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.

[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.

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.

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.

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.

A case of Vernet syndrome with varicella zoster virus infection.

A 40-year-old man was admitted to our department, because of sudden onset of dysphagia, hoarseness, left neck pain and headache. There were no skin lesions. On neurological examination, there were paralysis of the left soft palate and constrictor muscles of the pharynx, weakness of the left sternocleidomastoid and left upper trapezius. In cerebrospinal fluid (CSF) examination, cell count and protein concentration were elevated. Antibody titer to varicella zoster virus (VZV) was elevated in both the serum and CSF. And VZV-DNA was detected by PCR from CSF. Gd enhanced MRI showed the nodular lesion at the left jugular foramen. The diagnosis of Vernet's syndrome (VS) associated with VZV infection was made. The patient's symptoms were immediately improved with 30 mg of prednisone and 3 g of varaciclovir daily for 14 days. Only a few cases of VS due to VZV have been reported previously. Our case is the first case that detected VZV-DNA in CSF by PCR.

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.

Mixed cranial nerve neuroma revealing itself as baroreflex failure.

We report here the first case of baroreflex failure due to a mixed cranial nerve neuroma in which the clinical manifestations (recurrent severe hypertensive crisis, hypotension) due to baroreflex arc impairment preceded the clinical diagnosis of brain tumour and neurosurgery by a few months. Given the clinical suspicion of baroreflex failure, even in the absence of iatrogenic clues, we propose that the patient's study should include neuroradiologic evaluation of the ponto-cerebellar angulus.

Chiari Type I malformation presenting as glossopharyngeal neuralgia: case report.

OBJECTIVE AND IMPORTANCE: Chiari Type I malformation is an important pathological state in which the brainstem is compressed by the cerebellar tonsil. We present a case of glossopharyngeal neuralgia caused by Chiari Type I malformation. CLINICAL PRESENTATION: A 50-year-old male patient was admitted with glossopharyngeal neuralgia. Magnetic resonance imaging studies revealed caudal displacement of the left cerebellar tonsil. INTERVENTION: Small occipital craniectomy and C1 laminectomy were performed. The left cerebellar tonsil was resected. CONCLUSION: This glossopharyngeal neuralgia was caused by compression of the lower cranial nerves and brainstem by the displaced left cerebellar tonsil. Decompression and pain relief were obtained with resection of the cerebellar tonsil. The patient was pain-free 30 weeks after the operation.

Cranial root injury in glossopharyngeal neuralgia: electron microscopic observations. Case report.

Optical and electron microscopic examinations were made of a biopsy sample of the ninth and 10th cranial nerves obtained during posterior fossa surgery for the relief of pain in a patient suffering from glossopharyngeal neuralgia (GN). Pathological findings, which were restricted to a small fraction of fascicles in the nerves, included large patches of demyelinated axons in close membrane-to-membrane apposition to one another and zones of less severe myelin damage (dysmyelination). These observations, in the light of similar morphological changes observed in biopsy samples excised from patients with trigeminal neuralgia, and new information on the pathophysiological characteristics of injured peripheral nerve axons, can account for much of the symptomatology of GN.

Simple cysts of the cerebellum.

Four cases of simple cyst of the cerebellum treated at Stanford University Medical Center are presented. Review of the literature and past experience at Stanford suggest that they are uncommon. They seem to occur most often in middle age, presenting the signs and symptoms of an expanding cerebellar mass, and appear to be adequately treated by unroofing and draining the cyst cavity. It is suggested that these cysts do not have a congenital origin but rather represent a degenerated form of cerebellar astrocytoma.

The microsurgical anatomy of the glossopharyngeal nerve with respect to the jugular foramen lesions.

Removal of lesions involving the jugular foramen region requires detailed knowledge of the anatomy and anatomical landmarks of the related area, especially the lower cranial nerves. The glossopharyngeal nerve courses along the uppermost part of the jugular foramen and is well hidden in the deep layers of the neck, making this nerve is the most difficult one to identify during surgery. It may be involved in various pathological entities along its course. The glossopharyngeal nerve can also be compromised iatrogenically during the surgical treatment of such lesions. The authors define landmarks that can help identify this nerve during surgery and discuss the types of lesions that may involve each portion of the glossopharyngeal nerve.

Surgical treatment of dumbbell-shaped jugular foramen schwannomas.

