OBJECTIVE: Predicting language lateralization using functional MRI (fMRI) in patients with cerebral vascular malformations close to language areas is essential for treatment decision-making and patient outcomes. Function...OBJECTIVE: Predicting language lateralization using functional MRI (fMRI) in patients with cerebral vascular malformations close to language areas is essential for treatment decision-making and patient outcomes. Functional MRI-based prediction is challenged because of potential remodeling processes and hemodynamic phenomena. However, there is a lack of possible factors influencing laterality prediction. The authors hypothesized that there might be an impact of lesion type and location on language lateralization. METHODS: This retrospective study included 24 patients with arteriovenous malformations (AVMs), 11 patients with cavernomas, and 15 healthy controls. Participants performed a subvocal verb-generation task during fMRI. Data analysis in Statistical Parametric Mapping (SPM) 12 involved realignment, coregistration, and smoothing for preprocessing. The authors conducted a whole brain analysis using the general linear model approach at the individual level and calculated the lateralization indices (LIs) using the LI toolbox implemented in SPM independently based on the frontal, temporal, and parietal lobes. RESULTS: The mean absolute LIs were above 0.2 in all groups. Distribution between groups varied significantly (p = 0.032, f = 0.34). A significant difference was found between patients with AVMs and healthy controls (p = 0.038, r = 0.628). Specifically, patients with frontal AVMs showed significantly lower frontal LIs than did healthy controls (p = 0.032, r = 0.435). In contrast, LIs in cavernoma patients did not differ significantly from controls (p = 0.313). No significant difference was observed between language-adjacent and language-distant lesions (p = 0.14). CONCLUSIONS: The results of this study suggest that lesion type and location influence language lateralization prediction. Frontal AVMs exhibit significantly lower LIs, requiring caution and experience in interpreting results to ensure patient safety. Cavernomas did not influence LI. Further research with larger cohorts is necessary to understand the underlying causality and neuroplastic changes involved.
Singh AS, Seri S, Lo WB
… +11 more, Agrawal S, Kumar R, Sudarsanam A, MacPherson L, Gagen R, Williams H, Lawley A, Carr B, Lewis J, Stephanede A, Pepper J
OBJECTIVE: Stereo-electroencephalography (SEEG) is increasingly being used in pediatric epilepsy surgery to delineate the epileptogenic zone (EZ) and its relationship to functional cortex, ultimately guiding resection. H...OBJECTIVE: Stereo-electroencephalography (SEEG) is increasingly being used in pediatric epilepsy surgery to delineate the epileptogenic zone (EZ) and its relationship to functional cortex, ultimately guiding resection. However, accurately identifying which children with presumed focal epilepsy will benefit from electrode implantation remains challenging, particularly in MRI-negative cases. The 5-SENSE score, developed and validated primarily in adults, integrates 5 noninvasive clinical variables to estimate the likelihood of identifying an EZ using SEEG. This study presents the first validation of the 5-SENSE score in a purely pediatric population. METHODS: The authors conducted a retrospective analysis of electroclinical and imaging data of patients younger than 19 years seen at a tertiary pediatric epilepsy surgery center who underwent SEEG. Data were extracted from presurgical evaluations, and the 5-SENSE score was calculated for each patient based on 1) the presence of a focal lesion on MRI, 2) regional ictal onset on scalp video-EEG, 3) absence of bilateral independent interictal discharges, 4) localizing clinical semiology, and 5) neuropsychology. SEEG outcomes were reviewed to determine whether a focal EZ was identified and compared with 5-SENSE predictions. RESULTS: Seventy-nine patients (median age 13 years, range 3-18 years) with full information were included. The 5-SENSE score demonstrated a sensitivity of 70.7% (95% CI 58.3%-81.3%) and a specificity of 71.4% (95% CI 50.4%-87.5%) for identifying a focal EZ. The area under the receiver operating characteristic curve was 0.72 (95% CI 0.57-0.86), indicating that the 5-SENSE score accurately predicted favorable SEEG outcomes. The positive and negative predictive values were 87.2% and 46.9%, respectively. These findings are consistent with those of adult validation studies. CONCLUSIONS: This is the first validation of the 5-SENSE score in a purely pediatric population. While it shows moderate predictive accuracy, a low score does not exclude the possibility that SEEG might still identify a focal area of seizure onset.
OBJECTIVE: The inferior longitudinal fasciculus (ILF) is a major occipitotemporal white matter bundle involved in higher-order visual and cognitive functions. However, its anatomical consistency, structural organization,...OBJECTIVE: The inferior longitudinal fasciculus (ILF) is a major occipitotemporal white matter bundle involved in higher-order visual and cognitive functions. However, its anatomical consistency, structural organization, and distinction from adjacent tracts remain controversial. The aim of this study was to address these debates by investigating the dual nature of occipitotemporal connections, refining the classification of ILF subcomponents, and reassessing the inclusion of the dorsolateral occipital cortex component (DLOCC) in the ILF. METHODS: Cadaveric dissection was performed on 10 hemispheres from 5 neurologically healthy donors. Specimens were fixed, frozen, and dissected under an operating microscope according to Klingler's technique. The superficial U-fibers were peeled away to expose long associative fibers, and their cortical origins, trajectories, and relationships with surrounding structures were documented. Complementary diffusion tensor imaging (DTI) was performed in 5 subjects with no evidence of neurological disease and 20 subjects from the Human Connectome Project database. DTI data were acquired and analyzed with deterministic fiber tracking. Manual region-of-interest placement, length-based filtering, and shape analysis enabled reconstruction of short and long fiber tracts, corresponding to those identified in anatomical dissections. The integration of ex vivo and in vivo findings allowed detailed mapping and classification of occipitotemporal pathways. RESULTS: The investigation revealed consistent indirect U-fiber chains and direct long-range fascicles. The vertical occipital fasciculus and a distinct occipito-fusiform fasciculus (OFF) were identified, the latter of which connected the superior occipital gyrus to the anterior fusiform and inferior temporal gyrus. The fusiform component was found to be a thin intragyral tract with selective termination in the anterior fusiform gyrus. Furthermore, the cuneolingual component, which combines the lingual and cuneal fibers, was defined and showed common temporal terminations and overlapping anatomy. The DLOCC, while anatomically consistent, exhibited greater similarity and overlap with the middle longitudinal fasciculus (MdLF) than with the core ILF. Tractography confirmed these findings, showing diverging courses and terminations. CONCLUSIONS: These findings support a dual-model framework of occipitotemporal connectivity, comprising both direct and indirect fibers. The ILF should be redefined to include only the fusiform and cuneolingual components, with the DLOCC more accurately attributed to a distinct parietotemporal system alongside the MdLF. The OFF represents a separate underrecognized tract. This refined anatomical framework enhances the understanding of occipitotemporal pathways and might inform future functional and clinical studies.
