Successful surgical treatment of oculomotor palsy due to schwannoma of the cavernous sinus in a 7-year-old girl: a case report.

Oculomotor nerve schwannoma in children not associated with neurofibromatosis is a rare disease, with 26 pediatric cases reported so far. There is no established treatment plan. A 7-year-old girl presented with oculomotor nerve palsy. Surgical reduction of the tumor combined with postoperative gamma knife surgery preserved the oculomotor nerve, improved oculomotor nerve function, and achieved tumor control during the observation period of 20 months. The combination of partial surgical resection and gamma knife surgery as a treatment strategy for oculomotor nerve schwannoma resulted in a good outcome.

Cervical vagal nerve schwannoma induced arrhythmia: a rare case report and literature review.

BACKGROUND: Schwannomas are benign tumors deriving from the sheath of cranial and peripheral nerves. The vagus nerve is comprised of a complex neuro-endocrine-immune network that maintains homeostasis, most tracts of it play a role in parasympathetic activity. We present an example of a rare cervical vagal schwannoma case accompanied by arrhythmia. CASE PRESENTATION: A 35-year-old female patient with a left cervical vagus schwannoma and ventricular arrhythmia underwent schwannoma resection in the operating room. The patient's suppressed heart rate increased after tumor removal, and the cardiac rhythm returned to normal postoperatively. Pathological examination demonstrated the diagnosis of schwannoma. CONCLUSIONS: This case explains the link between the vagus nerve and the cardiovascular system, proving that a damaged cervical vagus nerve can inhibit the heart rate and lead to arrhythmias, and eventually requiring surgical intervention.

Recurrent Third Nerve Palsy Secondary to Instrinsic Schwannoma of the Third Cranial Nerve.

ABSTRACT: A 78 year-old woman has experienced multiple episodes of transient right third nerve palsy over the course of 15 years and has undergone multiple imaging studies as well as investigations for myasthenia gravis and giant cell arteritis in search for the diagnosis. When seen after the most recent episode, MRI with contrast and Fast-Imaging Employing Steady-State Acquisition protocol revealed a subtle enlargement and enhancement of the cisternal and proximal cavernous portions of the right third cranial nerve. An empiric diagnosis of schwannoma intrinsic to third cranial nerve was made. All patients with cyclical third nerve palsies should have appropriate neuroimaging to rule out subtle structural lesions before other investigations are undertaken.

Oculomotor Nerve Palsy Due to Unusual Causes.

BACKGROUND: An isolated oculomotor nerve (CN III) palsy is a diagnostic concern because of the potential for serious morbidity or life-threatening causes. We present 5 unusual causes of oculomotor nerve palsy that escaped initial diagnosis in order to raise awareness of their associated features that will facilitate correct diagnosis. METHODS: This study consisted of a retrospective analysis of clinical features and imaging of 5 patients who were referred for neuro-ophthalmologic evaluation with presumed diagnosis of oculomotor nerve palsy of unknown reasons. RESULTS: A complete CN III palsy and an inferior division CN III palsy were diagnosed with a schwannoma in the cavernous sinus and orbital apex portion, respectively; a middle-aged woman with aberrant regeneration was found to have a small meningioma; an adult man with ptosis was diagnosed with cyclic oculomotor paresis with spasms; and a patient after radiation was diagnosed with neuromyotonia. CONCLUSIONS: Localizing the lesion of oculomotor nerve palsy and careful examination of the imaging is crucial. Aberrant regeneration, cyclic pupil changes, and past medical history of amblyopia, strabismus, or radiation are also very helpful for diagnosis.

[A Case of Trochlear Nerve Neurinoma Presenting with Pathological Laughter].

Trochlear nerve neurinomas are rare and solitary tumors without neurofibromatosis are extremely rare. We report a case of trochlear nerve neurinoma presenting with pathological laughter and diplopia. A 40-year-old male patient presented with diplopia and pathological laughter 2 months before admission. MRI showed a multicystic enhanced mass in the left tentorial incisura compressing the midbrain and the upper pons. The tumor was excised using the left trans-Sylvian approach with partial uncal resection. After excision of the tumor, the left trochlear nerve was identified on the surface of the lateral midbrain. The nerve was connected to the tumor. Pathological laughter completely resolved after the operation. This is the second reported case of trochlear nerve neurinoma presenting with pathological laughter. The lesion responsible for pathological laughter could be the midbrain, upper pons, diencephalon, or all of these.

