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Purpose: Hemispherotomy is an effective surgery for intractable pediatric hemispheric epilepsy. Over the years, the surgical goal has shifted from a complete hemispheric resection (anatomical hemispherectomy) to a disconnective hemispherotomy (DH). Multiple techniques for DH have been described, and often, anterior temporal lobectomy (ATL, with hippocampal resection) is performed. The goal of the current study is to assess the role of hippocampal resection in DH. Methods: We retrospectively collected all clinical data of children (< 18 years old) who underwent DH between 2001 and 2022 at two tertiary large centers. Epilepsy status and surgical outcome were compared, based on whether the hippocampus was resected (as part of an ATL) or disconnected at the amygdala and atrial segment of the fornix (with no ATL). Results: A total of 86 patients (32 females) were included. The most common epilepsy etiologies were stroke (31), Rasmussen's encephalitis (16), cortical dysplasia (10), and hemimegaloencephaly (9). The mean age at surgery was 7 (± 4.9) years. The average number of anti-seizure medications (ASMs) at surgery was 3 (± 1.2). Hemispherotomy techniques included peri-insular (54), vertical (23 [19 endoscopic + 4 parasagittal]), and trans-sylvian (9). The mean follow-up was 41.5 (± 38) months. Forty-three patients had hippocampal resection, and 43 patients had a hippocampal disconnection. Both groups had similar Engel outcome scores (p = 0.53). Conclusions: Disconnective hemispherotomy is highly effective for pediatric intractable hemispheric epilepsy. Our data suggest that the inclusion of hippocampal resection does not provide additional benefit. [ABSTRACT FROM AUTHOR]

• Rasmussen syndrome (RS) currently lacks detailed diagnostic/treatment algorithms. • The survey highlights trends and significant variations in RS management. • Variations in practice are targets for research and consensus guidelines in RS. Rasmussen syndrome (RS) is marked by progressive unihemispheric atrophy, resulting in hemiparesis, refractory epilepsy, and cognitive/language decline. Detailed diagnostic and treatment algorithms are currently lacking. We aimed to survey medical providers on their current practices in the diagnosis and treatment of RS. A steering committee was formed to create the survey, which was disseminated to the international medical community. One hundred twelve surveys were completed. Descriptive statistics, as well as comparisons by level of experience, patient age group cared for, and geographic region using Fisher's exact test, were conducted. Analysis of cerebrospinal fluid (82 %) and serum (78 %) for autoimmune encephalitis (AE) are completed by most, while approximately one-third obtain genetic and metabolic studies in all patients (36 % and 38 %, respectively). Providers in US and Europe more readily pursue serum AE antibody panels (85 % and 85 %, respectively, versus 67 %, p = 0.019) and genetic testing (56 % and 47 %, respectively, versus 14 %, p < 0.001) than the rest of the world. Thirty-six percent proceed to biopsy in patients otherwise meeting diagnostic criteria, and US providers are more likely to suggest this than others (73 % versus 14–41 %, p < 0.001). Opinions differed on the prioritization of hemispherectomy/hemispherotomy versus immunotherapy in 14 clinical scenarios with various neurologic deficit severity provided. Preferred immunotherapy regimens also varied, with US providers more often choosing IVIG as first-line (67 %) compared to others (28 %-32 %, p = 0.030). Surgical standard of care was identified as functional hemispherectomy or hemispherotomy by 90 %. The survey highlights trends but also significant variations in clinical practice that can serve as targets for future research and expert consensus guidelines. [ABSTRACT FROM AUTHOR]

Introduction: Hemispheric surgery is a multistep, highly effective, and radical surgical procedure in the treatment of drug-resistant epilepsy due to extensive unilateral hemispheric disease. The procedure ranges from a resective procedure (hemispherectomy) to disconnection (hemispherotomy) and has developed substantially over the last century from morbid to elegant, minimally invasive, and routinely practiced procedures. Bearing in mind the numerous articles that have been published on hemispherectomy and hemispherotomy, we aimed to highlight the top 100 cited and impactful articles to create familiarity with the topic. We anticipate that this will be a helpful guide for clinicians and academics navigating the literature on this subject. Methods: A Scopus title-based search on the top 100 most-cited articles on "hemispherectomy" and "hemispherotomy" was performed in September 2023 with no restrictions. The top 100 most-cited articles were then retrieved. The article title, first author, first author's specialty, country of origin, first author's institution at the time of publication, journal of publication, year of publication, citation count, and citations per year were collected. The Google Scholar database citation count for each paper was added for correlation and comprehensive coverage. Results: The top 100 most-cited articles were cited 92 times per paper on average. The publication dates ranged from 1949 to 2016. The most frequently cited article "Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence" with 307 citations was published by A.M. Devlin et al. (2003) in the journal Brain. The USA was the highest publishing country (41 articles). The highest-publishing journal was Neurology. The most prolific first authors were A. Smith, J. Schramm, and J. Villemure, each with four publications. The institution with the most contributions was McGill University and its affiliated Health Centers, with nine publications in total. Neurosurgery was the most common specialty among the first authors. Most of the included studies were cohort studies or case series. Conclusion: We identified the top 100 cited articles on hemispherectomy and hemispherotomy using the Scopus database and supplemented our results with Google Scholar. We highlighted the most prominent authors, institutions, countries, journals, and study designs and illuminated the historical development of hemispherectomy and hemispherotomy procedures, in addition to landmark and currently trending papers. [ABSTRACT FROM AUTHOR]

Background: Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive seizures and unilateral cerebral atrophy with onset during childhood and unknown etiology. When medical therapy appears refractory, surgical disconnection of the affected hemisphere is indicated. Quality of life after functional hemispherectomy is largely good, affected females may therefore pursue pregnancy. However, data on pregnancy and delivery in RE post hemispherectomy is extremely rare. Case presentation: We present the case of a patient with left functional hemispherectomy for RE at the age of seven, who experienced two successful pregnancies. In both pregnancies, her post-surgical symptoms including right-sided spasticity, cephalgia, dizziness, and impairment of vision and speech deteriorated but improved to pre-pregnancy level after delivery. Neurologic sequelae post-hemispherectomy overlapped with clinical signs of preeclampsia and required close diagnostic surveillance during both pregnancies. Conclusion: There are no data on the interaction between RE, hemispherectomy and pregnancy, making maternal and fetal risk assessment difficult. Due to the complexity of the condition and symptoms, management of RE in pregnancy remains highly challenging and requires an interdisciplinary approach. This is the first case description of two successful pregnancies in a woman with RE and status post-hemispherectomy. Further evidence is urgently required to improve counseling and management of affected women. [ABSTRACT FROM AUTHOR]

