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770 mostly novel protein-protein interactions were identified using a stringent yeast two-hybrid screen. The protein-protein interaction network was developed for the 20 or more different inherited cerebellar ataxias characterized by Purkinje cell degeneration.

Drosophila Ataxin-1 (dAtx-1), which has conserved AXH domain but lacks a polyglutamine tract is studied to understand how stabilized human Atx-1 induces selective neuronal degeneration. Results show that the Atx-1/Gfi-1 interaction contribute to the selective Purkinje cell degeneration in spinocerebellar ataxia type1 (SCA1).

This study aimed to determine if partial removal of the occipital condyle provides a significant increase in visibility and “angle of attack” for treating lesions with extension ventral to the brainstem in children using CT morphometric data. Morphometric analysis was performed in 199 children using CT scans. Angle of attack was measured for both the paracondylar and transcondylar far lateral approach. Statistical analysis was performed using paired or unpaired Student’s t -tests ( p < 0.05) and linear regression analysis. For the far lateral paracondylar approach, the overall angle of attack was 85 ± 9 degrees (range, 60–119 degrees). The overall angle of attack for the far lateral transcondylar approach was 70 ± 9 degrees (range, 48–105 degrees). This difference was significant ( p < 0.0001). Based on our data, resection of one-third of the occipital condyle in a far lateral transcondylar approach can improve angle of attack by approximately 15 degrees, regardless of age or sex, in the pediatric age group. It is important to keep in mind that there are risks attendant to resection of the occipital condyle, thus the resection of the occipital condyle in children should not be a mandatory part of the far lateral approach; rather, the decision-making should be individualized and considered on a case-by-case basis. [ABSTRACT FROM AUTHOR]

The article describes the case of a 38-year-old woman who presented with new onset seizures and severe bifrontal headache of one week's duration. The patient was found to have an opacified right frontal sinus. She was diagnosed with frontal sinus encephalocele which was resected and the dural defect closed.

Abstract: Spinal epidural lipomatosis (SEL) is an abnormal accumulation of unencapsulated, epidural fat. SEL can be divided into idiopathic and secondary. Secondary SEL is often associated with chronic steroid use and endocrinopathies. Idiopathic SEL has been associated with obesity. SEL has been implicated in a variety of neurologic impairments and surgical decompression has been shown to prevent further worsening or result in improvement. We report a 53-year-old man with obesity and a history of chronic back pain who developed idiopathic SEL diagnosed by MRI, which subsequently resolved completely over an 8month follow-up period. To our knowledge, this is the first reported case of complete radiographic resolution of SEL without any treatment. [Copyright &y& Elsevier]

Abstract: Intracranial germinoma, a radiosensitive tumor, is seldom recurrent following initial treatment. When it does recur, it is usually soon after initial treatment and secondary to inadequate radiation field coverage of the tumor. Rarely, there have been case reports of late recurrence many years after initial therapy. Patients with recurrent germinoma in the spine have a less favorable prognosis in terms of treatment response compared to the initial lesion. Thus, careful consideration of the initial lesion, its treatment, and serial imaging of the neural axis with close follow-up is important. We report a patient with a rare delayed recurrence in the brain and cervical spinal cord despite close follow-up with clinical examination and serial imaging. [Copyright &y& Elsevier]

Abstract: Spinal osteoblastomas are considered benign tumors but can be locally aggressive. Patients usually present with pain and undergo radiologic and histologic work-up to establish a diagnosis. Osteoblastomas have discordant appearances on CT scans and MRI because of the inflammatory response seen on MRI that characterizes these tumors – the “flare” phenomenon. Solely using MRI can lead to over-resection, as signal abnormality may include areas devoid of tumor. There are a few reports of this phenomenon in the radiology literature, but to our knowledge, there are none in neurosurgery journals. We report an 11-year-old boy who presented with back pain and radiculopathy with an osteoblastoma at the L4 level. We totally excised the lesion to definitively treat the patient but also, based on MRI findings, differentially biopsied portions of the lesion and correlated them to the imaging studies, to confirm that the intense reactive portion of the lesion was devoid of tumor cells. [Copyright &y& Elsevier]

