OBJECTIVE:Enzyme replacement therapy (ERT) with cerliponase alfa (Brineura) has been shown to slow the progression of milestone deterioration in neuronal ceroid lipofuscinosis type 2 (CLN2), an inherited neurodegenerative lysosomal storage disorder. Cerliponase alfa must be administered intraventricularly every 2 weeks, necessitating the placement of ventricular reservoirs and requiring frequent access. Traditionally, positive cerebrospinal fluid (CSF) cultures obtained from implanted ventricular reservoirs are managed with device replacement and antibiotic treatment. The authors sought to establish which circumstances might allow for careful observation without device removal. In this study, the authors report their single-institution experience with the clinical outcomes of positive CSF cultures in 16 CLN2 patients with ventricular reservoirs over 6 years. METHODS:The authors retrospectively reviewed a cohort of 16 patients with CLN2 disease who had ventricular reservoirs placed for ERT administration. At each ERT infusion, CSF was collected by sterile technique and cultured with both thioglycolate broth and agar plate. This CSF collection was standard practice as per the initial trial protocol for use of ERT in CLN2. Epidemiological and microbiological data, symptomatology, total antibiotic days, removal and replacement of the ventricular reservoir, hospital length of stay, and mortality were analyzed for each patient. RESULTS:In the authors' cohort, 11 of 16 patients (69%) had at least 1 positive CSF culture. Of the 11 patients with positive cultures, only 3 had their device removed and replaced due to a positive culture with concurrent antibiotic treatment at the authors' center, and 2 patients subsequently were treated with prophylactic antibiotic infusion. Of 1401 total CSF cultures, 64 (4.56%) were positive. The most common organism grown was Cutibacterium acnes, which typically only grew in broth culture (82%), suggesting low bacterial burden. The other 8 patients with positive cultures remained asymptomatic with no intervention required. CONCLUSIONS:At the authors' institution, we have found that in asymptomatic patients with frequently accessed ventricular reservoirs, positive CSF culture with low virulence skin flora was common. Rarely did positive cultures with common skin flora necessitate intervention. The authors' experience has shown that nonvirulent infection of ventricular reservoirs with low virulence skin flora can be monitored without intervention. This strategy reduces the risks of invasive surgery, prolonged antibiotic courses, and missed infusions.

Background/Objectives: Anaplastic oligodendrogliomas (AOs) are central nervous system (CNS) World Health Organization (WHO) grade 3 gliomas characterized by isocitrate dehydrogenase (IDH) mutation (m)IDH and 1p/19q codeletion. AOs are typically treated with surgery and chemoradiation. However, chemoradiation can cause detrimental late neurocognitive morbidities and an accelerated disease course. The recently regulatory-approved vorasidenib, a brain-penetrating oral inhibitor of IDH1/2, has altered the treatment paradigm for recurrent/residual non-enhancing surgically resected CNS WHO grade 2 mIDH gliomas. Though vorasidenib can delay the time to chemoradiation for grade 2 gliomas, the implications for vorasidenib in non-grade 2 mIDH gliomas are not well understood. Results: We present a case of a 71-year-old male with a grade 3 non-enhancing oligodendroglioma successfully treated with vorasidenib with an 11% reduction in residual tumor volume. Vorasidenib was well tolerated in our patient with a mild elevation in his liver transaminases that resolved following a brief interruption in treatment. Conclusions: Our case suggests that vorasidenib may impart therapeutic benefits in this setting. This case illustrates the need for further investigation into these less commonly addressed scenarios and treatment strategies that extend beyond current guidelines.