Rasmussen's encephalitis is a rare neurological pathology affecting one cerebral hemisphere, therefore, posing unique challenges. Patients may undergo hemispherectomy, a surgical procedure after which cognitive development occurs in the isolated contralateral hemisphere. This rare situation provides an excellent opportunity to evaluate brain plasticity and cognitive recovery at a hemispheric level. This literature review synthesizes the existing body of research on cognitive recovery following hemispherectomy in Rasmussen patients, considering cognitive domains and modulatory factors that influence cognitive outcomes. While language function has traditionally been the focus of postoperative assessments, there is a growing acknowledgment of the need to broaden the scope of language investigation in interaction with other cognitive domains and to consider cognitive scaffolding in development and recovery. By synthesizing findings reported in the literature, we delineate how language functions may find support from the right hemisphere after left hemispherectomy, but also how, beyond language, global cognitive functioning is affected. We highlight the critical influence of several factors on postoperative cognitive outcomes, including the timing of hemispherectomy and the baseline preoperative cognitive status, pointing to early surgical intervention as predictive of better cognitive outcomes. However, further specific studies are needed to confirm this correlation. This review aims to emphasize a better understanding of mechanisms underlying hemispheric specialization and plasticity in humans, which are particularly important for both clinical and research advancements. This narrative review underscores the need for an integrative approach based on cognitive scaffolding to provide a comprehensive understanding of mechanisms underlying the reorganization in Rasmussen patients after hemispherectomy. [ABSTRACT FROM AUTHOR]

Rasmussen's encephalitis (RE) is a progressive inflammatory neurological process most commonly occurring in children characterized by drug-resistant focal epilepsy, hemiplegia, and cognitive decline, with brain atrophy and white matter changes typically localized to 1 hemisphere of the brain. Because the clinical course of RE is often indistinguishable from a variety of medical conditions, MRI has historically been the primary diagnostic tool. Here, we report both the clinical course and progression of neuroimaging findings of a 5-year-old female who had very subtle early cortical and white matter changes on MRI and was diagnosed with RE by correlating the clinical presentation, imaging, and electrographic findings.

Hemispherotomy is a surgical procedure aimed at the treatment of hemispheric epilepsy. Hemispherotomy disconnects the commissural fibers, projecting fibers, and limbic system while preserving most of the brain parenchyma, unlike conventional hemispherectomy. 1 Hemispherotomy is one of most complicated operations currently used in epilepsy surgery and requires a high-level understanding of the intrinsic neuroanatomy. Generally, 2 main techniques are applied in hemispherotomy: lateral hemispherotomy and vertical hemispherotomy. 2,3 Vertical hemispherotomy was developed after the lateral technique. Despite a deeper, narrower surgical corridor, vertical hemispherotomy requires a smaller craniotomy and a shorter disconnection line and allows easier disconnection of the insula than the lateral approach. 1 In performing vertical hemispherotomy, 2 options are available: the interhemispheric approach 4 and the parasagittal approach. 3 With the parasagittal approach, the lateral ventricle is opened and entered via superior frontal gyrus. To ease the surgery and minimize the operation time, we apply "partial superior frontal gyrus removal." This procedure is useful to widen a shallower surgical field while skipping the need for interhemispheric dissection. Total callosotomy is then performed via the lateral ventricle. To disconnect limbic system, we used the technique following the falx and tentorial edge as a landmark. 5 Here, we present our surgical steps for parasagittal hemispherotomy in a 5-month-old girl diagnosed as hemimegalencephaly (Figures 1-8). [ABSTRACT FROM AUTHOR]

Intracranial mirror aneurysms are pathologic dilatations of cerebral arteries that occur at identical locations in both hemispheres. They do not always have identical sizes, shapes, or rupture states, despite identical patient-specific risk factors. As a subgroup of multiple aneurysms and thus small amounts of data, clinical research about their causes and development has not been conclusive. Using eight patients with 16 mirror aneurysms with different rupture states, we investigated differences between the hemispheres. We virtually performed stitching: detaching the aneurysm domes to place them on the opposite parent vessel to assess the influence of aneurysm morphology and upstream parent vessel shape on hemodynamics. In the unaltered datasets, we found that blood flow within aneurysms in the left hemisphere has higher wall shear stress and velocity. After stitching, we found that both upstream parent vessel as well as aneurysm morphology influence hemodynamics. Morphologic parameters like size, undulation index, and aneurysm angle had a higher influence. In a short rupture risk analysis we found a correlation between rupture and aspect ratio and non-sphericity index. Overall, we found possible indicators for mirror aneurysm analysis that illustrated the importance of morphological analysis, though more datasets are necessary for conclusive statements about special treatment for mirror aneurysms. [ABSTRACT FROM AUTHOR]

• Epilepsy surgery is a safe and effective form of treatment to improve neuropsychological outcomes in patients with drug resistant EE/DEE-SWAS. • Hemispherectomies and focal resections are similarly effective in achieving good seizure control and SWAS resolution. • Surgery can achieve immediate termination of seizures and SWAS pattern on EEG. • Patients undergoing focal resections showed the best neuropsychological outcome. • Clinicians should consider early surgical intervention for patients with drug resistant EE/DEE-SWAS. Epileptic Encephalopathy / Developmental Epileptic Encephalopathy with spike-and-wave activation during sleep (EE/DEE-SWAS) is a self-limiting childhood epilepsy syndrome but may cause permanent neurocognitive impairment. Surgical interventions have been controversial in the treatment of EE/DEE-SWAS. This systematic review aims to evaluate the efficacy of various surgical procedures on the outcomes of EE/DEE-SWAS. A systematic review was performed per the PRISMA guidelines. A total of 14 retrospective studies were identified, comprising 131 cases of EE/DEE-SWAS treated with epilepsy surgery. The review analyzed presurgical data, surgical interventions, as well as outcomes related to seizures, EEG, and neuropsychological assessments. Epilepsy surgery was successfully performed in 131 cases with minor complications. The average age was 2.6 years at seizure onset and 5.0 years at diagnosis of SWAS. Excellent seizure control (Engel I and II) was achieved in 80.6 %, 78.6 %, 77.4 % and 27.2 % of patients receiving hemispherectomies, focal resections, multiple subpial transections (MSTs), and corpus callosotomies (CCTs), respectively. EEG SWAS resolution was seen in 79.7 % of hemispherectomy cases, 78.6 % in focal resections, 63.9 % in MSTs, and 8.3 % in CCTs. Neurocognitive and behavioral improvement was noted in 58.0 %, 71.4 %, 58.3 % and 16.7 % for patients receiving hemispherectomies, focal resections, MSTs, and CCTs, respectively. A correlation between improved seizure control and SWAS resolution was observed with improved neuropsychological outcomes. Epilepsy surgery is a safe and effective treatment for carefully selected children with drug-resistant EE/DEE-SWAS. Patients who underwent epilepsy surgery had reduction of seizure burden, SWAS resolution and improvements in neurocognitive and behavioral function. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Hemispheric surgery effectively treats unihemispheric pediatric drug-resistant epilepsy (DRE) by resecting and/or disconnecting the epileptic hemisphere. Modifications to the original anatomic hemispherectomy have generated multiple functionally equivalent, disconnective techniques for performing hemispheric surgery, termed functional hemispherotomy. While a myriad of hemispherotomy variants exist, all of them can be categorized according to the anatomic plane they are performed in, which includes vertical approaches at or near the interhemispheric fissure and lateral approaches at or near the Sylvian fissure. This meta-analysis of individual patient data (IPD) aimed to compare seizure outcomes and complications between the hemispherotomy approaches to better characterize their relative efficacy and safety in the modern neurosurgical treatment of pediatric DRE, given emerging evidence that outcomes may differ between them. METHODS: CINAHL, Embase, PubMed, and Web of Science were searched from inception to September 9, 2020, for studies reporting IPD from pediatric patients with DRE who underwent hemispheric surgery. Outcomes of interest were seizure freedom at last follow-up, time-to-seizure recurrence, and complications including hydrocephalus, infection, and mortality. The χ2 test compared the frequency of seizure freedom and complications. Multivariable mixed-effects Cox regression controlling for predictors of seizure outcome was performed on propensity score-matched patients to compare time-to-seizure recurrence between approaches. Kaplan-Meier curves were made to visualize differences in time-to-seizure recurrence. RESULTS: Fifty-five studies reporting on 686 unique pediatric patients treated with hemispheric surgery were included for meta-analysis. Among the hemispherotomy subgroup, vertical approaches resulted in a greater proportion of seizure free patients (81.2% vs 70.7%, p = 0.014) than lateral approaches. While there were no differences in complications, lateral hemispherotomy had higher rates of revision hemispheric surgery due to incomplete disconnection and/or recurrent seizures than vertical hemispherotomy (16.3% vs 1.2%, p < 0.001). After propensity score matching, vertical hemispherotomy approaches independently conferred longer time-to-seizure recurrence than lateral hemispherotomy approaches (hazard ratio 0.44, 95% CI 0.19-0.98). DISCUSSION: Among functional hemispherotomy techniques, vertical hemispherotomy approaches confer more durable seizure freedom than lateral approaches without compromising safety. Future prospective studies are required to definitively determine whether vertical approaches are indeed superior and how it should influence clinical guidelines for performing hemispheric surgery.