OBJECTIVE: Syringomyelia is a progressive neurological disorder characterized by intramedullary fluid-filled cavities. Shunt placement remains a key treatment option when decompression fails, yet comparative outcomes acr...OBJECTIVE: Syringomyelia is a progressive neurological disorder characterized by intramedullary fluid-filled cavities. Shunt placement remains a key treatment option when decompression fails, yet comparative outcomes across shunt types remain poorly defined. The aim of this study was to compare revision rates, time to failure, and neurological outcomes among patients treated with syringopleural, syringosubarachnoid, and syringoperitoneal shunts. METHODS: A retrospective review was conducted using a prospectively maintained database of the senior surgeon from 1997 to 2025 to identify patients who underwent syrinx shunt placement. Demographic data, shunt type, revision rates, time to revision, and neurological outcomes at early and late follow-up time points were analyzed. Revision was defined as a return to the operating room for shunt-related complications. RESULTS: Thirty-one patients (19 male, mean age 47.2 years) underwent 48 surgeries for placement of syringopleural (n = 20, 41.7%), syringosubarachnoid (n = 21, 43.8%), and syringoperitoneal (n = 7, 14.6%) shunts. Common etiologies included traumatic injury and Chiari malformation. Ten patients (32.2%) required at least one revision surgery, while 21 patients (67.7%) did not require revision. The mean hospital length of stay was significantly longer for syringosubarachnoid (10.1 days) and syringoperitoneal (10.0 days) compared with syringopleural (5.4 days) surgeries (p = 0.036). Revision rates were highest for syringopleural shunts (55.0%), followed by syringoperitoneal (28.6%) and syringosubarachnoid (19.0%) (p = 0.003). Early motor and/or sensory function improvement was observed in 55.0% of syringopleural cases, 33.3% of syringosubarachnoid cases, and 14.3% of syringoperitoneal cases (p = 0.117). Sustained long-term improvement was rare across all groups (p = 0.551). The mean time to revision surgery was 1093 days for syringopleural, 515 days for syringosubarachnoid, and 89 days for syringoperitoneal shunts (p = 0.144). CONCLUSIONS: Syrinx shunting provided modest early neurological benefit, but long-term durability remained limited. Syringopleural shunts demonstrated greater early clinical improvement but carried greater revision risk. Syringosubarachnoid shunts had lower mechanical failure rates but limited clinical efficacy. Optimizing patient selection and vigilant postoperative monitoring are crucial. Further research is needed to refine surgical strategies and improve durable outcomes.
OBJECTIVE: The aim of this study was to evaluate freedom from tumor progression and clinical outcomes in older adults with small- to medium-sized vestibular schwannoma (VS) managed by observation versus stereotactic radi...OBJECTIVE: The aim of this study was to evaluate freedom from tumor progression and clinical outcomes in older adults with small- to medium-sized vestibular schwannoma (VS) managed by observation versus stereotactic radiosurgery (SRS) to better inform optimal management in this patient population. METHODS: In this international multicenter study, patients aged ≥ 60 years with Koos grade I or II VS managed by observation or SRS were retrospectively reviewed. Propensity score matching was conducted using patient characteristics, tumor size, and hearing assessments. Outcome measures of freedom from tumor progression, serviceable hearing preservation (SHP), and neurological function (tinnitus, vestibulopathy, House-Brackmann grade, and trigeminal nerve function) were assessed for both groups. RESULTS: The observation and SRS groups each comprised 51 matched patients (median age 68 years for both). Ipsilateral serviceable hearing was observed at presentation for 35 patients in each group. The median follow-up duration was 39 months in the observation group versus 27 months in the SRS group (p = 0.5). Tumor progression was significantly lower with SRS than with observation (2% vs 52.9%, p < 0.001). The rate of 5-year freedom from tumor progression was 100% in the SRS group versus 43% (95% CI 29%-64%) in the observation group. The rate of 10-year freedom from tumor progression was 90% (95% CI 73%-100%) in the SRS group versus 20% (95% CI 8.5%-49%) in the observation group. At the last follow-up, hearing loss occurred in 42.9% of the observation group and 51.4% of those who underwent SRS (p = 0.5). The 3-year SHP rate was 68% (95% CI 53%-88%) versus 65% (95% CI 49%-85%) (p = 0.8), and the 5-year SHP rate was 53% in both groups (95% CI 36%-78% in the observation group and 95% CI 35%-79% in the SRS group, p = 0.8). The composite endpoint of tumor progression and/or worsened neurological outcome, including hearing loss, tinnitus, vestibulopathy, facial nerve dysfunction (House-Brackmann grade), or trigeminal dysfunction, demonstrated a significantly lower rate in the SRS group (17.6%) compared with the observation group (66.7%) (p < 0.001). CONCLUSIONS: SRS in older patients for management of Koos grade I or II VS resulted in significantly superior rates of freedom from tumor progression, comparable hearing preservation rates, and significantly higher rates of favorable overall radiographic and neurological outcomes than observation alone. Compared with observation, SRS might be the preferred management option in this patient population.