AIMS:Medically-refractory epilepsy is a serious neurological problem. Surgical resection may offer the greatest chance of seizure freedom, but is underutilized. Laser interstitial thermal therapy (LITT) has minimal incision size and variable efficacy by pathology. LITT can be repeated to provide seizure freedom, however, the number of patients who receive subsequent LITT treatments after a failed first attempt is unknown. We aimed to review the literature on this potential patient population who might benefit most from multiple surgical treatments. METHODS:We performed a record review of multiple medical databases for studies treating 5 or more epilepsy patients with LITT utilizing the PRISMA guidelines. From the search, we reviewed 55 articles with a total of 1734 patients with at least six months of follow up. We recorded clinical variables, rates of seizure control after LITT, and details of follow up treatments. RESULTS:Of 1734 total epilepsy patients treated with LITT, 46.4 % were listed as seizure free at last follow up. Out of 850 patients with data on additional procedural treatment, 40.5 % were seizure free and 59.5 % had persistent seizures. Of these, 29.1 % received subsequent intervention for continued seizures. These included additional LITT (55.4 %), surgical resections (41.9 %), and neurostimulator placement (2.0 %). Seizure freedom was achieved in 51.20 % of patients undergoing additional LITT, 62.9 % of patients undergoing subsequent surgical resection, and no patient undergoing neurostimulation. Lesions more likely to achieve seizure freedom with LITT were cavernous malformations (66.7 %) and hypothalamic hamartoma (66.4 %). Lesions less likely to achieve seizure freedom with LITT were temporal lobe epilepsy (43.6 %), focal cortical dysplasia (45.5 %), and periventricular nodular heterotopia (35 %). CONCLUSION:Though LITT has respectable efficacy in epilepsy, not all patients may be participating in subsequent procedures after a failed first treatment. Patients should be advised that repeat LITT or subsequent surgical resection may be necessary for best chance of seizure freedom.

BACKGROUND: In the management of multi-drug- resistant focal epilepsies, intracranial electrode implantation is used for precise localization of the ictal onset zone. In select patients, subdural grid electrode implantation is utilized. Subdural grid placement traditionally requires large craniotomies to visualize the cortex prior to mapping. However, smaller craniotomies may enable shorter operations and reduced risks. We aimed to compare surgical outcomes between patients undergoing traditional large craniotomies with those undergoing tailored "mini" craniotomies (the "mail-slot" technique) for subdural grid placement. METHODS: This retrospective cohort study included 23 patients who underwent subdural electrode implantation for epilepsy monitoring between 2014 and 2020. Patients were categorized into mini-craniotomies (n = 9) and traditional large craniotomies (n = 14) groups. Demographics, operative details, and outcomes were reviewed. Craniotomy size and number of electrodes were determined via post hoc radiographs. RESULTS: Of the 23 patients studied, the mini group had smaller craniotomy sizes (mean: 22.71 cm(2) vs. 65.17 cm(2) , P < 0.001) and higher electrode-to-size ratios (mean: 4.25 vs. 1.71, P < 0.0001). The mini group had slightly fewer total electrodes (mean: 88.67 vs. 107.43, P = 0.047). No significant differences were found in operative duration, blood loss, invasive electroencephalography duration, complications, or Engel scores between the groups. One patient per group required further invasive epilepsy monitoring for localization; all patients underwent therapeutic surgery. CONCLUSIONS: Our findings suggest that mini- craniotomies for subdural grid placement in epilepsy monitoring offer significant advantages, including smaller craniotomy sizes and shorter operation durations, without compromising safety or efficacy. These results support the trend towards minimally invasive, patient-tailored surgical approaches in epilepsy treatment.

We describe a case of a young patient with a recurrent pleomorphic xanthoastrocytoma (PXA) showing unusual cell-in-cell (CiC) phenomena. We observed mostly viable but also necrotic neutrophils engulfed within tumor cells. The recurrent tumor was immunopositive for BRAFV600E mutant protein and showed CDKN2 homozygous deletions typical of PXA. Both genetic alterations were also reported in the original primary tumor. Unlike the original tumor that was GFAP and Olig-2 immunopositive, the recurrent neoplasm was largely negative for GFAP and Olig-2 suggesting dedifferentiation. The large malignant cells that contained the neutrophils were negative for histiocytic and lymphohematopoietic markers. Whereas CDKN2 homozygous deletion is common in PXA, its presence is rare in histiocytic neoplasms. Both reactive astrocytes and glial neoplasms very rarely may engulf neutrophils in a process resembling emperipolesis or cellular cannibalism. Future work may clarify which type of CiC pathway is involved.

