An uncommon case of neurosarcoidosis presenting as trigeminal neuralgia.

Sarcoidosis is a systemic inflammatory condition causing increased immune system activity and manifesting as noncaseating granulomatous disease with the ability to affect multiple organ systems. Neurosarcoidosis is an uncommon presentation, with just 5-10% of patients with sarcoidosis experiencing intracranial disease. The diagnosis of neurosarcoidosis can be difficult, especially given the overlap of imaging findings with more common intracranial lesions. This case presents trigeminal neuralgia as the initial symptom of neurosarcoidosis and emphasizes the importance of a high clinical index of suspicion for neurosarcoidosis in patients with otherwise unexplained neurological symptoms.

Endovascular treatment of ruptured tiny (⩽3mm) intracranial aneurysms in the setting of subarachnoid hemorrhage: A case series of 20 patients and literature review.

Successful endovascular coiling of ruptured tiny saccular intracranial aneurysms (⩽3mm) is technically challenging and traditionally has been associated with technical failures, as well as morbidity related to thromboembolic events and high intraoperative rupture rates. This study analyzes the feasibility, technical efficacy, and clinical outcomes of coil embolization of ruptured tiny intracranial aneurysms using current coil and microcatheter technology and techniques. We performed a retrospective review of 20 patients with 20 ruptured tiny aneurysms treated with endovascular coil embolization from 2013 to 2016 at a single high-volume academic tertiary care practice. The mean aneurysm size was 2.4mm (median 2.5mm, 1-3). Complete occlusion was achieved in 12 of 20 patients (60%), the remaining 7 of 20 patients (35%) had a small neck remnant, and there was 1 failure (5%) converted to microsurgical clipping. Two patients had a failed attempted surgical clip reconstruction and were subsequently coiled. There was 1 intraprocedural rupture (5%) and 1 severe parent artery vasospasm (5%) during coiling. At discharge, 60% of patients were living independently. At follow-up three patients were deceased. Mean angiographic follow-up was 139days (SD 120). There were no aneurysm recurrences among occluded patients and there were no retreatments among those with neck remnants. Coiling of ruptured aneurysms ⩽3mm is feasible with high occlusion rates and low complication rates. The availability of softer coils with flexible detachment zones has led to safe and effective endovascular treatment of tiny ruptured aneurysms.

Delayed Presentation of an Extracranial Internal Carotid Artery Pseudoaneurysm and Massive Epistaxis Secondary to a Nasal Foreign Body: Case Report and Review of the Literature.

BACKGROUND: Epistaxis is a very common medical condition and can often be controlled with conservative measures. Rarely, uncontrolled and life-threatening epistaxis can occur. CASE DESCRIPTION: We present the case of a 58-year-old man who developed delayed, massive epistaxis caused by an extracranial left internal carotid artery pseudoaneurysm caused by an intranasal foreign object without apparent recent trauma. The patient was successfully treated with endovascular stenting of the affected vessel segment. CONCLUSIONS: Massive epistaxis is a potentially lethal condition. Although the source uncommonly originates from the internal carotid artery, pseudoaneurysm rupture needs to be considered on the differential diagnosis in selected patients. This case illustrates the need for vigilance for the presence of foreign objects and/or vessel injuries in the setting of acute, massive epistaxis. Additionally, we describe treatment options and review the literature.

Management of intracranial pathology during pregnancy: Case example and review of management strategies.

BACKGROUND: Intracranial tumors during pregnancy are uncommon, and they present an interesting challenge to both the neurosurgeon and the obstetrician. Special considerations must be made in every aspect of care. The authors use the rare case of a 27-year-old pregnant female with suspected pineal region tumor eventually diagnosed as a thalamic region ganglioglioma to review the current literature on management of pathology in this unique patient population. CASE DESCRIPTION: A 27-year-old female who was 26 weeks pregnant presented to her obstetrician with complaints of headaches, blurriness of vision, and left-sided numbness and tingling. She was diagnosed with 1-cm mass in the pineal region and obstructive hydrocephalus. She initially underwent an endoscopic third ventriculostomy with biopsy of what appeared grossly to be a thalamic mass. The child was delivered via cesarean section at 39 weeks. Serial postpartum imaging demonstrated increasing tumor size and enhancement, which led the authors to proceed with subtotal resection via a supracerebellar infratentorial approach with stereotactic neuronavigation. Tissue specimens obtained for pathological analysis resulted in a revised diagnosis of World Health Organization (WHO) grade II ganglioglioma. CONCLUSIONS: Pregnancy presents a challenge for any patient requiring neurosurgical intervention. We present an interesting case example with a rare central nervous system neoplasm and discuss the management of intracranial pathology in pregnant patients.

Microsurgical Pontine Descending Tractotomy in Cases of Intractable Trigeminal Neuralgia.

BACKGROUND: Current treatment strategies in patients with trigeminal neuralgia (TN) include trials of medical therapy and surgical intervention, when necessary. In some patients, pain is not adequately managed with these existing strategies. OBJECTIVE: To present a novel technique, ventral pontine trigeminal tractotomy via retrosigmoid craniectomy, as an adjunct treatment in TN when there is no significant neurovascular compression. METHODS: We present a nonrandomized retrospective comparison between 50 patients who lacked clear or impressive arterial neurovascular compression of the trigeminal nerve as judged by preoperative magnetic resonance imaging and intraoperative observations. These patients had intractable TN unresponsive to previous treatment. Trigeminal tractotomy was performed either alone or in conjunction with microvascular decompression. Stereotactic neuronavigation was used during surgery to localize the descending tract via a ventral pontine approach for descending tractotomy. RESULTS: Follow-up was a mean of 44 months. At first follow-up, 80% of patients experienced complete relief of their pain, and 18% had partial relief. At the most recent follow-up, 74% of patients were considered a successful outcome. Only 1 (2%) patient had no relief after trigeminal tractotomy. Of those with multiple sclerosis-related TN, 87.5% experienced successful relief of pain at their latest follow-up. CONCLUSION: While patient selection is a significant challenge, this procedure represents an option for patients with TN who have absent or equivocal neurovascular compression, multiple sclerosis-related TN, or recurrent TN.