Objective: The risk of hydrocephalus following hemispherectomy for drug resistant epilepsy (DRE) remains high. Patients with pre-existing hydrocephalus pose a postoperative challenge, as maintaining existing shunt patency is necessary but lacks a clearly defined strategy. This study examines the incidence and predictors of shunt failure in pediatric hemispherectomy patients with pre-existing ventricular shunts. Methods: We performed a retrospective chart review at our center to identify pediatric patients diagnosed with DRE who were treated with ventricular shunt prior to their first hemispherectomy surgery. Demographic and perioperative data were obtained including shunt history, hydrocephalus etiology, epilepsy duration, surgical technique, and postoperative outcomes. Univariate analysis was performed using Fisher's exact test and Pearson correlation, with Bonferroni correction to a = 0.00625 and a = 0.01, respectively. Results: Five of nineteen (26.3%) patients identified with ventriculoperitoneal shunting prior to hemispherectomy experienced postoperative shunt malfunction. All 5 of these patients underwent at least 1 shunt revision prior to hemispherectomy, with a significant association between pre- and post-hemispherectomy shunt revisions. There was no significant association between post-hemispherectomy shunt failure and valve type, intraoperative shunt alteration, postoperative external ventricular drain placement, hemispherectomy revision, lateralization of shunt relative to resection, postoperative complications, or postoperative aseptic meningitis. There was no significant correlation between number of post-hemispherectomy shunt revisions and age at shunt placement, age at hemispherectomy, epilepsy duration, or shunt duration prior to hemispherectomy. Conclusions: Earlier shunt revision surgery may portend a subsequent need for shunt revision following hemispherectomy. These findings may guide neurosurgeons in counseling patients with pre-existing ventricular shunts prior to hemispherectomy surgery. [ABSTRACT FROM AUTHOR]

Objectives: Hemimegalencephaly (HME) is a rare congenital brain malformation presenting predominantly with drug‐resistant epilepsy. Hemispheric disconnective surgery is the mainstay of treatment; however, little is known about how postoperative outcomes compare across techniques. Thus we present the largest single‐center cohort of patients with HME who underwent epilepsy surgery and characterize outcomes. Methods: This observational study included patients with HME at University of California Los Angeles (UCLA) from 1984 to 2021. Patients were stratified by surgical intervention: anatomic hemispherectomy (AH), functional hemispherectomy (FH), or less‐than‐hemispheric resection (LTH). Seizure freedom, functional outcomes, and operative complications were compared across surgical approaches. Regression analysis identified clinical and intraoperative variables that predict seizure outcomes. Results: Of 56 patients, 43 (77%) underwent FH, 8 (14%) underwent AH, 2 (4%) underwent LTH, 1 (2%) underwent unknown hemispherectomy type, and 2 (4%) were managed non‐operatively. At median last follow‐up of 55 months (interquartile range [IQR] 20–92 months), 24 patients (49%) were seizure‐free, 17 (30%) required cerebrospinal fluid (CSF) shunting for hydrocephalus, 9 of 43 (21%) had severe developmental delay, 8 of 38 (21%) were non‐verbal, and 15 of 38 (39%) were non‐ambulatory. There was one (2%) intraoperative mortality due to exsanguination earlier in this cohort. Of 12 patients (29%) requiring revision surgery, 6 (50%) were seizure‐free postoperatively. AH, compared to FH, was not associated with statistically significant improved seizure freedom (hazard ratio [HR] =.48, p =.328), although initial AH trended toward greater odds of seizure freedom (75% vs 46%, p =.272). Younger age at seizure onset (HR =.29, p =.029), lack of epilepsia partialis continua (EPC) (HR =.30, p =.022), and no contralateral seizures on electroencephalography (EEG) (HR =.33, p =.039) independently predicted longer duration of seizure freedom. Significance: This study helps inform physicians and parents of children who are undergoing surgery for HME by demonstrating that earlier age at seizure onset, absence of EPC, and no contralateral EEG seizures were associated with longer postoperative seizure freedom. At our center, initial AH for HME may provide greater odds of seizure freedom with complications and functional outcomes comparable to those of FH. [ABSTRACT FROM AUTHOR]

Objectives: Although hemispheric surgeries are among the most effective procedures for drug‐resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1‐, 2‐, and 5‐years post‐hemispherectomy. Methods: Retrospective data of all pediatric patients with DRE and seizure outcome data from the original Hemispherectomy Outcome Prediction Scale (HOPS) study were included. The primary outcome of interest was time‐to‐seizure recurrence. A multivariate Cox proportional‐hazards regression model was developed to predict the likelihood of post‐hemispheric surgery seizure freedom at three time points (1‐, 2‐ and 5‐ years) based on a combination of variables identified by clinical judgment and inferential statistics predictive of the primary outcome. The final model from this study was encoded in a publicly accessible online calculator on the International Network for Epilepsy Surgery and Treatment (iNEST) website (https://hops‐calculator.com/). Results: The selected variables for inclusion in the final model included the five original HOPS variables (age at seizure onset, etiologic substrate, seizure semiology, prior non‐hemispheric resective surgery, and contralateral fluorodeoxyglucose–positron emission tomography [FDG‐PET] hypometabolism) and three additional variables (age at surgery, history of infantile spasms, and magnetic resonance imaging [MRI] lesion). Predictors of shorter time‐to‐seizure recurrence included younger age at seizure onset, prior resective surgery, generalized seizure semiology, FDG‐PET hypometabolism contralateral to the side of surgery, contralateral MRI lesion, non‐lesional MRI, non‐stroke etiologies, and a history of infantile spasms. The area under the curve (AUC) of the final model was 73.0%. Significance: Online calculators are useful, cost‐free tools that can assist physicians in risk estimation and inform joint decision‐making processes with patients and families, potentially leading to greater satisfaction. Although the HOPS data was validated in the original analysis, the authors encourage external validation of this new calculator. [ABSTRACT FROM AUTHOR]

ObjectiveThis study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom.MethodsWe conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method.ResultsData for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique.SignificanceThis pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.