The 2021 U.S. neurosurgery residency match interviews were conducted virtually; we surveyed applicants and interviewers to determine satisfaction with that virtual interview process. Subsequently, we conducted a follow-up survey to determine satisfaction with the virtual interview process after the residency match for faculty interviewers and 2022 interns. A 22-question online faculty survey was sent to 116 U.S. neurosurgery training programs. A 26-question survey was sent to these programs for distribution to their intern classes. Data were analyzed quantitatively, including mean Likert score. Open-ended questionnaire responses were reviewed to identify themes. Overall, 32 interns representing 20 programs and 73 faculty representing 62 programs responded. Most respondents agreed that virtual interviews were more convenient (86% faculty, 90% interns) and cost-effective (100% interns) than in-person interviews. Faculty respondents agreed or strongly agreed that virtual interviews were effective to evaluate applicants' competence as residents (44%); fewer faculty agreed or strongly agreed that virtual interviews were an effective way to evaluate candidates' fit in the program (27%). For interns, 44% agreed or strongly agreed that virtual interviews gave them a good sense of the program faculty; 75% agreed or strongly agreed they were satisfied with the process related to where they matched. Virtual interviews offer an advantage in terms of time and cost but potentially at the expense of adequate faculty assessment of candidates' "fit" within a program's culture. Despite this, interns undergoing an all-virtual interview process report high satisfaction with the results of the residency match. [ABSTRACT FROM AUTHOR]

Papillary glioneuronal tumors (PGNTs) are rare World Health Organization grade I neoplasms that are characterized by a benign course and excellent response to surgical resection. A few reports exist of tumors with more aggressive clinical and histologic features. In this report we detail the case of an unusually aggressive PGNT in a 67-year-old woman. The patient had a 3-year history of seizures and was diagnosed with a frontoparietal mass on imaging. She underwent subtotal resection with a histologic diagnosis of PGNT. Less than a year after surgery, the patient experienced recurrence of disease and underwent reresection and adjuvant radiation treatment. The patient's disease continued to progress despite radiation treatment, so adjuvant temozolomide was initiated. Molecular testing was performed and revealed a TERT promotor mutation, an FGFR3-TACC3 oncogenic fusion, and a copy number loss in CDKN2A/CDKN2B. PGNTs, while typically benign, can rarely recur after surgery. Molecular testing should be performed on all PGNTs to help possibly identify more aggressive tumors and potentially reveal novel treatment options. [ABSTRACT FROM AUTHOR]

Background The pipeline embolization device (PED) is a flow-diverting stent that provides an additional treatment modality in the management of intracranial aneurysms. An aneurysm treated with a flow diverter is expected to involute over time, contrary to the immediate obliteration expected by surgical clipping or coiling. Yet, which aneurysms will respond to PED therapy and the time frame to expect full obliteration remain unclear. Case Description We report the unusual case of a 50-year-old woman with multiple (4 total) intracranial aneurysms who underwent multimodality treatment. Two aneurysms were treated with PEDs. Nine months later, the patient underwent a craniotomy for treatment of an additional aneurysm; at the time of surgery, one of the PED-treated aneurysms was noted to be clearly obliterated, and the other was visualized to be filling. The ophthalmic artery arose from the persistently filling aneurysm. The aneurysm was treated by clip ligation without incident. Conclusions The rate of PED aneurysm obliteration increases with longer follow-up; however, the time frame for observing a persistently filling aneurysm before additional treatment is considered remains unknown. Some aneurysms may never close even after discontinuation of dual antiplatelet therapy. Ophthalmic artery aneurysms have been noted to fail treatment with PED based on the anatomic relationship of the aneurysm to the ophthalmic artery. This case provokes us to consider factors that may affect the latency to aneurysm obliteration, including aneurysm size, aneurysm morphology, patient gender, failure of previous aneurysm treatment, and duration of time from initial endovascular treatment. [ABSTRACT FROM AUTHOR]