Faquini IV, Alves GDS, Cezar Junior AB
… +9 more, Lemos LEAS, Lages AL, da Silva Junior AB, Barbosa de Moura JF, Gomes EFP, de Lima JES, Monteiro MQ, Ferreira JG, de Lima Cabral WF
OBJECTIVE: Postinfectious hydrocephalus (PIH) is a severe complication following CNS infections and often requires urgent CSF diversion. However, definitive shunt placement procedures are often unfeasible in this setting...OBJECTIVE: Postinfectious hydrocephalus (PIH) is a severe complication following CNS infections and often requires urgent CSF diversion. However, definitive shunt placement procedures are often unfeasible in this setting due to active infection or residual inflammatory debris. In such scenarios, ventriculosubgaleal shunt (VSGS) insertion has emerged as a widely used temporary strategy. METHODS: The authors conducted a systematic search of publications in the PubMed, Embase, and Cochrane Library databases, covering studies published up to June 2025. Risk of bias was assessed using Cochrane's Risk of Bias in Non-randomized Studies of Interventions tool. Data analysis was performed using RStudio (version 2025.05.0). RESULTS: Seven studies involving 225 patients were included in the analysis. Meta-analysis of the reviewed studies revealed that the conversion rate to ventriculoperitoneal shunt placement was 88.6% (95% CI 68.78%-96.48%, I2 = 76.1%). Among the studies, 3 reported the time to conversion, with a 95% CI of 30.13-56.58 days (I2 = 89.4%). Regarding complications, the revision rate was 25.66% (95% CI 19.37%-33.15%, I2 = 0%), the obstruction rate was 13.7% (95% CI 6.34%-27.12%, I2 = 48.5%), the infection rate was 5% (95% CI 1.03%-21.09%, I2 = 68.7%), and the migration rate was 3.87% (95% CI 1.46%-9.87%, I2 = 0%). The overall mortality rate was 13.9% (95% CI 5.21%-32.16%, I2 = 68.2%). CONCLUSIONS: VSGS placement represents an acceptable temporary therapy for PIH. However, more studies with larger cohorts and direct comparisons with other therapeutic strategies are necessary to establish the efficacy and long-term outcomes of VSGS use.
Peeran Z, Shukla P, Tummala T
… +15 more, Kabir A, Osorio RC, Kourkoulakos C, Brumm ZG, Joe I, Lui A, Badani A, Khela HS, Young JS, El-Sayed IH, Gurrola JG, Theodosopoulos P, Blevins L, Kunwar S, Aghi MK
OBJECTIVE: The aim of the study was to evaluate the predictive value of postoperative day 1 (POD1) prolactin on long-term hyperprolactinemia normalization following prolactinoma resection. METHODS: The authors retrospect...OBJECTIVE: The aim of the study was to evaluate the predictive value of postoperative day 1 (POD1) prolactin on long-term hyperprolactinemia normalization following prolactinoma resection. METHODS: The authors retrospectively reviewed 260 prolactinomas that were resected from 1998 to 2020. Patients were classified on the basis of preoperative dopamine agonist (DA) use: the DA-positive group (n = 112 [43.1%]) used DAs < 3 weeks prior to surgery, while the DA-negative group (n = 148 [56.9%]) did not. The prolactin level (ng/ml) was categorized as follows: low normal (0-10 ng/ml for men, 0-12.5 ng/ml for women), high normal (10-20 ng/ml for men, 12.5-25 ng/ml for women), and mildly hyperprolactinemic (20-40 ng/ml for men, 25-50 ng/ml for women). Prolactins were analyzed at 6 time points: POD1, 1 day to 6 weeks, 6-12 weeks, 12 weeks to 6 months, 6 months to 1 year, and > 1 year. The authors identified cases with POD1 normalization, followed by hyperprolactinemia requiring treatment, multiple elevated prolactins, or hyperprolactinemia at latest follow-up, defining rebound hyperprolactinemia as these events that occurred < 1 year of surgery and recurrent hyperprolactinemia as these events that occurred ≥ 1 year after surgery. Remission was defined as persistent normalization during the 1st year. RESULTS: The mean (SD) age was 35.4 (11.4) years. In total, 78.1% (n = 203) of patients were female. In the DA-negative group, 78.9% of mildly hyperprolactinemic patients had persistent elevation, while 90.2% and 37.0% of low- and high-normal patients achieved remission, respectively. For the DA-positive group, 81.8% of mildly hyperprolactinemic DA-positive patients remained with elevated prolactin levels, whereas 72.6% and 50.0% of low- and high-normal patients experienced remission, respectively. Rebound rates were 2.0% versus 33.3% in low-normal versus high-normal DA-negative patients and 12.6% versus 33.3% in low-normal versus high-normal DA-positive patients. Recurrence rates were 7.8% versus 25.9% in low-normal versus high-normal DA-negative patients and 11.6% versus 16.7% in low-normal versus high-normal DA-positive patients. The cumulative incidence rates of rebound (p < 0.001) and recurrent (p < 0.001) hyperprolactinemia varied by group, with low-normal DA-negative patients being the least likely to experience either outcome. For DA-positive (p < 0.001) and DA-negative (p < 0.001) patients, retreatment during the 1st year after surgery was more often required in mildly hyperprolactinemic (47.4% of DA-negative patients vs 81.8% of DA-positive patients) and high-normal (18.5% DA-negative vs 33.3% DA-positive) patients than low-normal patients (1.0% DA-negative vs 18.1% DA-positive). CONCLUSIONS: POD1 prolactin is associated with long-term normalization following prolactinoma resection. However, patients should not be considered cured solely on the basis of the POD1 prolactin level, as rebound hyperprolactinemia can occur, particularly with recent DA use or a high-normal POD1 prolactin level.
