleavage plane between the cerebellum and the medulla, and its relationship to the cerebellomedullary cistern (CMC) and to describe a surgical technique that uses the unilateral trans-CMF approach for CMC surgeries. METHODS: In the anatomic study, 2 formalin-fixed cadaver heads were used. In the clinical study, 3 patients with vertebral artery-posterior inferior cerebellar artery aneurysms and 3 patients with glossopharyngeal neuralgia were surgically treated through the unilateral trans-CMF approach combined with the transcondylar fossa approach, which is a lateral foramen magnum approach. RESULTS: The CMC was present at the lateral end of the CMF. The CMF was closed by arachnoidal adhesion, and the cerebellar hemisphere was superiorly attached to the cerebellar peduncle. After the unilateral CMF was completely opened, the cerebellar hemisphere was easily retracted rostrodorsally. Clinically, almost completely opening the unilateral CMF markedly enabled the retraction of the biventral lobule to obtain a wide surgical field safely for vascular CMC lesions. We present 2 representative cases. CONCLUSION: Combined unilateral trans-CMF/lateral foramen magnum approaches provide a wide and close surgical field in the CMC, allowing easy and safe CMC surgery.

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.

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.

[Endoscopic assistance in microvascular decompression of cranial nerves].

Microvascular decompression (MVD) is an effective method for treatment of trigeminal neuralgia (TN), hemifacial spasm (HFS), glossopharyngeal neuralgia (GPN). The aim of this study was to assess the role of endoscopic assistance in MVD for the treatment of cranial neuropathies. Since 2009 till 2011 133 patients with cranial neuropathies were treated by MVD in Burdenko Neurosurgical Institute, Moscow. In 22 patients (11 patients with HFS, 10 patients with TN, 1 with GPN) endoscopic assistance was applied during the MVD. We used minimally invasive retrosigmoid approach in a unilateral position. Cerebellopontine angle was explored by 30-degree or 70-degree telescope to visualize the root entry zone of trigeminal, facial or glossopharyngeal nerves and to locate the neurovascular conflict. In 9 patients with HFS and in 1 patient with TN and in another patient with GPN endoscopy discovered offending vessels that were not visible through the microscope. In all cases endoscope was used to exclude another site of compression and to verify decompression and to identify position of teflon and offending vessel after MVD. Immediately after the surgery excellent outcome was observed in 10 patients with HFS (89%), one patient was reoperated 1.5 years after first operation with positive effect. Relief of pain in early postoperative period was observed in patients with TN and GPN. There were no major complications and postoperative mortality in our series. Endoscopic assistance is very effective and helpful technique in MVD of cranial nerves, especially in cases with HFS. In this study the use of the endoscope allowed to identify the site of compression and to confirm the position of teflon after MVD.

High-resolution 3D-constructive interference in steady-state MR imaging and 3D time-of-flight MR angiography in neurovascular compression: a comparison between 3T and 1.5T.

BACKGROUND AND PURPOSE: High-resolution MR imaging is useful for diagnosis and preoperative planning in patients with NVC. Because high-field MR imaging promises higher SNR and resolution, the aim of this study was to determine the value of high-resolution 3D-CISS and 3D-TOF MRA at 3T compared with 1.5T in patients with NVC. MATERIALS AND METHODS: Forty-seven patients with NVC, trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia were examined at 1.5T and 3T, including high-resolution 3D-CISS and 3D-TOF MRA sequences. Delineation of anatomic structures, overall image quality, severity of artifacts, visibility of NVC, and assessment of the SNR and CNR were compared between field strengths. RESULTS: SNR and CNR were significantly higher at 3T (P < .001). Significantly better anatomic conspicuity, including delineation of CNs, nerve branches, and assessment of small vessels, was obtained at 3T (P < .02). Severity of artifacts was significantly lower at 3T (P < .001). Consequently, overall image quality was significantly higher at 3T. NVC was significantly better delineated at 3T (P < .001). Six patients in whom NVC was not with certainty identifiable at 1.5T were correctly diagnosed at 3T. CONCLUSIONS: Patients with NVC may benefit from the higher resolution and greater sensitivity of 3T for preoperative assessment of NVC, and 3T may be of particular value when 1.5T is equivocal.

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.

Isolated paralysis of glossopharyngeal and vagus nerve associated with type II occipital condyle fracture: case report.