INTRODUCTION:Interventional treatment options for the lumbar degenerative spine have undergone a significant amount of innovation over the last decade. As new technologies emerge, along with the surgical specialty expansion, there is no manuscript that utilizes a review of surgical treatments with evidence rankings from multiple specialties, namely, the interventional pain and spine communities. Through the Pacific Spine and Pain Society (PSPS), the purpose of this manuscript is to provide a balanced evidence review of available surgical treatments. METHODS:The PSPS Research Committee created a working group that performed a comprehensive literature search on available surgical technologies for the treatment of the degenerative spine, utilizing the ranking assessment based on USPSTF (United States Preventative Services Taskforce) and NASS (North American Spine Society) criteria. RESULTS:The surgical treatments were separated based on disease process, including treatments for degenerative disc disease, spondylolisthesis, and spinal stenosis. CONCLUSIONS:There is emerging and significant evidence to support multiple approaches to treat the symptomatic lumbar degenerative spine. As new technologies become available, training, education, credentialing, and peer review are essential for optimizing patient safety and successful outcomes.

Gunshot wounds (GSWs) to the head and neck are a common etiology of traumatic brain injury. Tangential GSWs (TGSWs) are a subset of GSWs wherein the missile penetrates tissue adjacent to the cranium, causing varying degrees of intracranial injury. Most patients sustaining TGSWs present with relatively benign neurological findings, and while a significant proportion have varying degrees of intracranial hemorrhage (ICH) on computed tomography, these tend to respond well to nonoperative management. We present a case report of a 28-year-old male who sustained a TGSW to the occiput, with a nonfocal neurological examination, small-volume posterior fossa ICH, a blunt vertebral artery injury (BVAI), and hepatic coagulopathy, who rapidly decompensated six hours after presenting due to massive posterior fossa hemorrhage with brainstem compression, requiring emergent cardiopulmonary resuscitation followed by suboccipital decompression and cerebrospinal fluid diversion. We propose that the patient's BVAI led to an unexpected thromboembolic event, precipitating an ischemic stroke that underwent hemorrhagic conversion in the setting of coagulopathy. This case report emphasizes the insidious danger that TGSWs to the head and neck present to patients, and risk factors for poor outcomes, such as BVAI and coagulopathy. This report also highlights potential intraoperative challenges during surgery for acute mass lesions in the posterior fossa, such as neurogenic shock and pulmonary edema, that warrant careful consideration and preparation in neurosurgical cases.

BACKGROUND: Nonfunctional pituitary neuroendocrine tumors (PitNETs) exhibit wide variability in growth pattern based on subtype. Silent corticotroph adenomas (SCAs) demonstrate aggressive growth compared with other nonfunctional pituitary adenomas (NFPAs), especially into the cavernous sinus. In this study, we sought to characterize other growth patterns of SCAs compared with NFPAs. METHODS: We performed a retrospective analysis of all patients with nonfunctional PitNETs treated with surgical resection via endoscopic endonasal approach at a single institution from August 1, 2018, to May 11, 2024. Preoperative computed tomography and magnetic resonance imaging were reviewed to determine extension into the suprasellar space, sphenoid sinus, cavernous sinus, and clivus. RESULTS: The study comprised 91 patients, including SCAs and 71 NFPAs. SCAs demonstrated significantly greater rates of growth into the sphenoid sinus (55.0% vs. 23.94%, P = 0.013), clivus (65.0% vs. 16.9%, P < 0.0001), and cavernous sinus (defined as Knosp grade 3 or 4; 55.0% vs. 23.35%, P = 0.016). Other NFPAs were more likely to grow into the suprasellar space (92.96% vs. 75.0%, P = 0.038). Tumor volume was similar between groups (11.93 cm3 vs. 9.06 cm3, P = 0.2). CONCLUSIONS: Silent corticotroph PitNETs demonstrate predilection for invasion of bony structures, with higher rates of growing through the sellar floor into the sphenoid sinus, growing posteroinferiorly into the clivus and laterally into the cavernous sinuses. Other nonfunctional NETs tended to follow the path of least resistance, growing superiorly into the suprasellar space. These differences growth patterns may account for some of the clinical challenges of treating silent corticotroph PitNETs.