OBJECTIVE: The optimal order of treatment with programmed cell death protein 1 (PD-1) inhibitor immunotherapy and stereotactic radiosurgery (SRS) in malignant gliomas remains unclear. This study examined whether the timi...OBJECTIVE: The optimal order of treatment with programmed cell death protein 1 (PD-1) inhibitor immunotherapy and stereotactic radiosurgery (SRS) in malignant gliomas remains unclear. This study examined whether the timing of PD-1 inhibitor administration relative to SRS affects patient neurological outcomes and survival in a multi-institutional propensity score-matched cohort. METHODS: A retrospective analysis was conducted using the TriNetX Research Network database (2005-2022), identifying adults with malignant gliomas who received SRS and at least one PD-1 inhibitor. Patients were stratified into two cohorts based on whether PD-1 inhibition occurred 8 weeks before SRS (pre-SRS) versus after SRS (post-SRS). Propensity score matching (1:1) was performed for demographics, comorbidities, and relevant medications. Clinical outcomes, including mortality, cognitive impairments, cranial nerve deficits, seizures, hemorrhagic complications, and functional impairments, were assessed over a 3-year follow-up period. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated, and Kaplan-Meier (KM) survival analyses were used to compare cumulative incidence rates. RESULTS: Before matching, the pre-SRS (n = 429) versus post-SRS (n = 381) cohorts differed significantly in sex (female, unknown), ethnicity (Hispanic/Latino, unknown), race (Asian, American Indian, other), overweight/obesity, type 2 diabetes mellitus, lipidemia, anticoagulant use, durvalumab use, and atezolizumab use (all p < 0.05). After propensity score matching (n = 233 per group), PD-1 inhibition pre- versus post-SRS showed no significant differences in baseline characteristics except for age (mean 63.50 [SD 11.84] years vs 63.36 [SD 10.82] years, p = 0.012) and tremelimumab use (n = 0 [0%] vs n = ≤ 10 [≤ 4.29%], p = 0.001). In propensity score-matched patients, PD-1 inhibitor use before SRS was associated with significantly higher odds of cognitive impairments (OR 1.75, 95% CI 1.04-2.98, p = 0.034), cranial nerve deficits (OR 2.00, 95% CI 1.11-3.69, p = 0.021), and mortality (OR 1.53, 95% CI 1.06-2.20, p = 0.023), while other outcomes including behavioral/mood changes (OR 0.91, p = 0.726), gait/coordination disorders (OR 1.28, p = 0.421), hemorrhagic complications (OR 0.97, p = 0.930), motor deficits (OR 0.86, p = 0.590), seizures/epilepsy (OR 0.99, p = 0.971), and sensory disturbances (OR 0.91, p = 0.737) showed no significant differences. KM analyses confirmed elevated risks of cognitive impairment (p = 0.0076), cranial nerve deficits (p = 0.0104), and mortality (p = 0.0109) in the pre-SRS group. CONCLUSIONS: Pre-SRS PD-1 inhibitor use was associated with more neurological deficits and lower survival, highlighting the need for prospective studies on optimal SRS immunotherapy timing in malignant gliomas.
Elkaim LM, Chen JS, Abecassis IJ
… +11 more, Sigal A, Ibrahim GM, Fallah A, Venne D, Amlie-Lefond C, Hadjinicolaou A, Jabre R, Kaseka ML, Ellenbogen RG, Wang AC, Weil AG
OBJECTIVE: Moyamoya arteriopathy is a leading cause of cerebral ischemia in children. In pediatric patients with symptomatic or progressive moyamoya disease, surgical revascularization is the mainstay of treatment; howev...OBJECTIVE: Moyamoya arteriopathy is a leading cause of cerebral ischemia in children. In pediatric patients with symptomatic or progressive moyamoya disease, surgical revascularization is the mainstay of treatment; however, there is limited comparative evidence demonstrating an advantage of surgical revascularization over nonsurgical management in North American cohorts. The authors aimed to determine whether surgery reduces the risk of subsequent ischemic events in North American children with symptomatic ischemic moyamoya arteriopathy compared to nonsurgical management alone. METHODS: Patients treated conservatively at the Sainte-Justine University Hospital Center in Montréal, Canada, were compared to patients treated surgically through indirect revascularization, predominantly performed at the Seattle Children's Hospital. Data were acquired via retrospective review of patients treated at both institutions. Time to event analysis via multivariable Cox regression for postoperative ipsilateral ischemic stroke or transient ischemic attack (TIA) recurrence was performed. RESULTS: Forty-nine patients with a median (IQR) age of 6.0 (3.0-9.0) years were included. Twenty-four patients (49.0%) were treated nonsurgically, while 25 patients (51.0%) were treated surgically. Among the 42 patients with subtype data available, 15 (35.7%) had moyamoya disease and 27 (64.3%) had moyamoya syndrome. On Kaplan-Meier analysis, patients who underwent nonsurgical management had 12-, 24-, and 60-month recurrence-free rates from an ipsilateral ischemic event of 52.8%, 52.8%, and 31.7%, respectively. In contrast, patients who underwent surgical management had 12-, 24-, and 60-month recurrence-free rates from an ipsilateral ischemic event of 87.7%, 83.0%, and 83.0%, respectively. In the entire cohort, surgical intervention (HR 0.15, 95% CI 0.04-0.51) and White race (HR 0.18, 95% CI 0.06-0.56) were both independently associated with longer time to ipsilateral ischemic event recurrence. CONCLUSIONS: Pediatric patients with symptomatic ischemic moyamoya arteriopathy treated nonsurgically had worse outcomes compared with those managed surgically. These findings support pursuing surgical revascularization therapy in North American children and youth with symptomatic ischemic moyamoya arteriopathy.