Rapidly Growing and Asymptomatic Skull Base Lesion.

Trochlear schwannomas typically present with diplopia and are extremely rare benign intracranial neoplasms that require the combined expertise of neurotologists and neurosurgeons for surgical excision. A combined petrosal approach offers a surgical route for removal of growing and/or symptomatic tumors. A study of a trochlear nerve schwannoma that demonstrated rapid growth but was otherwise asymptomatic was presented in this study.

Schwannoma of the trochlear nerve-an illustrated case series and a systematic review of management.

Schwannomas of cranial nerves in the absence of systemic neurofibromatosis are relatively rare. Among these, schwannomas of the trochlear nerve are even less common. They can be found incidentally or when they cause diplopia or other significant neurological deficits. Treatment options include observation only, neuro-ophthalmological intervention, and/or neurosurgical management via resection or sterotactic radiosurgery (SRS). In recent years, the latter has become an attractive therapeutic tool for a number of benign skull base neoplasm including a small number of reports on its successful use for trochlear Schwannomas. However, no treatment algorithm for the management of these tumors has been proposed so far. The goal of this manuscript is to illustrate a case series of this rare entity and to suggest a rational treatment algorithm for trochlear schwannomas, based on our institutional experience of recent cases, and a pertinent review of the literature. Including our series of 5 cases, a total of 85 cases reporting on the management of trochlear schwannomas have been published. Of those reported, less than half (40 %) of patients underwent surgical resection, whereas the remainder were managed conservatively or with SRS. Seventy-six percent (65/85) of the entire cohort presented with diplopia, which was the solitary symptom in over half of the cases (n = 39). All patients who presented with symptoms other than diplopia or headaches as solitary symptoms underwent surgical resection. Patients in the non-surgical group were mostly male (M/F = 3.5:1), presented at an older age and had shorter mean diameter (4.6 vs. 30.4 mm, p < 0.0001) when compared to the surgical group. Twelve patients in the entire cohort were treated with SRS, none of whom had undergone surgical resection before or after radiation treatment. Trochlear schwannoma patients without systemic neurofibromatosis are rare and infrequently reported in the literature. Of those, patients harboring symptomatic trochlear Schwannomas do not form a single homogenous group, but fall into two rather distinct subgroups regarding demographics and clinical characteristics. Among those patients in need of intervention, open microsurgical resection as well as less invasive treatment options exist, which all aim at safe relief of symptoms and prevention of progression. Both open microsurgical removal as well as SRS can achieve good long-term local control. Consequently, a tailored multidisciplinary treatment algorithm, based on the individual presentation and tumor configuration, is proposed.

Intratemporal facial nerve schwannoma: clinical presentation and management.

Facial nerve schwannoma is the most common facial nerve tumor, but its therapeutic strategy remains debated. The aim of this study is to analyze the facial nerve function and the hearing outcomes after surgery or wait-and-scan policy in a facial nerve schwannoma series. A monocentric retrospective review of medical charts of patients followed for an intratemporal facial nerve schwannoma between 1988 and 2013 was performed. Twenty-two patients were included. Data were extracted pertaining to the following variables: patient demographics, tumor localization, clinical and imaging features, facial nerve function and hearing levels, and details of surgical intervention. The majority of tumors were located at the geniculate ganglion. Initial symptoms were mainly facial palsy and hearing loss. The average follow-up was 4.8 ± 4.5 years. Nineteen patients underwent surgery, and three patients were observed. After surgery, 11 patients had a stable or improved facial nerve function (57.9 %), and 8 patients had a worsened facial nerve function (42.1 %). Facial nerve function was in the majority of cases a HB grade III, depending on surgical strategy. No patient presented a postoperative HB grade V or VI. Regarding the hearing, it remained stable after surgery in 52.6 % of cases, and improved in 10.5 % of cases. Among monitored patients, facial nerve function and hearing remained stable. Surgery for facial nerve schwannoma is a safe and effective option in the treatment of these tumors.