Abstract Introduction Hemimegalencephaly is a syndrome of dysplastic cortical formation, with hamartomatous overgrowth of a cerebral hemisphere, classically associated with intractable focal epilepsy, hemiparesis, and hemianopia. While often cryptogenic, associations with various proliferative syndromes have been implicated, such as in our patients. Patient Description We present a newborn with intractable focal epilepsy due to hemimegalencephaly caused by an inherited mutation in nitrogen permease regulator‐like 3 (NPRL3). He underwent anatomic hemispherectomy. His phenotype was more severe than that of other family member, which is consistent with recent studies suggesting that NPRL3 and other genes implicated in familial focal epilepsy with variable foci (FFEVF) produce a phenotypic range. Conclusions Hemimegalencephaly can produce intractable focal epilepsy and has been associated with various genetic causes, including NPRL3 mutations. We describe the fifth patient with hemimegalencephaly secondary to NPRL3 and the only one to undergo anatomic hemispherectomy. Given the small number of documented patients, more research is needed to elucidate the role of interventions such as sirolimus and palliative surgical procedures such as hemispherectomy.

Hydrocephalus is a known complication following surgical resection of a cerebral hemisphere for refractory epilepsy, yet the pathological mechanism remains poorly understood. We present a case of refractory aseptic inflammatory hydrocephalus following cerebral hemispherectomy surgery for refractory epilepsy treated with a combination of cerebral spinal fluid (CSF) diversion and immunosuppression via IL-1 receptor agonist, Anakinra. At 6 month follow up, the patient had returned to neurologic baseline, with improvement in school and physical therapy performance. Further investigation into the beneficial role of immunosuppressive therapy is needed to better understand the relationship between neuro-inflammation and improving outcomes following epilepsy surgery.

Rasmussen encephalitis is a rare and unexplained chronic brain hemispheric inflammatory disease. We report a case of epilepsy in which magnetoencephalography showed dipoles localized only in the operculum. Because the patient’s clinical presentation and examination findings did not meet the diagnostic criteria for Rasmussen encephalitis, he underwent cortical electroencephalogram (ECoG) record and limited resection surgery. However, the seizures were not relieved after surgery, and imaging findings showed significant features of hemisphere atrophy. This young male patient was eventually diagnosed with Rasmussen encephalitis and the seizures was completely vanished following hemispherectomy. His data can provide a reference for the early identification of this devastating disease. [ABSTRACT FROM AUTHOR]

Hemispherectomy is a surgical procedure in which an entire hemisphere of a patient’s brain is resected or functionally disconnected to manage seizures in individuals with drug-resistant epilepsy. Despite the extensive loss of both ventral and dorsal visual pathways in one hemisphere, pediatric patients who have undergone hemispherectomy show a remarkably high degree of perceptual function across many domains. In the current study, we sought to understand the extent to which functions of the ventral and dorsal visual pathways reorganize to the contralateral hemisphere following childhood hemispherectomy. To this end, we collected fMRI data from an equal number of left and right hemispherectomy patients who completed tasks that typically elicit lateralized responses from the ventral or the dorsal pathway, namely, word (left ventral), face (right ventral), tool (left dorsal), and global form (right dorsal) perception. Overall, there was greater evidence of functional reorganization in the ventral pathway than in the dorsal pathway. Importantly, because ventral and dorsal reorganization was tested within the very same patients, these results cannot be explained by idiosyncratic factors such as disease etiology, age at the time of surgery, or age at testing. These findings suggest that because the dorsal pathway may mature earlier, it may have a shorter developmental window of plasticity than the ventral pathway and, hence, be less malleable after perturbation.

To compare functional and seizure outcomes in children with vascular and dysplastic etiologies of cerebral palsy and medically intractable epilepsy following functional hemispherotomy or anatomic hemispherectomy. Consecutive patients satisfying inclusion criteria from 07/01/2015 to 12/01/2019 were reviewed for demographic data and seizure (Engel classification) and functional (Functional Independence Measure for Children) outcomes. After a mean follow-up of 2 years 8 months (1 year 2 months), 11 of 18 patients achieved post-operative seizure freedom without significant difference between vascular (5/7) and dysplastic (6/11) etiologies (P = 0.64). Functional assessments were completed for 15 of 18 of subjects, split comparably between groups. Mean change in the Functional Independence Measure for Children from pre-operative baseline to inpatient rehabilitation admission (vascular, −35.3 [13.2]; malformation of cortical development{MCD}, −34.5 [25.0]; P = 0.69), inpatient rehabilitation admission to discharge (vascular, 18.7 [9.0]; MCD, 20.8 [11.4]; P = 0.60), and pre-operative evaluation to clinic follow-up (vascular, −7.6 [9.7]; MCD, −3.6 [19.3]; P = 0.61) did not differ between groups. Quantitative functional and seizure outcomes following functional hemispherotomy or anatomic hemispherectomy did not differ significantly between vascular and dysplastic etiologies of cerebral palsy and medically intractable epilepsy in this study. Hemispheric surgery resulted in minor functional declines from baseline following comprehensive multidisciplinary therapy. [ABSTRACT FROM AUTHOR]

Objective: Completeness as a predictor of seizure freedom is broadly accepted in epilepsy surgery. We focused on the requirements for a complete hemispherotomy and hypothesized that the disconnection of the insula contributes to a favorable postoperative seizure outcome. We analyzed surgical and nonsurgical predictors influencing long‐term seizure outcome before and after a modification of our hemispherotomy technique. Methods: We retrospectively studied surgical procedures, electroclinical parameters, magnetic resonance imaging (MRI) results, and follow‐up data in all children who had undergone hemispherotomy between 2001 and 2018 at our institution. We used logistic regression models to analyze the influence of different factors on seizure outcome. Results: A total of 152 patients were eligible for seizure outcome analysis only. Of these, 140 cases had complete follow‐up data for ≥24 months and provide the basis for the following results. The median age at surgery was 4.3 years (range =.3–17.9 years). Complete disconnection (including the insular tissue) was achieved in 63.6% (89/140). At 2‐year follow‐up, seizure freedom (Engel class IA) was observed in 34.8% (8/23) with incomplete insular disconnection, whereas this was achieved in 88.8% (79/89) with complete surgical disconnection (p <.001, odds ratio [OR] = 10.41). In the latter group (n = 89), a potentially epileptogenic contralateral MRI lesion was the strongest predictor for postoperative seizure recurrence (OR = 22.20). Significance: Complete surgical disconnection is the most important predictor of seizure freedom following hemispherotomy and requires disconnection of the insular tissue at the basal ganglia level. Even if the hemispherotomy is performed surgically completely, a potentially epileptogenic contralateral lesion on preoperative MRI significantly reduces the chances of postoperative seizure freedom. [ABSTRACT FROM AUTHOR]