OBJECTIVE: Awake resection in grade 2 glioma (G2G) improves quality of life (QOL) and overall survival (OS). Nonetheless, epilepsy occurs frequently in this context and can impair QOL, especially when the disorder is pha...OBJECTIVE: Awake resection in grade 2 glioma (G2G) improves quality of life (QOL) and overall survival (OS). Nonetheless, epilepsy occurs frequently in this context and can impair QOL, especially when the disorder is pharmacoresistant. Herein, the goal was to study patients with intractable epilepsy (IE) before and/or after surgery for a G2G. METHODS: Patients who underwent awake functional mapping-based resection of an IDH-mutant G2G in the period from June 2002 to March 2024 and had IE before and/or after surgery (follow-up > 1 year) were selected for this retrospective study. Onco-functional outcomes were compared among patients with preoperative IE who were completely seizure free (Engel class IA) postoperatively (group 1), patients with preoperative and postoperative IE (group 2), and patients who experienced IE only postoperatively (group 3). RESULTS: In a consecutive series of 105 patients (61 males [58.1%]) with a mean age of 35.6 ± 12.2 years, 134 awake surgeries were performed. At diagnosis, 101 patients (96.2%) presented with seizures, and 82 patients (78.1%) presented with IE. The mean preoperative Karnofsky Performance Status (KPS) was 88.7 ± 7.4, and 74 patients (70.5%) were able to work preoperatively. Sixty-three gliomas (60%) were left-sided and 42 were right-sided, which consisted of 66 insula-centered/paralimbic (62.8%), 13 central (12.4%), 14 frontal (13.3%), 6 temporal (5.7%), and 6 parietal (5.7%) tumors. The mean preoperative tumor volume was 82.8 ± 47.9 cm3. Only 1 patient (0.9%) had persistent postoperative deterioration, and the mean postoperative KPS was 89.2 ± 6.4. Sixty-one patients returned to work (82.4%). The mean extent of resection (EOR) was 86.8% ± 8.3% (mean residual volume 13 ± 18.4 cm3). Histopathologically, there were 65 astrocytomas (61.9%) and 40 oligodendrogliomas (38.1%). Twenty-two patients (21.0%) had immediate adjuvant therapy, and 29 patients (27.6%) underwent reoperation(s). The mean follow-up was 8.3 ± 4.7 years with an OS rate of 70.5%. Sixty patients (57.1%) were completely seizure free after surgery (group 1), and 45 patients (42.8%) had postoperative IE (22 patients [21.0%] in group 2 and 23 patients [21.9%] in group 3). The proportion of insula-centered/paralimbic G2Gs was higher in group 1 (p = 0.002), whereas the percentage of central G2Gs was greater in groups 2 and 3 (p = 0.01). Higher preoperative tumor volume (p < 0.00001) and lower EOR (p = 0.05) were correlated to IE. The postoperative KPS (p < 0.00002) and return to work (RTW) rate (p = 0.0004) were higher in group 1. CONCLUSIONS: These original findings show that G2G location, tumor volume, and EOR are associated with perioperative IE, itself correlated to QOL, especially KPS and RTW. Such data may help neurosurgeons better evaluate the epilepto-onco-functional balance of surgery in G2G.
OBJECTIVE: Tethered cord syndrome (TCS) is heterogeneous, and a filum with lipomatous tissue can present in multiple patterns. The aim of this study was to determine whether filum characteristics are associated with part...OBJECTIVE: Tethered cord syndrome (TCS) is heterogeneous, and a filum with lipomatous tissue can present in multiple patterns. The aim of this study was to determine whether filum characteristics are associated with particular symptomatology. METHODS: A descriptive cross-sectional retrospective study was conducted over 2 years (2023-2025) at a single institution. Patients with TCS and filum with lipomatous tissue were evaluated using 24 TCS-related variables and 10 filum-related variables. Statistical methods included using the chi-square test and ANOVA for frequency differences, multivariable logistic regression with odds ratios, and statistical weight analysis. RESULTS: Sixty-eight patients (35 male, mean age 9.01 years) were included in the analysis. A significantly younger age at diagnosis was seen in patients with proximal lipomatous tissue (mean 4.67 ± 3.90 years), lipomatous tissue > 3 cm (mean 7.07 ± 3.17 years), conus medullaris below L2 (mean 6.43 ± 3.74 years), and syrinx (mean 5.42 ± 3.03 years). Vesicourethral dyssynergia was more frequent in patients with filar lipoma versus lipomatous filum (58.3% vs 26.8%), those with complete conus versus caudal regression (63.6% vs 26.3%), and those with vertebral dysraphism versus without (44.4% vs 18.8%). In the multivariate analysis, smaller total lipomatous tissue was associated with less overactive bladder (OR 0.04 and 0.044). Conus medullaris below L2 (OR 31.5) and proximal lipomatous tissue (OR 18.1) were related to underactive bladder. Proximal lipomatous tissue (OR 26.2) and low-lying conus (OR 9.8) were associated with high-risk neurogenic bladder. Lipomatous filum < 1 cm was associated with idiopathic scoliosis (OR 3.25). Distal lipomatous tissue interactions such as filum ≤ roots with distal lipomatous tissue (OR 121.20), dural adhesion with distal lipomatous tissue (OR 43.90), and yin-yang signal pattern with distal lipomatous tissue (OR 19.40) were associated with foot deformities. The yin-yang signal intensity pattern on MRI and vertebral dysraphism (OR 5.8) increased the likelihood of moderate-to-severe sciatic pain. CONCLUSIONS: Filum patterns were associated with features of TCS, supporting classification of the filum based on lipomatous tissue.