Bilateral ptosis as initial presentation of gliomatosis cerebri: case report.

Gliomatosis cerebri is a rare, diffuse glioma of neuroepithelial origin involving more than two cerebral lobes. Clinical presentation of gliomatosis cerebri is variable and depends on the degree, extent, and location of cortical involvement. Signs and symptoms related to supratentorial cortical involvement predominate and the diagnosis is reached through a combination of clinical, radiographic, and histopathological evaluations. This is a report of a young man who presented with visual problems and bilateral ptosis, which were eventually attributed to gliomatosis cerebri. Standard radiation and chemotherapy were administered but the patient eventually succumbed to the disease. The unique clinical presentation is discussed in light of this rare neoplasm of the central nervous system.

Cerebrospinal Fluid Leakage from Tegmen Tympani Defects Repaired via the Middle Cranial Fossa Approach.

Spontaneous cerebrospinal fluid (CSF) otorrhea due to tegmen tympani defects can result in hearing impairment and predispose to meningitis. Seizures or neurological deficits are additional risks, particularly when associated with an encephalocele. Surgical repair of the dural defect through a middle cranial fossa (MCF) approach is a treatment option under these circumstances. This series describes eight individuals who presented with CSF otorrhea and MCF encephaloceles associated with conductive hearing loss. Defects in the tegmen tympani were noted in all patients on preoperative cranial imaging, and six of the eight patients had an associated encephalocele. The average age was 57 years (range 26 to 67) with a male:female ratio of 7:1. Most defects occurred on the left side (6 left/2 right). A standard MCF approach and repair of the dural defect with an autologous dural graft (Durepair or DuraGen, Medtronic, Minneapolis, Minnesota, USA) and a synthetic polymer glue (DuraSeal, Covidien, Mansfield, Massachusetts) was performed in each case with universal success. Resolution of the CSF otorrhea was noted in all cases. All cases but one exhibited an improvement in hearing. One patient developed a delayed methicillin-resistant Staphylococcus aureus meningitis 3 months after surgery that resolved with surgical re-exploration and antibiotic therapy. Facial nerve monitoring was standard. All patients exhibited normal facial function postoperatively. Prophylactic lumbar drain placement was only utilized in the first three patients. The MCF approach is an excellent route to effectively repair CSF leaks and encephaloceles due to tegmen tympani and dural defects.

Hemifacial spasm in a patient with Marfan syndrome and Chiari I malformation. Case report.

Marfan syndrome, hemifacial spasm, and Chiari malformation are all relatively rare and seemingly separate entities. Marfan syndrome is caused by a defect in the gene that encodes fibrillin and leads to weakness of the artery wall. Hemifacial spasm results from compression of the facial nerve by an abnormal artery. Chiari malformation is characterized by a small posterior fossa. The authors report the case of a patient with Marfan syndrome who presented with hemifacial spasm and was also found to have a Chiari malformation Type I. The patient's Chiari malformation and hemifacial spasm were successfully treated by performing suboccipital and microvascular decompression surgeries, respectively. The pathophysiological characteristics of Marfan syndrome, hemifacial spasm, and Chiari malformation are discussed, and the authors propose a link between these conditions in this patient. The authors hypothesize that the patient's Marfan syndrome contributed to the abnormal shape of his vertebral artery and that, given the lack of space in his crowded posterior fossa due to the Chiari malformation, the artery caused compression of his facial nerve, resulting in hemifacial spasm.

Glucocorticoids induce neuroendocrine cell differentiation and increase expression of N-myc in N-type human neuroblastoma cells.

Human neuroblastoma cell lines comprise cellular counterparts of normal differentiation phenotypes arising from the developing neural crest Three distinct cell types have been isolated from cell lines: N-type cells with properties of embryonic sympathoadrenoblasts, S-type cells resembling nonneuronal Schwannian/glial/melanoblastic precursors, and I-type stem cells that can differentiate into either N- or S-type cells. Sympathoadrenoblasts from the normal neural crest further differentiate into neuronal or neuroendocrine cells. In this study, we show that malignant N-type neuroblasts likewise can differentiate futher along these same pathways. Retinoic acid and forskolin induce a neuronalphenotype, denoted morphologically by cell aggregation and increased neurite formation and biochemically by increases in neurofilament proteins, tyrosine hydroxylase, and secretogranin II and decrease inchromogranin A. By contrast, dexamethasone, a synthetic glucocorticoid, induces a chromaffin cell phenotype characterized by increased cell flattening, loss of neuritic processes, increased chromogranin A and tyrosine hydroxylase proteins, and decreased amounts of secretogranin II and neurofilaments. N-myc gene expression is upregulated by glucocorticoids; dexamethasone-treated N-type cells show significant (2.3- to 7.8-fold) increases in N-myc mRNA and protein steady-state levels. This effect is specific for glucocorticosteroids, is blocked by addition of the steroid receptor antagonist RU486, and involves direct activation of the N-myc promoter. These findings are the first to show that glucocorticoids upregulate N-myc expression in human neuroblastoma cells.