Abstract INTRODUCTION Pituitary neuroendocrine tumors (PitNETs) are the second most common intracranial neoplasm. Silent or non-functional PitNETs represent a common neuro-oncological challenge due to their size and unpredictable invasion of parasellar structures. This makes their complete surgical resection difficult and puts patients at higher risk for recurrence. OBJECTIVE To characterize differences in tumor volume, cavernous sinus invasion, and recurrence in nonfunctional PitNETs. METHODS Out of 615 transsphenoidal surgeries from 2012 to 2024, there were 309 overall PitNETs and 181 nonfunctional PitNETs. Based on new WHO criteria for transcription factor profiling (PIT-1, TPIT, and SF-1), there were 98 silent gonadotroph adenomas (SGA), 31 silent corticotroph adenomas (SCA), 10 silent pleurihormonal tumors (PHT), and 2 null cell adenomas. Tumor volume was calculated using (AxBxC/2). Knosp grade was calculated. Cavernous sinus invasion was defined as Knosp ≥ 3. Residual disease at 3 months postoperative was recorded and surgical intervention for growing residual disease was captured. RESULTS Average tumor volume of PHT (18.08 cm3) was significantly higher than SGA (8.44 cm3, p=0.007) and similar to SCA (10.33 cm3, p=0.11). Percentage of cavernous sinus invasion was significantly different between SCA (31.82%) and SGA (10.20%, p=0.003), but not PHT (33.33%; p>0.05). There was a significantly higher residual rate in SCA compared to SGA (31.71% vs. 11.22%, p=0.006). There were significantly more reoperations in the SCA group compared to SGA and PHT. CONCLUSIONS Despite similar tumor volume between SCA and SGA, there was a significantly higher rate of cavernous sinus invasion and residual disease among SCA. This has important neuro-oncological implications regarding surgical technique, postoperative surveillance, and need for adjuvant therapy to mitigate aggressive recurrent disease.

Energy intake, fiber intake, and percentage of calories from carbohydrates, sugar, protein, and fat calculated from 24-hour diet recalls. Data are represented as mean ± SD

AbstractPurpose: Chronic inflammation is integral to myeloproliferative neoplasm (MPN) pathogenesis. JAK inhibitors reduce cytokine levels, but not without significant side effects. Nutrition is a low-risk approach to reduce inflammation and ameliorate symptoms in MPN. We performed a randomized, parallel-arm study to determine the feasibility of an education-focused Mediterranean diet intervention among patients with MPN. Experimental Design: We randomly assigned patients with MPN to either a Mediterranean diet or standard U.S. Dietary Guidelines for Americans (USDA). Groups received equal but separate education with registered dietician counseling and written dietary resources. Patients were prospectively followed for feasibility, adherence, and symptom burden assessments. Biological samples were collected at four timepoints during the 15-week study to explore changes in inflammatory biomarkers and gut microbiome. Results: The Mediterranean diet was as easy to follow for patients with MPN as the standard USDA diet. Approximately 80% of the patients in the Mediterranean diet group achieved a Mediterranean Diet Adherence Score of ≥8 throughout the entire active intervention period, whereas less than 50% of the USDA group achieved a score of ≥8 at any timepoint. Improvement in symptom burden was observed in both diet groups. No significant changes were observed in inflammatory cytokines. The diversity and composition of the gut microbiome remained stable throughout the duration of the intervention. Conclusions: With dietician counseling and written education, patients with MPN can adhere to a Mediterranean eating pattern. Diet interventions may be further developed as a component of MPN care, and potentially incorporated into the management of other hematologic conditions. Significance: Diet is a central tenant of management of chronic conditions characterized by subclinical inflammation, such as cardiovascular disease, but has not entered the treatment algorithm for clonal hematologic disorders. Here, we establish that a Mediterranean diet intervention is feasible in the MPN patient population and can improve symptom burden. These findings warrant large dietary interventions in patients with hematologic disorders to test the impact of diet on clinical outcomes.