Background X-linked dystonia-parkinsonism (XDP) is a progressively debilitating movement disorder that begins with focal dystonia and eventually generalizes. It exclusively affects Filipino inhabitants of the island of Panay. We report a case of XDP successfully treated by deep brain stimulation (DBS) and review the literature. Methods A 36-year-old man with XDP failed medical management and underwent bilateral globus pallidus internus DBS. A search of the PubMed database was performed to identify all articles discussing DBS and XDP. “Stimulation,” “DYT3,” “Lubag,” “torsion dystonia,” and “dystonia-parkinsonism” were used as MeSH headings. Results The patient's postoperative course was notable for delayed emergence from anesthesia. When stimulation was started, he had immediate improvement in his symptoms, and at 6-month follow-up, he is able to ambulate with the assistance of a walker. Review of the literature revealed 5 previously reported cases of XDP treated with DBS. Conclusions The published experience with globus pallidus internus DBS for XDP has been very positive to date. Although long-term follow-up data are needed, early results provide optimism for patients with this debilitating disorder. [ABSTRACT FROM AUTHOR]

Objective: Higher benchmarks in safety for patients undergoing neurosurgery have been introduced. With these principles, new tools and techniques were established, including intraoperative neurophysiological monitoring (IONM). Current trends as a function of patient-, surgeon-, and procedure-related factors and complication rates in the utilization of IONM as an adjunct to the practice of pediatric neurosurgery have not been investigated previously. Methods: Between 2008 and 2011, 4467 neurosurgical procedures were performed on 2352 patients at Texas Children's Hospital. A retrospective chart review was performed in which surgeon, procedure, and patient characteristics, as well as perioperative complications, were recorded for IONM and non-IONM cases. Results: Neurosurgical procedures performed with IONM steadily increased. Surgeon-related factors associated with IONM use included surgeons with <10 years of practice (P < .0001), and subspecialty interest in spine (P < .0001) and oncology (P = .0048). Procedure-related factors associated with IONM use included operations involving the spinal cord (P < .0001). Patient-related factors associated with IONM use included children older than 3 years of age and with increased American Society of Anesthesiologists score (P < .0001). The neurological complication rate in the IONM cohort (range 3.4% to 11.3%; mean 6.4%) was significantly higher compared to the non-IONM cohort (range 1.1% to 1.8%; mean 1.5%) (P < .0001). Conclusions: The percent of procedures performed with IONM increased. However, these trends do not seem governed by improvement to patient outcomes because the complication rates were higher in the IONM cohort than the non-IONM cohort. [Copyright &y& Elsevier]

Due to the COVID-19 pandemic, the ACGME recommended all interviews for the 2021 residency application cycle be held virtually. Because this is major shift from neurosurgical interviews in past years, this study aims to evaluate both applicant and interviewer satisfaction of conducting interviews virtually. For faculty, an 11-question online survey was sent to 116 United States neurosurgery training programs. A 14-question online survey was sent to 255 neurosurgery applicants. The resulting data were analyzed qualitatively and quantitatively. From applicants, 118 responses were received. From faculty, 171 individual responses were received. Thirty-five percent (34.7%) of applicants agreed that they were satisfied with the virtual interview process as a whole. Although 44.5% of faculty disagreed with the statement "I would like to replace in-person interviews with virtual interviews in the future", 57.3% of faculty agreed that they were likely to implement virtual interviews in the future. Some things might be better assessed through in-person interviews, but there are clear benefits to virtual interviews. Future iterations of the interview process, incorporating virtual interviews, might help determine how and in which situations virtual interviews can be utilized in future residency application cycles. [ABSTRACT FROM AUTHOR]