Abstract Background Hemispherectomy is an effective treatment option for patients with drug-resistant epilepsy caused by hemispheric lesions. However, patients often have deterioration of their motor functions postoperatively. Diffusion spectrum imaging (DSI) was reliable in presenting the natural shape of the white matter fibers. At the same time, the natural sprawl pyramid tract (PT) might be more intuitive for predicting postoperative motor functions. Therefore, we assessed the motor functions by the natural shape revealed by DSI tractography. Methods Ten children with drug-resistant epilepsy who were candidates for hemispherectomy performed DSI PTs tractography and transcranial magnetic stimulation (TMS) for motor mapping. The motor function was evaluated with muscle strength and hand grasping capability. Pyramidal tract (PT) structural integrity and TMS mapping results were compared between patients who remained stable and those with deteriorated motor functions. Receiver operating characteristic (ROC) curves with PTs asymmetric ratio were analyzed to evaluate DSI tractography diagnostic value. Results All patients underwent DSI acquisition, while four patients successfully performed TMS. One patient had no response to TMS until the maximal machine output was reached. Four patients failed to perform TMS due to lacking cooperation. One patient was contraindicated to TMS. DSI successfully reconstructed the sharp angle fan-shaped PTs within the hemisphere. The accurate fiber distribution with fiber termination and thickness within the lesioned hemisphere was replicated with DSI tractography. No significance was found in patients’ age, sex, seizure frequency, or medication between patients with stable or deteriorated postoperative motor functions. DSI effectively predicted postoperative motor function as stable with damaged PTs, mild deterioration with atrophied PTs, and intact PTs with contralateral innervation confirmed by intracranial stimulation. The area under the curve (AUC) of DSI tractography was 0.84. According to ROC, the cut-off value of PTs asymmetric ratio was 11.5% with 100% sensitivity and 75% specificity. The sensitivity and specificity of TMS were 2/3 and 1/2, respectively. Conclusions The anatomic integrity of PTs with DSI tractography could effectively predict postoperative motor function after hemispherectomy. This enables neurosurgeons to inform patients and relatives about postoperative motor functions with direct morphological evidence of PTs to help them with their surgical decisions.

Intensive therapies have become increasingly popular for children with hemiparesis in the last two decades and are specifically recommended because of high levels of scientific evidence associated with them, including multiple randomized controlled trials and systematic reviews. Common features of most intensive therapies that have documented efficacy include: high dosages of therapy hours; active engagement of the child; individualized goal-directed activities; and the systematic application of operant conditioning techniques to elicit and progress skills with an emphasis on success-oriented play. However, the scientific protocols have not resulted in guiding principles designed to aid clinicians with understanding the complexity of applying these principles to a heterogeneous clinical population, nor have we gathered sufficient clinical data using intensive therapies to justify their widespread clinical use beyond hemiparesis. We define a framework for describing moment-by-moment therapeutic interactions that we have used to train therapists across multiple clinical trials in implementing intensive therapy protocols. We also document outcomes from the use of this framework during intensive therapies provided clinically to children (7 months–20 years) from a wide array of diagnoses that present with motor impairments, including hemiparesis and quadriparesis. Results indicate that children from a wide array of diagnostic categories demonstrated functional improvements. [ABSTRACT FROM AUTHOR]

Introduction: In carefully selected patients with medically refractory epilepsy, disconnective hemispherotomy can result in significant seizure freedom; however, incomplete disconnection can result in ongoing seizures and poses a significant challenge. Completion hemispherotomy provides an opportunity to finish the disconnection. We describe the use of magnetic resonance-guided laser interstitial thermal ablation (MRgLITT) for completion hemispherotomy. Methods: Patients treated with completion hemispherotomy using MRgLITT at our institution were identified. Procedural and seizure outcomes were evaluated retrospectively. Results: Five patients (3 males) underwent six MRgLITT procedures (one child treated twice) for completion hemispherotomy at a median age of 6 years (range 1.8–12.9). Two children had hemimegalencephaly, two had Rasmussen encephalitis, and one had polymicrogyria. All five children had persistent seizures likely secondary to incomplete disconnection after their functional hemispherotomy. The mean time from open hemispherotomy to MRgLITT was 569.5 ± 272.4 days (median 424, range 342–1,095). One patient underwent stereoelectroencephalography before MRgLITT. The mean number of ablation targets was 2.3 ± 0.47 (median 2, range 2–3). The mean length of the procedure was 373 min ± 68.9 (median 374, range 246–475). Four of the five patients were afforded improvement in their neurocognitive functioning and speech performance after ablation, with mean daily seizure frequency at 1 year of 1.03 ± 1.98 (median 0, range 0–5). Two patients achieved Engel Class I outcomes at 1 year after ablation, one was Engel Class III, and two were Engel Class IV. The mean follow-up time was 646.8 ± 179.5 days (median 634, range 384–918). No MRgLITT-related complications occurred. Delayed retreatment (>1 year) occurred in three patients: one child underwent redo ablation and two underwent anatomic hemispherectomy. Conclusion: We have demonstrated the feasibility of a minimally invasive approach for completion hemispherotomy using MRgLITT. Delayed retreatment was needed in three patients; thus, further study of this technique with comparison to other surgical techniques is warranted. [ABSTRACT FROM AUTHOR]

Lennox-Gastaut syndrome (LGS) is a form of severe epileptic encephalopathy in children. LGS with encephalomalacia cysts is rare in children. We report a six-year-old mentally retarded boy who was referred for an intractable seizure. Seizures were tonic, atonic, and dialeptic in frequency. EEG showed generalized SSW discharges of 1.5-2 Hz, polyspikes, and burst suppression typical for LGS. Head MRI showed an encephalomalacia cyst in the right subcortical temporoparietal lobes with hemiatrophy in the right cerebral hemisphere. He was already treated with three antiepileptic drugs, but the seizures persisted. The patient was then performed right hemispherectomy and corpus callosotomy. It resulted in a good response. A combination of hemispherectomy and corpus callosotomy could be promising in this form of epilepsy disease. Seizure reduction was achieved and showed cognitive improvement and hemiparesis.

Background: Hemispherectomy is an effective treatment option for patients with drug-resistant epilepsy caused by hemispheric lesions. However, patients often have deterioration of their motor functions postoperatively. Diffusion spectrum imaging (DSI) was reliable in presenting the natural shape of the white matter fibers. At the same time, the natural sprawl pyramid tract (PT) might be more intuitive for predicting postoperative motor functions. Therefore, we assessed the motor functions by the natural shape revealed by DSI tractography. Methods: Ten children with drug-resistant epilepsy who were candidates for hemispherectomy performed DSI PTs tractography and transcranial magnetic stimulation (TMS) for motor mapping. The motor function was evaluated with muscle strength and hand grasping capability. Pyramidal tract (PT) structural integrity and TMS mapping results were compared between patients who remained stable and those with deteriorated motor functions. Receiver operating characteristic (ROC) curves with PTs asymmetric ratio were analyzed to evaluate DSI tractography diagnostic value. Results: All patients underwent DSI acquisition, while four patients successfully performed TMS. One patient had no response to TMS until the maximal machine output was reached. Four patients failed to perform TMS due to lacking cooperation. One patient was contraindicated to TMS. DSI successfully reconstructed the sharp angle fan-shaped PTs within the hemisphere. The accurate fiber distribution with fiber termination and thickness within the lesioned hemisphere was replicated with DSI tractography. No significance was found in patients' age, sex, seizure frequency, or medication between patients with stable or deteriorated postoperative motor functions. DSI effectively predicted postoperative motor function as stable with damaged PTs, mild deterioration with atrophied PTs, and intact PTs with contralateral innervation confirmed by intracranial stimulation. The area under the curve (AUC) of DSI tractography was 0.84. According to ROC, the cut-off value of PTs asymmetric ratio was 11.5% with 100% sensitivity and 75% specificity. The sensitivity and specificity of TMS were 2/3 and 1/2, respectively. Conclusions: The anatomic integrity of PTs with DSI tractography could effectively predict postoperative motor function after hemispherectomy. This enables neurosurgeons to inform patients and relatives about postoperative motor functions with direct morphological evidence of PTs to help them with their surgical decisions. [ABSTRACT FROM AUTHOR]