Osteogenesis imperfecta (OI) predisposes individuals to easy bone fracture, vessel fragility, and platelet dysfunction. We report the first known case of neurointerventional treatment with flow diversion of intracranial aneurysms in a patient with OI. A 62 year-old female with known OI Type I, history of >40 lifetime bone fractures and hypertension, underwent workup for transient ischemic attacks revealing a 4-mm right A1 segment aneurysm in 2016. Perioperative dual antiplatelet therapy was aspirin 81 mg and clopidogrel 37.5 mg daily. Tri-axial access was utilized to deploy a 3.5 × 16-mm Pipeline Flex device without complication. Two-month follow-up revealed Raymond I (O’Kelly Marotta I) obliteration of the aneurysm. Five-year follow-up revealed a de novo left-sided 3-mm A1–A2 junction aneurysm. A 4 × 12-mm Surpass Evolve was placed without complication. Six-month follow-up revealed Raymond I (O’Kelly Marotta I) obliteration of the second aneurysm. The patient remained asymptomatic at all follow-up visits.

Mesial temporal lobe epilepsy (MTLE) is a common cause of seizures, and hippocampal sclerosis (HS) is the predominant subtype. BRAFV600E mutations in MTLE-HS have only been reported infrequently. Herein, we illustrate the neurologic, radiological, and histopathological details of a patient with MTLE-HS and BRAFV600E mutant neurons. A 31-year-old male with medically refractory epilepsy presented with magnetic resonance imaging (MRI) and electroencephalography (EEG) findings typical of mesial temporal sclerosis without a mass lesion. The surgical specimens showed ILAE Type 1 HS with neurons immunopositive for BRAFV600E mutant protein distributed along the Cornu Ammonis (CA) curvature. Instead of the normal mostly perpendicular orientation of pyramidal neurons relative to the hippocampal surface, the BRAF mutant neurons were often oriented in a parallel manner. On CD34 immunostaining, sparse clusters or nodules of CD34+ stellate cells and single immunopositive stellate cells were identified. BRAFV600E or CD34 immunopositive cells were less than 1 % of total cells. The patient responded well to surgery with no further seizures after 2 years and occasional auras. Hippocampal BRAF mutant non-expansive lesion (HBNL) has been used to describe such lesions with preserved cytoarchitecture and without overt tumor mass. Others may argue for the dual pathology of HS with early ganglioglioma. Whether pre-neoplastic lesions or early tumors, these cases are important for understanding early glioneuronal tumorigenesis and suggest that BRAFV600E studies should be routinely performed on MTLE-HS cases in the setting of clinical trials. With next-generation sequencing, a FANCL deletion was detected in almost half of the alleles in our case, suggesting that many of the histologically normal-appearing cells of the hippocampus contain this alteration. FANCL mutations can result in cytogenetic anomalies and defective DNA repair and therefore may underlie the development of a low frequency BRAF alteration.

Mesial temporal lobe epilepsy (MTLE) is a common cause of seizures, and hippocampal sclerosis (HS) is the predominant subtype.

