Astroblastoma With a Novel YAP1::BEND2 Fusion: A Case Report.

In the most recent fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System, astroblastoma has been defined by molecular rearrangements involving the MN1 gene, with common partners being BEND2 or CXXC5 . Accordingly, this tumor entity is now known as "astroblastoma, MN1 -altered." However, gliomas with EWSR1::BEND2 fusions, devoid of MN1 fusion alterations, have recently been shown to exhibit astroblastoma-like histomorphologic features and reside in a distinct epigenetic subgroup based on DNA methylation studies similar to high-grade neuroepithelial tumor with MN1 alteration, which includes astroblastoma, MN1 altered tumors. This new epigenetically distinct subtype of astroblastoma containing EWSR1::BEND2 fusions lacks the required MN1 alteration and, thus, does not satisfy the current molecular classification of these lesions. Here, we describe a case of glioma with histologic features and DNA methylation profiling consistent with astroblastoma with a novel YAP1: : BEND2 fusion. This case and others further expand the molecular findings observable in astroblastoma-like tumors outside the constraints of MN1 alteration. Such cases of astroblastoma with EWSR1::BEND2 and YAP1::BEND2 fusions challenge the current molecular classification of astroblastoma based solely on an MN1 alteration.

Early experience with percutaneous balloon kyphoplasty for treatment of osteoporotic vertebral compression fracture in Nepal: A case report.

Key Clinical Message: Balloon kyphoplasty is a promising treatment option for osteoporotic vertebral compression fractures with posterior cortical defect, offering pain relief, vertebral height restoration, and low risk of cement leakage. Abstract: Millions of people worldwide suffer from osteoporotic vertebral compression fractures (OVCFs) annually, which cause pain and functional limitations, particularly in the elderly. Conservative treatments such as pain management, rest, and medication are frequently used, while surgical options such as vertebroplasty and kyphoplasty are considered. We present a case of 68-year-old female with vertebral compression fracture of L1 vertebra with posterior cortical defect and posterior wall retropulsion. She was treated successfully with balloon kyphoplasty. Kyphoplasty appears to be a better option than vertebroplasty in cases with posterior cortical defect due to lower chance of cement leakage.

Monocyte Count on Admission Is Predictive of Shunt-Dependent Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage.

The authors sought to evaluate whether immunologic counts on admission were associated with shunt-dependent hydrocephalus following aneurysmal subarachnoid hemorrhage. A retrospective analysis of 143 consecutive patients with aneurysmal subarachnoid hemorrhage over a 9-year period was performed. A stepwise algorithm was followed for external ventricular drain weaning and determining the necessity of shunt placement. Data were compared between patients with and without shunt-dependent hydrocephalus. Overall, 11.19% of the cohort developed shunt-dependent hydrocephalus. On multivariate logistic regression analysis, acute hydrocephalus (OR: 61.027, 95% CI: 3.890-957.327; p = 0.003) and monocyte count on admission (OR: 3.362, 95% CI: 1.024-11.037; p = 0.046) were found to be independent predictors for shunt dependence. Receiver operating characteristic curve analysis for the prediction of shunt-dependent hydrocephalus confirmed that monocyte count exhibited an acceptable area under the curve (AUC = 0.737, 95% CI: 0.601-0.872; p < 0.001). The best predictive cutoff value to discriminate between successful external ventricular drain weaning and shunt-dependent hydrocephalus was identified as a monocyte count ≥0.80 × 103/uL at initial presentation. These preliminary data demonstrate that a monocyte count ≥0.80 × 103/uL at admission predicts shunt-dependent hydrocephalus in patients with aneurysmal subarachnoid hemorrhage; however, further large-scale prospective trials and validation are necessary to confirm these findings.

Successful Treatment of a Balamuthia mandrillaris Cerebral Abscess in a Pediatric Patient With Complete Surgical Resection and Antimicrobial Therapy.

Cerebral amebic encephalitis due to Balamuthia mandrillaris is a rare yet typically fatal disease. As such, identification of the clinical characteristics, appropriate diagnostic workup and commencement of treatment is frequently delayed. Here, we present a case of a 4-year-old male with a B. mandrillaris cerebral abscess successfully treated with expedited neurosurgical resection and broad-spectrum antimicrobial therapy.