Preoperative venogram of the superior sagittal sinus (SSS) is helpful to plan the surgical resection strategy in patients with parasagittal meningiomas invading the SSS. Catheter 3-dimensional rotational venography (3D-RV) allows for SSS patency assessment and detection of alternative venous cortical drainage routes in patients with contraindication for magnet resonance venography. It is unknown if separate bilateral internal carotid artery 3D-RV followed by postprocessing 3D-3D fusion (technique 1) achieves the same imaging results as simultaneous bilateral internal carotid artery 3D-RV without postprocessing fusion (technique 2) needed. In this report we were able to confirm in 2 patients that both techniques achieve comparable imaging quality with similar amount of contrast use. Although technique 2 requires less radiation, technique 1 is favored due to the need for only 1 vessel access site and catheter with reduced risk for access site and ischemic complications. [ABSTRACT FROM AUTHOR]

The surgical decision-making process for cavernous malformation (CM) must weigh the risks of surgery against the burden of patient symptoms/hemorrhage and anticipated natural history. Here, we sought to internally validate an International Classification of Disease (ICD)-10 search algorithm for CM surgery to use to analyze a nationwide administrative database. Institutional records were accessed to test the validity of a novel ICD-10 search algorithm for CM surgery. The algorithm identified patients with positive predictive value (92%), specificity (100%), and sensitivity of 55%. The algorithm was applied to extract our target population from the Nationwide Readmissions Database. Univariate and multivariable analyses were used to identify factors influencing patient outcomes. We identified 1235 operations for supratentorial (87%) or infratentorial (13%) CM surgery from the Nationwide Readmissions Database (2016–2017). The overall rate of adverse disposition and 30-day readmission were 19.7% and 7.5%, respectively. The rate of adverse disposition was significantly higher for infratentorial (vs. supratentorial cases) (34.3% vs. 17.6%, P = 0.001) and brainstem (vs. cerebellar) cases (55% vs. 28%, P = 0.03). Hospital case-volume percentile was associated with decreasing rates of adverse disposition (1–74th: 22%, 75th: 16%, 90th: 13%, 95th: 7%). Treatment at HVCs was also associated with shorter average length of stay (4.6 vs. 7.3 days, P < 0.001) without significant changes to average cost of hospitalization (P = 0.60). Our ICD-10 coding algorithm reliably identifies CM surgery with minimal false positives. Outcomes were influenced by patient age, clinical presentation, location of CM, and experience of institution. Centralization of care may improve outcomes and warrants further investigation. [ABSTRACT FROM AUTHOR]

Intracranial solitary fibrous tumors (ISFTs) are rare neoplasms of mesenchymal origin that originate from the meninges. ISFTs of the skull base can be challenging to treat, as resection can be complicated by skull base anatomy. We present 2 cases of ISFT, the first manifesting with compressive cranial neuropathy from Meckel cave involvement and the second a posterior fossa lesion causing symptomatic hydrocephalus. A systematic review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The PubMed database was queried with title/abstract keywords "intracranial," "solitary fibrous tumor," "hemangiopericytoma," "SFT," and "HPC." Search results were reviewed to exclude cases not involving the skull base. References from all selected articles were reviewed for potential additional cases. Patient demographic and clinical data from 58 identified skull base cases were collected for qualitative synthesis. Visual disturbances were the most common presenting symptom (30 cases, 52%) followed by headache (22 cases, 38%). The most common site of involvement was the sellar/parasellar region (18 cases, 31%) followed by middle fossa/temporal bone (14 cases, 24%). Resection was performed in 55 cases; gross total resection was reported in 26 cases (45%) and subtotal resection was reported in 21 cases (36%). Tumor recurrence was documented in 15 cases (26%) with median and mean follow-up periods of 16 and 29.9 months, respectively. We discuss presentation, imaging, histopathology, and management considerations for ISFTs while highlighting the potentially complex nature of skull base lesions and need for multidisciplinary approach to treatment. [ABSTRACT FROM AUTHOR]