Introduction: Hydrocephalus is a common pediatric neurosurgical pathology, typically treated with a ventricular shunt, yet approximately 30% of patients experience shunt failure within the first year after surgery. As a result, the objective of the present study was to validate a predictive model of pediatric shunt complications with data retrieved from the Healthcare Cost and Utilization Project (HCUP) National Readmissions Database (NRD). Methods: The HCUP NRD was queried from 2016 to 2017 for pediatric patients undergoing shunt placement using ICD-10 codes. Comorbidities present upon initial admission resulting in shunt placement, Johns Hopkins Adjusted Clinical Groups (JHACG) frailty-defining criteria, and Major Diagnostic Category (MDC) at admission classifications were obtained. The database was divided into training (n = 19,948), validation (n = 6,650), and testing (n = 6,650) datasets. Multivariable analysis was performed to identify significant predictors of shunt complications which were used to develop logistic regression models. Post hoc receiver operating characteristic (ROC) curves were created. Results: A total of 33,248 pediatric patients aged 6.9 ± 5.7 years were included. Number of diagnoses during primary admission (OR: 1.05, 95% CI: 1.04–1.07) and initial neurological admission diagnoses (OR: 3.83, 95% CI: 3.33–4.42) positively correlated with shunt complications. Female sex (OR: 0.87, 95% CI: 0.76–0.99) and elective admissions (OR: 0.62, 95% CI: 0.53–0.72) negatively correlated with shunt complications. ROC curve for the regression model utilizing all significant predictors of readmission demonstrated area under the curve of 0.733, suggesting these factors are possible predictors of shunt complications in pediatric hydrocephalus. Conclusion: Efficacious and safe treatment of pediatric hydrocephalus is of paramount importance. Our machine learning algorithm delineated possible variables predictive of shunt complications with good predictive value.

There are numerous emergency department visits in the United States for all types of marine animal injuries each year. These injuries may result in significant morbidity or mortality if not managed appropriately. Accurate identification of the offending species, thorough wound hygiene, and judicious use of antibiotics are important for preventing infections. This review aims to describe management strategies and antimicrobial considerations for nonmammalian marine vertebrate penetrating trauma in North America, the Caribbean, and Hawaii. A literature search was performed to identify studies on this subject. This literature consisted of clinical case reports and case series. Reports extracted included those on sharks, barracuda, eels, catfish, stingrays, lionfish, stonefish, and scorpionfish. The majority of reported trauma occurred to beachgoers, fishermen, or commercial aquarium employees who routinely handle these animals. Injury patterns depended on the species but most commonly affected the lower extremities. Infections were seen from saltwater bacteria, human skin flora, or marine animal oral flora. After thorough wound irrigation and exploration, most authors recommended prophylactic antimicrobials to cover Vibrio species, in addition to other gram-negative and gram-positive species. The literature is notable for the lack of controlled studies. Some authors recommended radiographic and/or ultrasonographic imaging to identify retained foreign bodies, such as spines, sand, or teeth.

Embolization of the middle meningeal artery (MMA) is a safe and effective adjunct in the treatment of chronic subdural hematoma. While prior authors describe the use of coils to assist embolization by preventing reflux through eloquent collaterals, we de- scribe the use of coils to further open the MMA, allowing the administration of greater amounts of embolisate for a more robust embolization. The objective of this study was to demonstrate that helical coils can safely open the MMA following the administration of polyvinyl alcohol (PVA) particles. This allows for more embolisate to be administered into the MMA for more effective treatment. A retrospective review was conducted at our institution including intraoperative images and postoperative clinical and radiographic follow up. Failure rates using MMA embolization with PVA and helical coil augmentation were compared to failure rates in the literature of MMA embolization with PVA or ethylene vinyl-alcohol copolymer alone. A total of 8 cases were reviewed in which this technique was implemented. There were no immediate complications after treatment. All patients that underwent helical coil embolization following the administration of PVA had increased amount of embolisate delivered into the MMA. All patients at follow up had resolution of the subdural hematoma on outpatient imaging. Helical coil embolization allows for more embolisate administration into the MMA and provides a technical advantage for patients that fail traditional techniques of embolization. Case series are taking place to further test this hypothesis and identify the ideal patient population that may gain maximal yield from this novel technique.