OBJECTIVE: The aim of this study was to evaluate whether indirect revascularization is associated with a reduction in systemic blood pressure (BP) over time in pediatric patients with moyamoya. METHODS: This is a retrosp...OBJECTIVE: The aim of this study was to evaluate whether indirect revascularization is associated with a reduction in systemic blood pressure (BP) over time in pediatric patients with moyamoya. METHODS: This is a retrospective cohort study of pediatric patients with moyamoya who were treated at a single institution between June 2019 and January 2025. BP measurements were collected preoperatively (preadmission clinic evaluation) and postoperatively (outpatient follow-up evaluation at ≥ 3 months). BP was categorized as hypertensive or normotensive based on percentile thresholds adjusted for age, sex, and height, with measurements at the 95th and 99th percentiles indicating hypertension. The chi-square test was used to assess changes in hypertensive classification, and the paired t-test was used to compare mean absolute BP values at the two time points. RESULTS: Overall, 63 children (39 female, mean age 11 years) with moyamoya who underwent indirect revascularization surgery were included in this analysis. The mean follow-up duration was 20.8 months. Preoperatively, 21 patients (33.9%) were classified as hypertensive for systolic BP and 9 (14.5%) for diastolic BP. These measurements were acquired preoperatively at a mean of 3.5 months (range 1 day to 11 months). Postoperatively, the proportion of hypertensive patients decreased to 10 (16.1%) and 3 (4.8%) for systolic BP and diastolic BP (p = 0.02 and 0.06, respectively). There was no significant change between the preoperative and postoperative mean arterial pressure measurements (77.3 mm Hg vs 76.2 mm Hg, respectively; p = 0.52). CONCLUSIONS: Indirect revascularization surgery in pediatric patients with moyamoya was associated with a reduction in hypertension over time. This might reflect the delayed physiological effect of indirect revascularization, in which neovascularization and improved cerebral perfusion typically develop 3-6 months after surgery. Understanding BP changes following surgery might help avoid unnecessary use of antihypertensive drugs, which carry risk of precipitating hypotension and thus stroke.
OBJECTIVE: Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) has decreased rates of shunt dependence in infants with hydrocephalus. The ETV Success Score (ETVSS) is the standard for predicting...OBJECTIVE: Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) has decreased rates of shunt dependence in infants with hydrocephalus. The ETV Success Score (ETVSS) is the standard for predicting the 6-month success rate for ETV in children based on age, hydrocephalus etiology, and shunt history. However, the ETVSS does not account for the impact of CPC or preoperative ventricular volume. It also relies on the independence of each contributing variable and does not predict success beyond 6 months. In this study, the authors used a machine learning approach to create a tool specifically for ETV/CPC to predict the likelihood of success at 1 year. METHODS: The records of 206 pediatric patients younger than 2 years of age who received ETV/CPC as a primary, definitive treatment for hydrocephalus at a single institution between 2009 and 2021 were reviewed for patient demographics, presenting characteristics, and medical history. Data on corrected age at surgery, frontal occipital horn ratio (FOHR), hydrocephalus etiology, and whether there was prior CSF diversion were used in developing logistic regression, XGBoost, random forest, and gradient boosting algorithms to predict the percentage likelihood of ETV/CPC failure within postoperative year 1. RESULTS: Unlike the ETVSS, etiology of hydrocephalus did not substantially influence any model's predictions and was removed. A logistic regression model produced the best area under the receiver operating characteristic curve (AUROC) and was used as the final model. Three features selected for the final model (corrected age, prior CSF diversion, and FOHR) proved relevant. The logistic regression predictor had an AUROC of 0.85 (compared to 0.66 for the ETVSS) and an outcome classification accuracy of 76%. The model correctly classified its predictions for success slightly more accurately than its predictions for failure (sensitivity 78%, specificity 75%). CONCLUSIONS: This model can reliably predict the likelihood of ETV/CPC failure at 1 year. Its outperformance of the ETVSS is likely due to the impact of CPC, the novel use of preoperative ventricle size as a predictive parameter, and accounting for variable interdependence. Further validation in additional patient populations is needed.