OBJECT: Schwannomas of the jugular foramen are rare, comprising between 2 and 4% of intracranial schwannomas. The authors retrospectively analyzed their surgical experience with schwannomas of the lower cranial nerves that presented with intra- and extracranial extensions through an enlarged jugular foramen. The transcondylar suprajugular approach was used without sacrificing the labyrinth or the integrity of the jugular bulb. In this report the clinical and radiological features are discussed and complications are analyzed. METHODS: This retrospective study includes six patients (three women and three men, mean age 31.6 years) with dumbbell-shaped jugular foramen schwannomas that were surgically treated by the senior author during a 5.5-year period. One patient had undergone previous surgery elsewhere. Glossopharyngeal and vagal nerve deficits were the most common signs (appearing in all patients), followed by hypoglossal and accessory nerve deficits (66.6%). Two or more signs or symptoms were present in every patient. Three tumors presented with cystic degeneration. In four patients the jugular bulb was not patent on neuroimaging studies. The suprajugular approach was used in five patients; the origin of the tumor from the 10th cranial nerve could be defined in three of them. All lesions were completely resected. No death or additional postoperative cranial nerve deficits occurred in this series. Aspiration pneumonia developed in one patient. Preoperative deficits of the ninth and 10th cranial nerves improved in one third of the patients and half recovered mobility of the tongue. No recurrence was discovered during the mean follow-up period of 32.8 months. CONCLUSIONS: With careful, extensive preoperative evaluation and appropriate planning of the surgical approach, dumbbell-shaped jugular foramen schwannomas can be radically and safely resected without creating additional neurological deficits. Furthermore, recovery of function in the affected cranial nerves can be expected.

Jacobson's nerve schwannoma: a rare middle ear mass.

The case of a 55-year-old woman with a middle ear mass is presented. The preoperative diagnostic workup, including an audiogram and imaging studies, and the histopathologic findings of the tumor are reviewed. The tumor, a schwannoma, arose from Jacobson's nerve in the middle ear. The surgical anatomy of Jacobson's nerve and the surgical approach to this tumor and to other tumors of the middle ear space are discussed. Tumors of the tympanic cavity are rare, with the exception of cholesteatoma; otherwise, the most common among them are paraganglioma and facial nerve neuroma. This report represents the first documented case of a schannoma arising from Jacobson's nerve in the tympanic cavity.

Preoperative visualization of microvascular compression of cranial nerve IX using constructive interference in steady state magnetic resonance imaging in glossopharyngeal neuralgia.

We report a case of successfully treated glossopharyngeal neuralgia, in whom preoperative magnetic resonance imaging (MRI) with a sequence of constructive interference in steady-state (CISS) precisely delineated microvascular compression to the glossopharyngeal nerve at its cisternal portion. A 70-year old female experienced paroxysmal and excruciating pain, starting in the right ear and descending to the tongue and throat. The pain was brief, burning and triggered by swallowing food and drinks. Preoperative CISS-MRI delineated a small vascular loop compressing the glossopharyngeal nerve at its cisternal portion. The patient was diagnosed with glossopharyngeal neuralgia, and then, microvascular decompression surgery was performed. Intraoperative inspection revealed that the glossopharyngeal nerve root was in contact with a small arterial loop at its cisternal portion and was deformed as demonstrated by preoperative CISS-MRI. The glossopharyngeal nerve was displaced using a piece of Teflon felt, freeing it from the arterial loop compression. The patient's neuralgia resolved immediately after surgery. During a period of 2-years follow up, no recurrence of the neuralgia has occurred. The usefulness of CISS-MRI for the diagnosis of glossopharyngeal neuralgia is discussed.

The glossopharyngeal nerve, glossopharyngeal neuralgia and the Eagle's syndrome--current concepts and management.

Glossopharyngeal neuralgia is not just a painful condition. At times, it may be life-threatening as a result of associated cardiovascular consequences. Even in the absence of life-threatening consequences, it can be a severe debilitating disease with depression, suicidal tendencies, fear of swallowing, loss of weight and under-nutrition. The treatment for glossopharyngeal neuralgia and Eagle's syndrome has evolved over time. This review summarises the scientific evidence and philosophy about current management and therapy. Emphasis is placed on the importance of excluding secondary causes of glossopharyngeal neuralgia before embarking on nerve section through the posterior cranial fossa approach. The Eagle's syndrome due to an elongated styloid process is the most important cause of secondary glossopharyngeal neuralgia. Stylectomy is effective and should be considered before embarking on any neurosurgical procedure. Peripheral cervical and trans-tonsillar approaches to the glossopharyngeal nerve are also discussed.

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.