Optic pathway gliomas (OPGs) are relatively rare, and their presentation after the first decade of life is even less common. Although many treatment options exist, surgery is typically reserved for tumors significantly compressing surrounding structures. Pregnancy can complicate the management of these tumors, as fetal developmental considerations limit the ways in which they are imaged and treated. In this report we detail the case of a 27-year-old pregnant woman who was found to have an OPG during her third trimester. After a decline in this patient's vision and clinical status, a decision was made to induce labor at 31 weeks so that her disease could be more thoroughly addressed. While OPGs are typically benign tumors, pregnancy complicates their management significantly. Contrast media and anesthesia pose significant risks to the fetus, while pregnancy may contribute to increased rates of tumor growth and clinical deterioration. Managing OPGs in pregnant patients thus requires balancing the risks to the fetus and patient. [ABSTRACT FROM AUTHOR]

Background Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis that typically occurs in middle-aged patients. It is usually characterized by multifocal osteosclerotic lesions of the long-bones, however many cases have extraskeletal involvement. Central nervous system (CNS) involvement is common, but isolated CNS involvement at presentation has rarely been reported. Case Description Here we report two cases of dural-based ECD mimicking meningioma on imaging with no other identified sites of disease. Conclusion ECD is a rare disease, with isolated CNS involvement reported only a few times in the literature. The significance of this presentation requires additional study and long-term follow up. [ABSTRACT FROM AUTHOR]

We present a parietal interhemispheric approach to resect a pineal region falcotentorial meningioma. Three-dimensional (3D) modeling was used to visualize and plan the surgical approach in virtual reality (Video 1). A 78-year-old woman was incidentally found to have a slow-growing falcotentorial meningioma. The tumor was first treated with stereotactic radiosurgery, but the patient had progressive confusion, memory issues, and bilateral temporal field cuts with interval expansion of the tumor and significantly increased peritumoral vasogenic edema on magnetic resonance imaging. After recommending tumor resection, the patient consented to surgery and underwent resection via a posterior interhemispheric subsplenial approach. Using a 3D model in virtual reality, we discuss the advantages and disadvantages of various classical approaches to the pineal region for resection of this tumor. 1-3 Falcotentorial meningiomas often displace the deep veins inferiorly, making an interhemispheric approach more favorable. 4-6 Preoperative visualization of critical deep venous structures with the 3D model was a valuable adjunct to magnetic resonance imaging for achieving safe resection. We depict key steps of the surgical planning process using virtual reality and demonstrate how simulation can be used to evaluate risks and benefits of different surgical corridors. A small rim of residual tumor adherent to the deep cerebral veins was intentionally left behind in order to minimize risk of morbidity to the patient. 7 At 1-month follow-up, the patient's cognition had returned to baseline and her vision had significantly improved. Ultimately, surgical planning using virtual reality promotes both neurosurgical education and patient safety through clear visualization and understanding of different surgical approaches. [ABSTRACT FROM AUTHOR]

Background Intraventricular cavernous malformations are relatively rare benign vascular malformations. Patients may be asymptomatic or present with headache, seizure, hemorrhage, or neurologic deficits. We report 2 cases of patients with cavernomas in the third ventricle and at the foramen of Monro. We also performed a systematic review of the literature to examine the clinical features and efficacy of the current standard of care for these lesions. Methods We performed the systematic review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Multiple databases were queried; the title/abstract and MeSH keywords used included “cavernous malformation,” “cavernoma,” “cavernous hemangioma,” “cavernous angioma,” “foramen of Monro,” “third ventricle,” and “intraventricular,” along with “AND” and “OR” operators. Patient demographic and clinical data were collected for qualitative synthesis. Results Patients presented at a median age of 38 years; the most common symptom was headaches. Gross total resection was performed in 84.6% of patients, and 81.8% had clinical improvement with intervention. The incidence of intraventricular hemorrhage and hydrocephalus was 15.4% and 59%, respectively. Conclusions The specific location of the cavernoma determines clinical features seen and approach used in surgical resection. Ventriculoperitoneal shunting was not required in most cases, as hydrocephalus improved with removal of the obstruction at the foramen of Monro. Gross total resection appears to be the optimal management strategy in symptomatic patients and leads to a good outcome in most cases. [ABSTRACT FROM AUTHOR]