OBJECTIVE: Insular gliomas present a unique surgical challenge due to their location near critical vascular structures and proximity to functional brain regions. While different surgical approaches to insular tumors exis...OBJECTIVE: Insular gliomas present a unique surgical challenge due to their location near critical vascular structures and proximity to functional brain regions. While different surgical approaches to insular tumors exist, language and motor stimulation mapping are critical for a transcortical approach. The aim of this study was to determine if the transcortical approach to resection of insular gliomas remains a safe and effective strategy by assessing tumor characteristics, surgical outcomes, and postoperative functional outcomes in patients with newly diagnosed or recurrent tumors. METHODS: This retrospective analysis included 502 newly diagnosed and recurrent low-grade gliomas (LGGs) and high-grade gliomas (HGGs) of the insula (in 394 unique patients) resected between September 1997 and December 2022 at a single center. Tumors were classified based on the Berger-Sanai zone schema, and contrast-enhancing and non-contrast-enhancing tumor volumes were manually segmented on MRI and used to calculate the extent of resection (EOR). Patient morbidity was assessed at multiple time points from the initial presentation to at least 6 months of follow-up. Progression-free survival (PFS) and overall survival (OS) were compared between subgroups using unadjusted and propensity score-adjusted Kaplan-Meir and Cox regression analyses. RESULTS: Overall, 316 (165 LGG, 151 HGG) newly diagnosed and 186 (69 LGG, 117 HGG) recurrent cases were included. Grade 2 gliomas were typically larger than grade 4 IDH-wildtype gliomas (43 cm3 vs 17.5 cm3, p < 0.001). Persistent postoperative motor and language deficits occurred in < 4% of cases with newly diagnosed grade 2 tumors, although transient deficits occurred more frequently (9.5% of cases with transient motor deficit and 20% of cases with transient language deficit). For patients with newly diagnosed grade 2 insular gliomas, OS was improved when the residual tumor volume was < 2.7 cm3. Minimizing residual tumor volume was also associated with prolonged PFS and OS for recurrent grade 2 insular gliomas. Similarly, contrast-enhancing tumor EOR > 88.6% was associated with improved PFS and OS for patients with newly diagnosed IDH-wildtype glioblastoma. Overall, surgical and medical complications occurred in < 3% of cases. Finally, new permanent arm or leg weakness was significantly associated with worse OS in multivariable analyses (HR 2.06, 95% CI 1.14-3.74; p = 0.017). CONCLUSIONS: Maximum safe resection using a transcortical approach and cortical and subcortical mapping continues to be a robust surgical strategy with low surgical morbidity for patients with newly diagnosed and recurrent insular gliomas.
OBJECTIVE: The aim of this study was to evaluate the long-term efficacy and safety of Gamma Knife surgery (GKS) for sporadic vestibular schwannoma (VS), focusing on the durability of tumor control and the necessity of ex...OBJECTIVE: The aim of this study was to evaluate the long-term efficacy and safety of Gamma Knife surgery (GKS) for sporadic vestibular schwannoma (VS), focusing on the durability of tumor control and the necessity of extended posttreatment imaging surveillance. METHODS: This retrospective study included patients with sporadic VS treated with single-session GKS between May 1991 and January 2020 at a single center. Tumors were classified into the following anatomical types using a modified Koos-based system: type A (intracanalicular), type B (cerebellopontine angle), type C (mild brainstem compression), and type D (severe brainstem compression with fourth ventricle deviation). A subgroup analysis in patients without salvage treatment within the first 5 years after GKS and with ≥ 5 years of follow-up was performed to assess long-term stability. Salvage treatment rates, functional outcomes, and adverse events were evaluated. RESULTS: Overall, 878 patients (488 female, median age 57 years) with sporadic VS treated with single-session GKS were included in the analysis. A subgroup of 793 patients (438 female, median age 58 years) remained free of salvage treatment during the first 5 years after GKS and had ≥ 5 years of follow-up. The median clinical follow-up duration was 154.5 months. Salvage treatment was required in 7.5% of the patients, with a significantly higher incidence in patients with type D tumors (24.7% at 5 years) compared with type A-C tumors (3.6%) (subdistribution hazard ratio 2.319, p = 0.036). In the subgroup of patients with stable disease, the cumulative incidence of salvage treatment at 15 years was 2.1%, and true tumor progression was identified in only 2 patients (0.3%). Notably, tumor type at the time of GKS did not significantly influence the risk of salvage treatment beyond 5 years in the subgroup. Serviceable hearing preservation improved in more recent treatment periods: patients with pre-GKS Gardner-Robertson class 1 hearing had a 10-year preservation rate of 63% in the late treatment period (2005-2023) compared with 48% in the early period (1991-2004). Among late adverse events, cyst-related complications were the most common (3.1%), whereas trigeminal neuralgia (1.6%), persistent facial palsy (0.1%), and malignant transformation (0.2%) were infrequent. CONCLUSIONS: GKS provided durable long-term tumor control and functional preservation in patients with small- to medium-sized VS. True tumor progression beyond 10 years was not observed in this cohort, suggesting that the intensity or frequency of routine imaging surveillance can be reduced or individualized after 10 years in patients with stable disease during the first 5 years after GKS. However, because delayed adverse events (e.g., cyst formation, trigeminal neuralgia, and malignant transformation) can still occur, follow-up strategies should be tailored according to initial tumor characteristics and the posttreatment clinical course.
OBJECTIVE: Advancements in medulloblastoma management have improved survival; however, high-risk metastatic cases remain challenging, with approximately 60% 5-year event-free survival and significant long-term toxicity....OBJECTIVE: Advancements in medulloblastoma management have improved survival; however, high-risk metastatic cases remain challenging, with approximately 60% 5-year event-free survival and significant long-term toxicity. Standard treatment includes resection of the posterior fossa tumor, followed by multimodal oncological therapy. Yet, primary tumor resection can result in treatment delay and sometimes surgical morbidity. The aim of this study was to evaluate outcomes and assess the potential of a treatment approach that includes biopsy only followed by chemotherapy and radiation therapy as a viable alternative in selected clinical scenarios. METHODS: This retrospective study included pediatric patients (age < 18 years) who were diagnosed with metastatic medulloblastoma and underwent biopsy (with or without CSF diversion) without primary tumor resection at a tertiary pediatric center between 2010 and 2023. Clinical, surgical, pathological, molecular, and imaging data were analyzed. Tumor response was evaluated on MRI. RESULTS: During the study period, 60 patients with medulloblastoma were treated at the medical center; 12 male patients (mean age 6.5 years, range 1.1-16.1 years) with metastatic disease who were treated with the upfront biopsy-only approach met the inclusion criteria. The median follow-up duration was 3.2 years. At the time of analysis, 9 patients (75%) were alive, with an estimated 5-year survival rate of 63%, and 3 patients had died (2 with very high-risk MYC-amplified tumors and 1 with a late supratentorial relapse). No cases of posterior fossa syndrome were observed. All surviving patients showed stable or resolving residual abnormalities on MRI without progressive disease. CONCLUSIONS: In pediatric patients with metastatic medulloblastoma, primary tumor resection might be avoidable. A biopsy-based approach followed by timely multimodal therapy can preserve survival outcomes while minimizing surgical risks, as long-term prognosis is likely related to the disease subtype and prompt oncological treatment. The proposed strategy warrants further investigation and might have broader implications for medulloblastoma treatment paradigms.