Neutrophil Count on Admission Predicts Acute Symptomatic Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage.

OBJECTIVE: To investigate the association between immunologic counts on admission and acute symptomatic hydrocephalus after aneurysmal subarachnoid hemorrhage (aSAH). METHODS: We conducted a retrospective analysis of 143 consecutive patients with aSAH. Patient demographics, clinical parameters, laboratory values, and radiographic imaging were obtained. Univariate and multivariate logistic regression analyses were performed to investigate parameters independently associated with acute symptomatic hydrocephalus. Receiver operating characteristic (ROC) curve analysis determined the best threshold value of neutrophil count to differentiate patients with and without hydrocephalus. RESULTS: Overall, acute symptomatic hydrocephalus developed in 39.16% of patients. In an adjusted multivariate logistic regression model, Hunt and Hess grade 4-5 (odds ratio [OR]: 16.052, 95% confidence interval [CI]: 1.188-216.983; P = 0.037), modified Fisher score 3-4 (OR: 10.107, 95% CI: 1.715-59.572; P = 0.011), intraventricular hemorrhage (OR: 4.578, 95% CI: 1.417-14.788; P = 0.011), neutrophil count (OR: 1.183, 95% CI: 1.033-1.354; P = 0.015), and prior ischemic stroke (OR: 7.003, 95% CI: 1.293-37.929; P = 0.024) were significantly associated with hydrocephalus. ROC analysis for neutrophil count confirmed an acceptable area under the curve (AUC 0.780, 95% CI: 0.701-0.859; P < 0.001). The best threshold value of neutrophil count to predict hydrocephalus was ≥9.80 × 103/mL. Overall, 81.25% of patients who developed shunt dependence had a neutrophil count ≥9.80 × 103/mL on admission (P = 0.003). CONCLUSIONS: Neutrophil count ≥9.80 × 103/mL on admission predicts acute symptomatic hydrocephalus after aSAH in an adjusted multivariate logistic regression model. Moreover, shunt dependence was associated with higher neutrophil counts.

Idiopathic fourth ventricular outlet obstruction misdiagnosed as normal pressure hydrocephalus: A cautionary case.

BACKGROUND: Fourth ventricular outlet obstruction is an infrequent but well-established cause of tetraventricular hydrocephalus characterized by marked dilatation of the ventricular system with ballooning of the foramina of Monro, Magendie, and Luschka. Multiple processes including inflammation, infection, hemorrhage, neoplasms, or congenital malformations are known to cause this pathological obstruction. However, true idiopathic fourth ventricular outlet obstruction is a rare phenomenon with only a limited number of cases reported in the literature. CASE DESCRIPTION: A 61-year-old female presented with several months of unsteady gait, intermittent headaches, confusion, and episodes of urinary incontinence. Conventional magnetic resonance imaging demonstrated tetraventricular hydrocephalus without transependymal flow, but with ventral displacement of the brainstem and dorsal displacement of the cerebellum without an obvious obstructive lesion on pre- or post-contrast imaging prompting a diagnosis of normal pressure hydrocephalus. However, constructive interference in steady state (CISS) and half-Fourier acquisition single-shot turbo spin echo (HASTE) sequences followed by fluoroscopic dynamic cisternography suggested encystment of the fourth ventricle with thin margins of arachnoid membrane extending through the foramina of Luschka bilaterally into the pontocerebellar cistern. Operative intervention was pursued with resection of an identified arachnoid web. Postoperative imaging demonstrated marked reduction in the size of ventricular system, especially of the fourth ventricle. The patient's symptomatology resolved a few days after the procedure. CONCLUSION: Here, we describe an idiopathic case initially misdiagnosed as normal pressure hydrocephalus. The present case emphasizes the necessity of CISS sequences and fluoroscopic dynamic cisternography for suspected cases of fourth ventricular outlet obstruction as these diagnostic tests may guide surgical management and lead to superior patient outcomes.