Introduction: Partial preservation of sensory and motor functions in the contralateral extremities after hemispherectomy is likely secondary to cortical reorganization of the remaining hemisphere and can be improved by rehabilitation. This study aims to investigate behavioral and structural cerebral cortical changes that may occur after a 2-week novel robotic rehabilitation program in children with prior anatomic hemispherectomy. Methods: Five patients with prior anatomic hemispherectomy (average age 10.8 years; all female) participated in a 2-week novel robotic rehabilitation program. Pre- and post-treatment (2 time points) high-resolution structural 3D FSPGR (fast spoiled gradient echo) magnetic resonance images were analyzed to measure cortical thickness and gray matter volume using a locally designed image processing pipeline. Results: Four of the five patients showed improvement in the Fugl-Meyer score (average increase 2.5 + 2.1 SD. Individual analyses identified small increases in gray matter volume near the hand knob area of the primary cortex in three of the five patients. Group analyses identified an increase in cortical thickness near the hand knob area of the primary motor cortex, in addition to other sensorimotor regions. Conclusion: This small pilot study demonstrates that potentially rehabilitation-associated cortical changes can be identified with MRI in hemispherectomy patients. [ABSTRACT FROM AUTHOR]

• Clinically meaningful and statistically significant improvements in motor function and behavior observed following a novel robotic rehabilitation two-week program in 18 patients with prior hemispherectomy. • Training camps enhance patient and parent confidence in task performance. • Rehabilitation related improvement in motor function and behavior years after hemispherectomy procedure suggest continued long-term neuroplasticity. Partial preservation of sensory and motor functions in the contralateral extremities after hemispherectomy is likely secondary to cortical reorganization of the remaining hemisphere and can be improved by rehabilitation. This study aims to investigate behavioral changes that may occur after a 2-week novel robotic rehabilitation program in 18 children with prior anatomic hemispherectomy. Other conventional rehabilitation methods were also reviewed and compared. This study examined the impact of a novel robotic rehabilitation 2-week program on 18 hemispherectomy patients (average age 14.3 ± 3.9 years; age at hemispherectomy 5.6 ± 4.5 years). Statistically significant improvements were seen in the six-minute walk test (29 m, p < 0.001), Canadian Occupational Performance Measure performance (1.64 points, p = 0.002) and satisfaction (2.49 points, p = 0.001), and individual perceived performance on survey (1.72 points, p = 0.042). Fifteen patients showed improvement in the upper extremity Fugl-Meyer scores with an average increase of 3 points (p = 0.006). This study demonstrates clinically meaningful and statistically significant improvements in motor function and behavior following a novel robotic rehabilitation two-week program. [ABSTRACT FROM AUTHOR]

The human auditory system consists of both peripheral and central components, both of which play a role but contribute distinctly to overall auditory functioning and can be differentially impacted by pathophysiologic states. Hemispheric surgery (HS), a procedure used for the treatment of drug-resistant epilepsy, involves complete disconnection of the auditory cortex in the operative hemisphere, leaving hearing acuity (peripheral function) intact but having heavy implications for auditory processing (central function). The literature describing pre- and post-operative auditory processing abilities of individuals who have undergone HS is sparse, but the research available provides evidence that several central auditory processes including auditory spatial analysis and temporal processing may be impacted. Deficits noted in standardized testing within the clinical or research environment have concrete functional impacts that may be currently under-appreciated and could lead to under-utilization of appropriate therapeutic strategies and accommodations. This review describes the profile of central auditory processing abilities in patients who have undergone HS by synthesizing available literature and incorporating research in other clinical populations to help fill critical gaps in our understanding of how cerebral disconnection impacts the central auditory system. • Hemispheric surgery significantly disrupts central auditory structures. • Knowledge gaps exist in documenting auditory processing after hemispheric surgery. • Auditory processing deficits have functional impact on social and academic success. [ABSTRACT FROM AUTHOR]

The Hemispherectomy Outcome Prediction Scale (HOPS) was developed to aid both clinicians and patients in determining the chance of success after hemispheric surgery for medically refractory epilepsy. The original study generating HOPS had a multi-institutional, large cohort format yielding near perfect patient stratification. Evidence suggests that methodologies utilized to create such predictive models, including cross-validation as well as stratification utilizing the same data employed for model generation, may be at risk of an undesirable modeling phenomenon known as overfitting. We posed the question of whether overfitting may be influencing HOPS results and aimed for preliminary evidence of external validation with parameters from patients at our institution not included in the original HOPS study. We found HOPS to stratify our limited post-operative cohort adequately. However, the likelihood of complete seizure freedom among the patients predicted by HOPS to be at greatest chance of success was ∼75 %, about 20 points lower than in the original HOPS cohort. This reduction in absolute chance of success predicted by HOPS may represent some degree of overfitting. It will be informative to aim for external validation of HOPS utilizing patient cohorts entirely separate from those used for model generation. External validation of HOPS and similar models could optimize realistic prediction of success after intervention. • Cross-validation is not immune to overfitting. • The original study defining HOPS may be influenced by the phenomenon of overfitting. • We applied HOPS to a small but similar cohort, finding qualitative validation but quantitative evidence of overfitting. • HOPS may benefit from a coordinated effort at external validation yielding more realistic reporting of success rates. [ABSTRACT FROM AUTHOR]

Introduction: Hemispherectomy/hemispherotomy has been employed in the management of catastrophic epilepsy. However, initial reports on the associated mortality and morbidity raised several concerns regarding the technique's safety. Their actual, current incidence needs to be systematically examined to redefine hemispherotomy's exact role. Research question: Our current study examined their incidence and evaluated the association of the various hemispherotomy surgical techniques with the reported complications. Material & methods: A PRISMA-compliant systematic review and meta-analysis was performed. We searched PubMed, Scopus, and Web of Science until December 2022. Fixed- and random-effects models were employed. Egger's regression test was used for estimating the publication bias, while subgroup analysis was utilized for defining the role of the different hemispherotomy techniques. Results: We retrieved a total of 37 studies. The overall procedure mortality was 5%, with a reported mortality of 7% for hemispherectomy and 3% for hemispherotomy. The reported mortality has decreased over the last 30 years from 32% to 2%. Among the observed post-operative complications aseptic meningitis and/or fever occurred in 33%. Hydrocephalus requiring a shunt insertion occurred in 16%. Hematoma evacuation was necessary in 8%, while subgaleal effusion in another 8%. Infections occurred in 11%. A novel post-operative cranial nerve deficit occurred in 11%, while blood transfusion was necessary in 28% of the cases. Discussion and conclusion: Our current analysis demonstrated that the evolution from hemispherectomy to hemispherotomy along with neuroanesthesia advances, had a tremendous impact on the associated mortality and morbidity. Hemispherotomy constitutes a safe surgical procedure in the management of catastrophic epilepsies.