OBJECTIVE: Rathke's cleft cysts (RCCs) and craniopharyngiomas (CPs) are lesions of the sellar region, both originating from remnants of Rathke's diverticulum. The presence of squamous metaplasia (SM) within RCCs often cr...OBJECTIVE: Rathke's cleft cysts (RCCs) and craniopharyngiomas (CPs) are lesions of the sellar region, both originating from remnants of Rathke's diverticulum. The presence of squamous metaplasia (SM) within RCCs often creates histological overlaps with papillary CPs (PCPs), complicating accurate diagnosis. Given the distinct treatment and clinical outcomes associated with these lesions, precise identification is essential. BRAF V600E and CTNNB1 mutations have emerged as distinguishing genetic markers for PCP and adamantinomatous CP (ACP), respectively. This study aimed to evaluate the utility of BRAF V600E and β-catenin immunohistochemistry in differentiating RCC from CP. METHODS: The authors retrospectively reviewed the clinical, radiological, and histopathological data of 383 RCCs diagnosed between January 2015 and May 2024 at Beijing Tiantan Hospital. BRAF V600E and CTNNB1 immunohistochemistry were performed on all cases, with BRAF results confirmed via Sanger sequencing. Clinical outcomes were evaluated during follow-up. RESULTS: Sixty-nine RCC cases met the inclusion criteria (61 primary and 8 recurrent). Of these, 52 cases were in intrasellar region (75.4%), with 9 cases (13.0%) in the suprasellar region and 8 cases (11.6%) involving both regions. Histologically, 39 cases (56.5%) exhibited epithelial SM. No nuclear β-catenin accumulation localization was detected. BRAF V600E expression was observed in 7 cases (10.1%), all within areas of SM, and confirmed with Sanger sequencing. These positive cases also showed elevated Ki-67 indices, with proliferative activity concentrated at the basal layer of the epithelium with SM. Based on these molecular and histological findings, the 7 BRAF V600E-positive cases were reclassified as PCPs. Notably, Kaplan-Meier analysis demonstrated significantly worse progression-free survival in BRAF V600E-positive cases compared to wild-type cases (p = 0.023). CONCLUSIONS: BRAF V600E and CTNNB1 mutation analysis is a valuable diagnostic tool for distinguishing RCC from CP. Given the potential for RCC to transform into PCP, the authors recommend BRAF V600E testing for all RCC cases. For BRAF V600E-positive cases, close monitoring of tumor progression or adjuvant therapies is advised.
OBJECTIVE: Protocols for intraoperative mapping during awake craniotomy vary widely. The aim of this study was to evaluate how the number and type of intraoperative neuropsychological paradigms administered via NeuroMapp...OBJECTIVE: Protocols for intraoperative mapping during awake craniotomy vary widely. The aim of this study was to evaluate how the number and type of intraoperative neuropsychological paradigms administered via NeuroMapper, a tablet-based platform, during awake glioma surgery affect positive mapping rates, return to work (RTW), and oncological outcomes. Factors associated with the operative duration and early postoperative neurological deficits were of additional interest. METHODS: This single-center retrospective study included patients with diffuse low- and high-grade gliomas who underwent awake craniotomy with NeuroMapper-guided mapping between 2018 and 2024. Primary outcomes were RTW, progression-free survival (PFS), and overall survival (OS); operative duration and early postoperative deficits were secondary outcomes. Covariate balancing propensity score (CBPS) analysis was used to address confounding by tumor grade, with all postweighting standardized mean differences < 0.10. A weighted logistic regression model assessed RTW, and CBPS-weighted Cox models assessed PFS and OS. Linear regression was used to analyze intraoperative variables, operative duration, and early postoperative deficits. RESULTS: Of 189 patients included in the analysis, 65 had LGG (median age 39 years) and 124 had HGG (median age 56 years). Mapping complexity varied, with 1-6 tasks used (median 4). Weighted multivariate logistic regression analysis identified the following independent predictors of RTW: preoperative Karnofsky Performance Status score ≥ 80 (OR 1.37, p = 0.039), absence of recurrent disease (OR 0.30, p < 0.001), use of 5-6 paradigms (OR 1.54, p = 0.047), positive mapping (OR 2.41, p = 0.017), and subtotal resection (OR 4.43, p = 0.002). Intraoperative seizure, early postoperative deficit, and the resection technique were not significant. IDH mutation status was associated with improved PFS (HR 0.15, p < 0.001), while subtotal resection was associated with worsened PFS (HR 3.00, p < 0.001). Positive mapping was associated with better OS (HR 0.57, p = 0.04). The operative duration was unaffected by mapping variables but was longer in patients with obesity (β = 0.28, p = 0.038). CONCLUSIONS: Positive NeuroMapper-guided mapping of more diverse linguistic functions was associated with RTW and longer OS. Additionally, mapping complexity did not prolong surgery times, supporting potential standardization.