Trochlear Nerve Schwannoma With Repeated Intratumoral Hemorrhage.

Trochlear nerve schwannoma is extremely rare, with only 35 pathologically confirmed patients being reported in the literature. Here, the authors report a patient of trochlear nerve schwannoma in the prepontine cistern manifesting as facial pain and double vision and presenting the image characteristics of repeated intratumoral hemorrhage, which has never been reported in the literature. Total tumor along with a portion of the trochlear nerve was removed by using a retrosigmoid approach. Facial pain disappeared after operation, and the diplopia remained. Follow-up studies have shown no tumor recurrence for 2 years and the simultaneous alleviation of diplopia. Information regarding the clinical presentation, radiological features and surgical outcomes of trochlear nerve schwannoma are discussed and reviewed in the paper.

MULTIPLE INTRACRANIAL SCHWANNOMAS: CASE REPORT.

Schwannomas are benign encapsulated tumors arising from the sheaths of peripheral nerves. They present as slowly enlarging solitary lumps, which may cause neurological defects. Multiple schwannomas in non-neurofi bromatosis type 2 patients are extremely rare. We report a case of a 60-year-old female patient, without any family history of neurofibromatosis or schwannomatosis, presented with trigeminal neuralgia and progressive facial nerve palsy. Magnetic resonance imaging revealed the presence of acoustic schwannoma involving facial nerve and trigeminal schwannoma of the cisternal part of the nerve involving gasserian ganglion (Meckel's cave). After gamma knife radiosurgery, trigeminal neuralgia was relieved completely with improvement of facial nerve palsy.

Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy: report of two cases and review of the literature.

BACKGROUND: In recurrent painful ophthalmoplegic neuropathy (RPON) that was previously termed as ophthalmoplegic migraine, enhancement of the ocular motor cranial nerves could be seen in the cisternal segment during the acute phase. However, various tumors involving the oculomotor nerve may mimic RPON. METHODS: We report two patients with MRI findings of oculomotor nerve schwannoma who initially presented with RPON, and found through the literature review five more patients with oculomotor nerve tumors that masqueraded as RPON. RESULTS: All patients showed an involvement of the oculomotor nerve. The radiological or pathological diagnosis included schwannoma in five, venous angioma in one, and neuromuscular harmatoma in another one. MRIs with gadolinium documented an enhancing nodule involving the cisternal portion of the oculomotor nerve in six of them, which was also observed on follow-up MRIs without an interval change. CONCLUSIONS: It should be recognized that an incomplete recovery may occur during future attacks in patients with otherwise uncomplicated RPON. Follow-up MRIs are required to detect tumors involving the ocular motor cranial nerves, especially in patients with suspected RPON when the recovery is incomplete.

Trigeminal neuralgia and facial pain imaging.

The trigeminal nerve or fifth cranial nerve has an extensive distribution in the head and face. It is the source for pain conduction and thereby is often implicated in a variety of disorders including inflammatory and neoplastic diseases. To determine the disease source, understanding the trigeminal nerve anatomy is essential, and further being able to image the trigeminal nerve provides insight into the location and type of pathology. The best approach to imaging is to consider the nerve in segments. The nerve segments may be divided into the brainstem, cisternal, Meckel's cave, cavernous sinus, and peripheral divisions. This review utilizes these segments to explore imaging options to help understand trigeminal neuralgia and pain in the trigeminal nerve distribution.

Cranial and spinal oligodendrogliomatosis: a case report and review of the literature.