Delayed cerebral ischemia causing cortical blindness due to repeat cocaine use weeks subsequent to aneurysmal subarachnoid hemorrhage.

Cocaine is a known vasoactive drug associated with poor clinical outcomes and high in-hospital mortality related to aneurysmal subarachnoid hemorrhage; however, the association of prior cocaine use with the incidence of vasospasm and delayed cerebral ischemia remains controversial. We report a case of a 42-year-old male with a history of active cocaine use who presented with a severe headache. Imaging demonstrated diffuse cisternal subarachnoid hemorrhage due to a ruptured basilar apex aneurysm, which was successfully treated with endovascular coil embolization. Despite expedited endovascular treatment and an initially benign clinical course, he suffered from delayed cerebral ischemia resulting in cortical blindness due to bilateral posterior cerebral artery vasospasm secondary to repeat cocaine use weeks after his initial ictus. To our knowledge, the present case is the first to describe delayed cerebral ischemia resulting in a severe neurologic deficit due to repeat cocaine use weeks subsequent to aneurysm rupture. We review the current literature on the association of cocaine use with the incidence of vasospasm and delayed cerebral ischemia as well as the effects of cocaine on the cerebrovasculature.

Factors affecting R01 grant funding among academic neurosurgeons over the last decade.

BACKGROUND: Recent studies have reported a gender and medical degree disparity for those receiving Research Project Grants in surgical specialties. The aim of the present study is to analyze factors among academics neurosurgeons that correlate to higher amounts of R01 grant monies awarded. MATERIALS AND METHODS: The National Institutes of Health Research Portfolio Online Reporting Tools Expenditures and Results database was queried for neurosurgery funding between 2008 and 2018. Grant recipients were categorized among type of degree, secondary degree(s), professorship, gender, and h - index. Statistical analysis was performed. RESULTS: The National Institutes of Health awarded 480 R01 grants totaling $182,482,644 to 81 allopathic neurosurgeons between 2008 and 2018. No osteopathic neurosurgeons were awarded an R01 grant during this timeframe. There was a significant difference for type of professorship on the total awarded amount at the p < 0.05 level for the three types of professorship [F (2,78) = 4.85, p < 0.01)]. There was a significant difference for magnitude of h - index on total R01 monies (p < 0.00001). Males accounted for the majority of R01 monies (93.99%); however, no significant difference between average amount awarded and gender was identified (p = 0.86). A secondary degree was without significant difference for R01 amount awarded (p = 0.75). CONCLUSIONS: The present study establishes a medical degree disparity for academic neurosurgeons who receive an R01 grant. Statistically significant factors found to affect amount of R01 grant monies awarded were limited to type of professorship and magnitude of h - index.

N-butyl cyanoacrylate embolization of a traumatic pseudoaneurysm and arteriovenous fistula of the middle meningeal artery.

We report a case of combined traumatic pseudoaneurysm and arteriovenous fistula of the middle meningeal artery, which presented clinically 1 year after initial head trauma. A 39-year-old male presented with seizure activity after a closed head injury from a fall. He was hospitalized for multiple intraparenchymal hemorrhages and ultimately discharged 2 weeks later without neurologic deficits. One year later, he presented with a new right anterior temporal intraparenchymal hemorrhage after a syncopal event. Selective angiography of the right external carotid artery demonstrated a chronic appearing traumatic laceration of the proximal middle meningeal artery with a 6 × 10 mm pseudoaneurysm and a single fistula with venous varix draining into the lateral pterygoid veins. The pseudoaneurysm and arteriovenous fistula were successfully embolized with n-butyl cyanoacrylate.

Neurosurgical Management of Lateral Meningocele Syndrome: A Clinical Update for the Pediatric Neurosurgeon.