INTRODUCTION: Occipital condyle fractures (OCFs) can occasionally be complicated with lower cranial nerve palsies. DISCUSSION: Isolated 9th and 10th cranial nerve palsies following OCF are very rare. To our knowledge, we report the first case of an early onset of 9th and 10th cranial nerve palsies with an early full recovery in short period of time and discuss the probable mechanism of isolated nerve palsy in the light of the relevant literature.

Glossopharyngeal neuralgia as onset of multiple sclerosis.

OBJECTIVES AND METHODS: Glossopharyngeal neuralgia is a painful condition, affecting the ninth cranial nerve, rarely described in the course of multiple sclerosis. Here we describe a case of multiple sclerosis presenting with glossopharyngeal neuralgia. RESULTS AND DISCUSSION: We suggest the presence of demyelinating areas at the nerve root entry zone as principal trigger mechanism.

Microvascular decompression for glossopharyngeal neuralgia: a long-term retrospectic review of the Milan-Bologna experience in 31 consecutive cases.

OBJECTIVE: To examine surgical findings and results of microvascular decompression (MVD) for glossopharyngeal neuralgia (GN). METHODS: Between 1990 and 2007, 31 consecutive patients affected by drug-resistant GN underwent MVD through a retromastoid keyhole in the supine position with the head rotated to the opposite side. A retrospective analysis was performed that paid particular attention to the relationship among surgical technique, pain control and side effects. RESULTS: A vascular compression of the glossopharyngeal nerve was found in all cases. Twenty-eight out of 31 patients (90.3%) were found to be pain free without medication at long-term follow-up (1-17 years, mean 7.5 years). Three patients (9.7%) were found to require medication to control pain paroxysms that were less frequent and less severe than those observed preoperatively. Two patients required repeated surgery for a drug-resistant recurrence of pain for a total of 33 MVDs. We observed no mortality and did not find any long-term surgical morbidity. Cranial nerve impairment, when observed, always resolved in the following months. CONCLUSIONS: MVD is a safe and effective treatment for GN in patients of all ages.

[Neurosurgical treatment of vago-glossopharyngeal neuralgia]. / Traitement neurochirurgical de la névralgie vago-glossopharyngienne.

Glossopharyngeal neuralgia, more accurately called vago-glossopharyngeal neuralgia (VGPN) because of the frequent association with pain irradiation in the sensory territory of the vagus nerve, is not always recognized because its incidence is much lower than the incidence of trigeminal neuralgia (100 times more frequent). As in trigeminal neuralgia, when pain becomes resistant to anticonvulsants - its specific medical treatment - VGPN can almost always be cured by surgery. The first option is microvascular decompression, since vascular compression is the main cause of the neuralgia. Percutaneous thermorhizotomy at the foramen jugularis (pars nervosa) is only indicated as a second option, because of unavoidable sensorimotor deficits in the ninth and tenth nerves. Tractonucleotomies at the medullary level should be reserved essentially for pain of malignant origin.

Eagle syndrome: case report and review of the literature.

Eagle syndrome, which is an uncommon sequela of elongation of the styloid process, can manifest as pain in the anterolateral neck, often with referred pain to the ear. In most cases, the elongation is an acquired condition, often occurring as a result of a traumatic incident, including tonsillectomy. We describe the case of a 57-year-old man who experienced unremitting right neck pain for several years following an accidental fall. A multidisciplinary investigation identified an elongated styloid process. Surgical shortening of the structure provided definitive relief of the patient's symptoms. We review the anatomy of the peristyloid structures and discuss the etiology, diagnosis, and treatment of Eagle syndrome.

Vagolysis.

We describe the case of a 75-year-old man presenting with labile hypertension and symptomatic postural hypotension 13 months following radiotherapy for squamous cell carcinoma of his external auditory canal. Magnetic resonance image (MRI) scan demonstrated scarring and a probable recurrence of his tumour. He underwent autonomic testing, including muscle sympathetic nerve activity (MSNA), heart rate (HR) and blood pressure (BP) responses to a variety of stimuli. Results were consistent with baroreflex failure. Urinary catecholamine levels were within the high normal range. We postulate that baroreflex failure was caused by vagal and glossopharyngeal nerve damage secondary to radiotherapy and tumour recurrence. This diagnosis is rare, but should be considered with pure autonomic failure and phaeochromocytoma in the presence of labile hypertension, especially in patients with a history of radiotherapy to the neck and high-normal catecholamine levels.