Objective: Sampling the insulo‐opercular region with invasive recordings is crucial given the importance of this region in epileptic networks and a variety of electroclinical presentations. However, implantation of the insulo‐opercular region via stereoelectroencephalography (sEEG) is considered technically challenging given complex vascular and gray matter relationships in this region. We investigated the safety of insulo‐opercular sEEG exploration in children and young adults using standard sEEG approaches: (1) orthogonal insulo‐opercular (including the pseudo‐orthogonal insulo‐opercular approach) and (2) medial‐lateral insular oblique approach. Methods: We performed a retrospective cohort study of 30 consecutive patients who underwent 33 sEEG implantations. All patients had drug‐resistant focal epilepsy, were between the ages of 4 and 21, and were operated at one institution between January 2019 and March 2021. Medical records and neuroimaging were reviewed. Hemorrhage, infection, and other complications were considered as outcome variables. Results: A total of 519 electrodes were placed. Eighty‐one were placed orthogonally into the temporal operculum, 53 orthogonally into the frontal operculum, and 19 obliquely into the insula. sEEG electrodes localized seizure onset to the insulo‐opercular region in eight patients, leading to a resection three times, an ablation four times, and one peri‐insular hemispherectomy. Of the 519 electrodes placed, none of them exhibited hemorrhage or serious complications. Of the 153 electrodes placed into the insula, none had any permanent deficits or complications and one had minor bleeding due to the electrode breaking. Significance: These results demonstrate that the orthogonal (including pseudo‐orthogonal) and medial approaches to sampling the insula are safe. [ABSTRACT FROM AUTHOR]

The seizure outcomes after hemispheric epilepsy surgery have been excellent, with 54%–90% of patients achieving long-term freedom from seizures. Similarly, the neuropsychological outcomes have been favorable. The prognostic value of pre- and postoperative electroencephalography (EEG) has not been well-studied. In the present study, we characterized the value of the pre- and postoperative EEG findings for predicting the seizure and neuropsychological outcomes for pediatric patients undergoing hemispherectomy. A total of 22 children who had undergone functional hemispherectomy at our institution from 2010 to 2020 were included. The ictal and interictal findings were categorized as ipsilateral to the operated hemisphere, independently arising from the contralateral hemisphere, and/or generalized. The seizure outcomes were classified using the Engel scale. All neuropsychological evaluations were performed in accordance with our institution's protocol. The relationship between the EEG findings and outcomes was analyzed. Of the 22 patients, 19 (86%) were seizure free (Engel class IA) at the latest follow-up (mean, 4.2 years). On the preoperative EEGs, 9 had had seizures, all had had ipsilateral interictal discharges, and 9 had had contralateral interictal discharges. On the postoperative EEGs, obtained a median of 1 year after surgery, 3 had had seizures, 16 had had ipsilateral interictal discharges, and 5 had had contralateral interictal discharges. Of the 3 patients with seizures found on the postoperative EEG, all were clinically free of seizures. The patients who had not achieved Engel class IA were not significantly more likely to have abnormalities found on the EEG. The neuropsychological scores were stable from before to after surgery, with no evidence of EEG abnormalities having predictive value. The seizure and neuropsychology outcomes after hemispherectomy were excellent in our study, with 86% of our cohort achieving freedom from seizures. The presence, lateralization, and evolution of pre- and postoperative EEG abnormalities were not predictive of the outcomes. [ABSTRACT FROM AUTHOR]

The right and left cerebral hemispheres are important for face and word recognition, respectively--a specialization that emerges over human development. The question is whether this bilateral distribution is necessary or whether a single hemisphere, be it left or right, can support both face and word recognition. Here, face and word recognition accuracy in patients (median age 16.7 y) with a single hemisphere following childhood hemispherectomy was compared against matched typical controls. In experiment 1, participants viewed stimuli in central vision. Across both face and word tasks, accuracy of both left and right hemispherectomy patients, while significantly lower than controls' accuracy, averaged above 80% and did not differ from each other. To compare patients' single hemisphere more directly to one hemisphere of controls, in experiment 2, participants viewed stimuli in one visual field to constrain initial processing chiefly to a single (contralateral) hemisphere. Whereas controls had higher word accuracy when words were presented to the right than to the left visual field, there was no field/hemispheric difference for faces. In contrast, left and right hemispherectomy patients, again, showed comparable performance to one another on both face and word recognition, albeit significantly lower than controls. Altogether, the findings indicate that a single developing hemisphere, either left or right, may be sufficiently plastic for comparable representation of faces and words. However, perhaps due to increased competition or "neural crowding," constraining cortical representations to one hemisphere may collectively hamper face and word recognition, relative to that observed in typical development with two hemispheres. [ABSTRACT FROM AUTHOR]

Recent genetic studies have revealed that hemimegalencephaly (HME) is a multi-system disorder associated with germline or mosaic variants within the PI3K-mTOR-GATOR1 signaling pathways. Patients with HME typically develop drug-resistant epilepsy necessitating extensive evaluation, hemispherectomy, and long-term management. We describe the role of a multidisciplinary team (MDT) for the diagnosis and management of recent patients with HME at UCLA who underwent hemispherectomy. Genetic evaluation identified nine patients with the following variants: NPRL3 x2 germline, PIK3CA mosaicism x4, MTOR mosaicism x1, AKT3 mosaicism x1, unknown x1. Each patient's MDT comprised 4-9 specialties. One child with a MTOR variant had persistent epilepsy after hemispherectomy, but addition of everolimus resulted in an 80% decrease in seizure frequency. Another child with hemihypertrophy and PIK3CA mosaic variant was offered targeted PIK3CA inhibitor treatment, alpelisib, for overgrowth. A third child with germline NPRL3 variant inherited from their unaffected mother resulted in a sibling being diagnosed with the variant who later developed seizures secondary to focal cortical dysplasia. The implementation of a MDT offers essential guidance for families affected by HME, encompassing prognostication, surveillance, and therapeutic strategies. Identifying the etiology of HME can facilitate the development of targeted treatments and enable timely genetic counseling. PLAIN LANGUAGE SUMMARY: Hemimegalencephaly (HME) is a complex brain disorder caused by genetic changes. It often leads to severe epilepsy that doesn't respond to standard treatments and frequently requires surgery. In this case series, nine patients with HME were identified and found to have genetic mutations in key growth-regulating genes. A multidisciplinary team model was developed to facilitate patients' care. For example, one patient's seizures improved with surgery, another with a new targeted medication, and another received treatment for symptoms of overgrowth. This team approach provides comprehensive care for patients and can lead to efficient care coordination and implementation of novel therapies., (© 2024 The Author(s). Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Purpose: Since it was first described in the 1970s, functional hemispherotomy has been an essential tool in treating disabling, medically refractory epilepsy resulting from diffuse unilateral hemispheric disease. We report our experience with 23 patients who underwent hemispherotomy, both using the functional hemispherotomy (FH) as well as a modified peri-insular hemispherotomy (PIH) technique. We present the surgical technique for the latter, review outcomes following disconnection surgery and discuss the differences between the techniques when it comes to complications and postoperative results., Methods: A retrospective study of 23 patients with refractory seizures who underwent cerebral hemispherectomy. A thorough analysis of the clinical, imaging, surgical features and postoperative results was performed. We also present the surgical technique for a modified PIH technique., Results: Between 2000 and 2020, 23 pediatric patients with refractory seizures underwent hemispherotomy (12 FHs, 11 modified PIHs). 91.3% of patients were seizure free at 6 months, 87% at 1 year, and 78.3% at last follow-up. None of the 23 patients presented Engel IV outcome. FH was found to have statistically longer surgical duration (5 ± 1.5 vs. 3.83 ± 0.5 h; p = <0.001). Neurocognition was improved in two thirds of the patients (66.9%). Our study also shows improvement of motor activity in the majority of the patients, regardless of the pathology and surgical technique. In the present report we modified the Cook et al. technique by implementing an amygdalohippocampectomy with resection of the tail of the hippocampus posteriorly and medially, to achieve temporo-occipital disconnection, instead of a complete temporal lobectomy., Conclusion: When patients are wisely selected, the hemispherectomy procedure should be considered as a most attractive and curative treatment for children with refractory seizures, not only giving the patient a high chance of seizure freedom but also providing an improvement in motor and cognitive skills. In our particular case and based on the present study, the modified PIH proves to be a highly effective technique. It not only has a shorter surgical time but also a very low complication rate., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Objective: The goal of this study was to assess the complications associated with vertical parasagittal hemispherotomy (VPH), the impact of incomplete disconnection on long-term seizure freedom, and how VPH impacts cognitive development., Methods: A retrospective evaluation was performed in all patients who had undergone VPH during 1991-2022 at the authors' institution. Two-year follow-up data were available for 45 patients, and there were 6-month data for 3 more. All available postoperative MRI studies (31/48, 64.6%) were reviewed. Before 2010, postoperative MRI was only performed if seizures recurred., Results: Primary VPH led to Engel class I in 73% of patients. Acquired etiologies had a higher rate of Engel I compared to developmental and progressive etiologies (96% vs 46% and 44%, p < 0.001). Nearly half of patients (45%) showed improved cognitive trajectories as opposed to their preoperative ones, whereas in 45% trajectories remained unchanged. Additionally, 5 patients (10%) exhibited new major deficits or accelerated cognitive deterioration after VPH. Surgical complications occurred in 14 patients (29%) after the first VPH; 4 cases were classified as transient, resolving during follow-up without surgical intervention. Nontransient complications included 8 cases of hydrocephalus requiring surgical treatment, 1 shunted subdural hygroma, and 1 case of CSF leakage from the wound. Diabetes insipidus occurred in 6 patients, with all resolving spontaneously. Residual connections were present in 16 patients, primarily in the temporomesial region. Seven patients remained seizure free despite visible residual connections., Conclusions: VPH is a highly effective treatment for drug-resistant hemispheric epilepsy, resulting in durable seizure freedom and often favorable cognitive outcomes. Diabetes insipidus in addition to hydrocephalus is a common complication after VPH. Incomplete disconnection does not necessarily preclude seizure freedom.