BACKGROUND: Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos syndrome, and Loeys-Dietz syndrome. However, this syndrome is invariably associated with the presence of multiple lateral thoracolumbar spinal meningoceles: a distinct point of phenotypic divergence from other connective tissue disorders. The etiopathogenesis of this syndrome has recently been linked to truncating mutations within exon 33 of NOTCH3. Despite numerous reports, neurosurgical management of multiple spinal meningoceles remains poorly defined in the literature. We conducted a literature review to provide insight into the nosology, clinical significance, and neurosurgical management strategies of this distinct connective tissue disorder. SUMMARY: Our literature search revealed 11 articles (16 cases) of LMS, which included 9 males and 7 females, belonging to 14 different families. Half of these cases underwent genetic screening: all of which were discovered to exhibit a truncating mutation within exon 33 of NOTCH3. All patients exhibited multiple lateral thoracolumbar spinal meningoceles with craniofacial dysmorphisms. Other clinical characteristics included pathologic changes in spine morphology, Chiari I malformation, syringomyelia, hydrocephalus, and tethered cord. Operative management of multiple spinal meningoceles in LMS is complicated by the presence of such coexisting structural neurologic pathologies, which may alter cerebrospinal fluid flow dynamics and, ultimately, impact operative intervention. Key Messages: LMS is an exceedingly rare connective tissue disorder with severe spinal dural involvement. Neurosurgical management of multiple spinal meningoceles is complex, which is further complicated by the presence of coexisting neuropathology, such as pathologic transformation of spine morphology and Chiari I malformation. Patients with a connective tissue disorder phenotype found to have multiple spinal meningoceles on imaging studies may benefit from evaluation by a medical geneticist and a pediatric neurosurgeon.

Corticosteroid-Induced Regression of Glioblastoma: A Radiographic Conundrum.

Corticosteroid-induced regression of lesion contrast enhancement on imaging studies is most commonly appreciated with primary central nervous system lymphoma; however, although exceedingly rare, a limited number of primary and metastatic intracranial lesions have been reported to exhibit similar radiographic changes subsequent to corticosteroid therapy. To date, there have been six cases of glioblastoma reported to exhibit such changes. Lesion transformation on repeat imaging after the initiation of steroids represents a diagnostic dilemma for clinicians when attempting to differentiate between a diagnosis of glioblastoma and lymphoma. Stereotactic biopsy may be inadvertently postponed due to high clinical suspicion for steroid-induced cytotoxicity traditionally seen with lymphomatous cells. To highlight this radiographic conundrum, we present a rare case of corticosteroid-induced regression of glioblastoma and discuss the relevant literature. To our knowledge, this is the first case report to describe the molecular profile of a glioblastoma that underwent corticosteroid-induced regression.

Short- and Long-Term Geriatric Mortality After Acute Traumatic Subdural Hemorrhage.

BACKGROUND: Acute subdural hemorrhage often occurs in those ≥65 years of age after trauma and tends to yield poor clinical outcomes. Previous studies have demonstrated a propensity toward high in-hospital mortality rates in this population; however, postdischarge mortality data are limited. The objective of the present study was to analyze short- and long-term mortality data after acute traumatic subdural hemorrhage in the geriatric population as well as review the impact of associated clinical variables including mechanism of injury, pre-morbid antithrombotic use, and need for surgical decompression on mortality rates. METHODS: We retrospectively reviewed 455 patients who presented with an isolated traumatic acute subdural hemorrhage to our level-1 trauma center over a 5 year period using our data registry. Patients were then cross-referenced in the National Social Security Death Index for postdischarge mortality rates. United States life tables were used for peer-controlled actuarial comparisons. RESULTS: Acute traumatic subdural hemorrhage is often a fatal injury in the geriatric population, especially if taking antithrombotics or requiring surgical decompression. Specifically, they have greater in-hospital mortality rates than adults with similar injuries and have significantly lower survival rates for several years following discharge compared with their peer-matched controls. CONCLUSIONS: Here, we found that age is a significant predictor of both short- and long-term survival after acute traumatic subdural hemorrhage. Moreover, the present study corroborates that the need for surgical decompression or the use of pre-morbid antithrombotic medications is associated with increased overall mortality.

Quantitative Description of Osteopathic Physician Authorship in Prominent Neurosurgery Journals Since 1944: Coming of Age?