INTRODUCTION: Oligodendrogliomatosis is a rarely reported entity in literature associated with poor prognosis in terms of length and quality of life. In this paper, we describe oligodendrogliomatosis in a 15-year-old male who initially presented with altered mental status due to diabetic ketoacidosis. CLINICAL PRESENTATION: He was refractory to temozolomide initially but demonstrated disease regression with radiotherapy (XRT). More recently, he has had disease recurrence, which was stabilized with temozolomide therapy for a period of time. CONCLUSION: Contrary to most reports in literature, our patient has had excellent quality of life since his initial diagnosis and continues to carry good prognosis. In addition to oligodendrogliomatosis, our patient also developed multiple intracranial cavernomas secondary to radiation therapy, which have remained stable and asymptomatic.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

BACKGROUND: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well. METHODS: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits. RESULTS: Schwannomas of the third, fourth, and fifth nerves resulted in fluctuating deficits of all 3 ocular motor nerves. Persistent nodular cranial nerve enhancement was present on sequential MRI studies. Several episodes of transient oculomotor (III) deficts were associated with headaches, mimicking ophthalmoplegic migraine. CONCLUSIONS: Cranial nerve schwannomas may result in relapsing and remitting cranial nerve symptoms.

Stripping surgery in facial nerve schwannomas with favorable facial nerve function.

OBJECTIVE: We report 18 cases of facial nerve schwannomas in which stripping surgery was attempted to preserve facial nerve integrity and favorable facial nerve function. METHODS: We attempted stripping surgery on 18 cases of facial nerve schwannomas. Postoperative facial nerve function was evaluated. RESULTS: Stripping surgery was successfully achieved in 11 cases, and facial nerve decompression was performed on the remaining 7 cases in which stripping surgery was impossible. Favorable facial nerve function was successfully maintained in all cases who underwent stripping surgery and 5 of 7 cases who underwent facial nerve decompression. CONCLUSIONS: It was possible to accomplish stripping surgery in most cases with favorable facial nerve function, which maintained good facial nerve function after total tumor removal.

Clinical characteristics and outcomes of intratemporal facial nerve neurofibromas.

UNLABELLED: This study aimed to present clinical features and outcomes of 10 patients with intratemporal facial nerve neurofibromas. METHODS: The ten patients underwent complete tumor removal, and nerve grafting was performed on 3 cases whose nerve integrity was sacrificed. They were followed up after surgery. RESULTS: All patients firstly complained of facial palsy, and progressive sensorineural hearing loss was also present in one case. Insidious onset of facial palsy was found in 6 of 10 cases (60.0%). There was multi-segment involvement in 5 of 10 cases (50.0%). During the follow-up of 4.2±1.5 ys (range, 2-6 ys), 1 of 6 cases who successfully maintained nerve integrity recovered to Grade III, and the others remained or recovered to Grade V or even Grade VI. In contrast, all of the 3 cases with nerve grafting recovered from Grade VI to Grade III or Grade IV. No tumor recurrence was noted. CONCLUSIONS: Facial nerve neurofibromas mainly presented with insidious facial palsy, and tended to affect multiple segments of facial nerve. It seemed that complete tumor removal and nerve grafting had better outcomes of facial nerve compared to complete tumor removal with nerve integrity preserved.

Surgical outcomes of facial nerve hemangiomas in temporal bones.

OBJECTIVE: To report surgical outcomes of 15 cases who had facial nerve hemangiomas. METHODS: All cases underwent complete removal of hemangiomas, and preservation of nerve integrity was attempted. The postoperative outcomes of facial nerve was assessed. RESULTS: Nerve integrity was successfully preserved in 10 cases, all of which maintained or recovered to Grade I or Grade II, and facial nerve was sacrificed in 5 cases, who recovered to Grade III or Grade IV. CONCLUSIONS: When possible, facial nerve preservation should be attempted, which was critical to yield better outcomes of facial nerve.

Spontaneous regression of a cystic hypoglossal schwannoma causing unilateral tongue atrophy.

A 60-year-old lady presented with intermittent headaches. Examination revealed striking marked unilateral tongue atrophy. Magnetic resonance imaging (MRI) revealed a cystic lesion in the hypoglossal canal and a provisional diagnosis of cystic hypoglossal schwannoma made. Annual surveillance scans showed stable appearances but surprisingly at 3 years they showed a significant reduction in the size of the lesion. Most patients with hypoglossal schwannomas present with ipsilateral hypoglossal nerve palsy; careful cranial nerve examination is vital in diagnosing such rare lesions. Little is known of their natural history, with most lesions undergoing surgery. This case highlights spontaneous regression following non-operative management.