Background The Accreditation Council for Graduate Medical Education and the American Osteopathic Association recently agreed to establish a single graduate medical education system for the United States allopathic and osteopathic resident physicians by 2020. Consequential to this merger, new standards will be implemented for academic and research requirements within medical schools as well as residency programs. In the United States, osteopathic medicine is considered to be a parallel profession to allopathic medicine. However, recent studies have revealed that the percentages of United States osteopathic physicians currently in practice are not proportional to the percentages of editorial board member positions they hold in several high-profile medical journals as well as neurosurgical journals. To our knowledge, there is currently no published literature examining osteopathic physician author representation of any neurosurgical journal. In the present study, we analyze the number of osteopathic physicians and osteopathic neurosurgeons serving as authors in prominent neurosurgical journals. Methods American neurosurgical journals with the highest number of citations plus an affiliation with a neurosurgical society open to osteopathic neurosurgeons were used as criteria for journal selection. The Journal of Neurosurgery Publishing Group journals (Journal of Neurosurgery, Journal of Neurosurgery: Spine, Journal of Neurosurgery: Pediatrics, and Neurosurgical Focus) fulfilled these criteria. The number of allopathic and osteopathic physicians who have published at least one manuscript in a Journal of Neurosurgery Publishing Group journal was counted. The specialty of each osteopathic author was examined. Results Our analysis found that allopathic physicians represented 105,157 (99.68%) and osteopathic physicians represented 335 (0.32%) of the 105,492 authorship positions held by these physicians in these journals since 1944. Statistical significance was found comparing the number of allopathic authors versus the number of osteopathic authors (p < 0.0001). The most common specialty represented by osteopathic authors in all journals was neurosurgery (45%). Osteopathic neurosurgeons represented 153 (0.15%) of the total number of allopathic and osteopathic authors. Conclusions These data establish that the percentages of the United States osteopathic physicians and osteopathic neurosurgeons currently in practice are not proportional to the percentages of authorship positions they hold in Journal of Neurosurgery Publishing Group journals. We postulate that this apparent disproportionality may originate from significant differences between allopathic and osteopathic medical school research funding, research opportunities, scholarly activities, and dual-degree programs.

ACTA2 Cerebral Arteriopathy: Not Just a Puff of Smoke.

BACKGROUND: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. SUMMARY: We performed a literature search using PubMed with the key words "ACTA2 mutation," "ACTA2 cerebral arteriopathy," and "multisystemic smooth muscle dysfunction syndrome." Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.

Rapidly Expanding Lateral Ventricular Meningioma Presenting with Intraventricular Hemorrhage following Remote Whole Brain Radiation and Stereotactic Radiosurgery.

Intraventricular meningiomas are uncommon intracranial tumors and infrequently present with hemorrhage. With only 10 reported cases in the literature, it is exceedingly rare for meningiomas of the ventricular system to present with hemorrhage. To our knowledge, this is the first report of a patient presenting with an acute intraventricular hemorrhage in relation to a ventricular meningioma suspected to be radiation induced. In addition, we review the current literature on hemorrhagic intraventricular meningiomas and review the natural history of radiation-induced meningiomas.

Microsurgical Clipping of a Giant Middle Cerebral Artery Aneurysm with Successful Postoperative Endovascular Mechanical Thrombectomy for Emergent Treatment of Large Vessel Occlusion.

BACKGROUND: Giant intracranial aneurysms (>25 mm) are uncommon. These lesions typically manifest clinically due to mass effect, acute hemorrhage, or thromboembolic events. To minimize the risk of poor clinical outcome, detailed operative planning and a consideration of all neurosurgical and endovascular techniques are essential before proceeding with microsurgical clipping of ruptured giant aneurysms. CASE DESCRIPTION: We describe a case involving a 15-year-old male with a ruptured giant middle cerebral artery aneurysm treated with microsurgical clipping. After clip application, poor distal flow was demonstrated intraoperatively, and emergent angiography demonstrated an M1 occlusion with thrombus. A salvage procedure using endovascular mechanical thrombectomy reestablished distal flow resulting in a good neurologic outcome. CONCLUSIONS: To our knowledge, this is the first case report to describe microsurgical clipping of an aneurysm followed by successful postoperative endovascular mechanical thrombectomy.