Background: Children undergoing hemispheric surgery for intractable seizures are susceptible to visual complications including strabismus. This systematic review aims to investigate the rates and characteristics of strabismus development after hemispheric surgery and evaluate clinical implications for ophthalmologic care., Methods: A systematic search of MEDLINE, EMBASE, Cochrane, PsychINFO, and Web of Science databases was performed from database inception to May 2022. Included articles referred to strabismus outcomes in pediatric populations after hemispherectomy or hemispherotomy. Reviews and non-English-language publications were excluded. Risk of bias was assessed using Joanna Briggs Institute critical appraisal tools. Demographic data and characteristics of strabismus were extracted and tabulated., Results: Of 41 articles identified, 10 studies consisting of 384 pediatric participants (48% females) and age at surgery between 6 months and 16 years were included. Preoperative strabismus rates ranged between 3% and 56%, whereas postoperative rates ranged between 38% and 100%. With respect to the site of hemispheric surgery, contralateral exodeviation was the most common (16%-67%; n = 7) and then ipsilateral exodeviation (16%-56%; n = 2), whereas ipsilateral esodeviation was infrequent (4%-9%; n = 3)., Conclusions: Contralateral exotropia and ipsilateral esotropia may occur after hemispheric surgery and may have the potential to be field expanding. Concerns regarding negative social reactions should be balanced with the risk of visual field reduction and (or) diplopia by strabismus surgery. Higher-quality articles with large, homogeneous, and well-described populations (i.e., complete pre- and postoperative ophthalmologic assessments) are required to establish the risks and rates of strabismus development after hemispheric surgery., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Background: Rasmussen’s encephalitis (RE) is a rare chronic neurological disorder that presents with progressive neurological and cognitive deterioration, and intractable seizures that lacks definitive management. We aimed to identify case reports of RE in the literature to assess the treatment based on the symptoms and outcomes after the treatment. Methods: We searched the databases PubMed, Google Scholar, and the Cochrane Library from 1990 to 22nd February 2022 in order to review the case reports that included the treatment options and clinical outcomes of RE. A pre-established protocol was registered on the International Prospective Register of Systematic Reviews (PROSPERO) database (ID: CRD42021261999). Results: The mean age of onset of seizure was 31.44 and 6.87 years in adult onset RE and children (below 15 years of age) with RE respectively. A total of four (44.44%) out of nine adult patients and 81 (66.39%) out of 122 children underwent surgery. Tacrolimus was used in 17 (13.93%) and steroid therapy was used in 11 (9.01%) of the children. Conclusions: Large scale-controlled trials are needed to study the beneficial effect of tacrolimus, IV Ig, and plasma therapy. Hemispherectomy should be opted as a treatment modality by assessing the impairment of patients’ motor or language functions following the treatment procedure.

Introduction: Pediatric hemispherectomy is a technically demanding procedure with significant risk. There are relatively few opportunities for surgeon trainees to gain confidence in this nuanced surgery solely through traditional apprenticeship prior to independent practice. Surgeon confidence has been linked to better intraoperative performance and surgical simulation has, according to literature, resulted in improved surgeon confidence. This manuscript measures the effect of epilepsy simulation on the procedural confidence of neurosurgical trainees as a marker of future improved intraoperative performance. Methods: Eleven neurosurgery residents and fellows were allowed to practice on a novel hemispherectomy simulator. Pre- and post-simulation procedural confidence was measured using a 10-item questionnaire, with total scores ranging from 10 (least confident) to 50 (most confident). Matched pair t-testing was performed to determine participant mean difference between the pre- and post-procedural data sets. Results: The assessment tool bore a high reliability coefficient (Cronbach's α = 0.93). The procedural confidence of all the study participants increased following simulation (p value < 0.001). The overall mean increase in confidence was 7.2 ± 4.0 (mean ± standard deviation), 7.5 ± 4.7 among fellows and 6.8 ± 3.6 among residents. Procedural confidence values were higher among the fellows (26.9 ± 8.1) compared to the residents (19.0 ± 7.4). This difference in means was statistically significant (p value 0.03). A positive association was calculated between the perceived confidence and the postgraduate year of training (p value 0.005, r = 0.57). Conclusion: The hemispherectomy simulator moderately improves perceived confidence among neurosurgical trainees and may augment pre-operative surgical practice opportunities. [ABSTRACT FROM AUTHOR]

Background: Posterior quadrant disconnection (PQD) is intended to interrupt the propagation of intractable unilateral temporo-parieto-occipital epilepsy. Method: An enhanced operative video presents the illustrative case of a total PQD indicated for a 15-year-old boy with Sturge-Weber syndrome suffering from seizure recurrence after a partial PQD. We describe the surgical procedure with emphasis on relevant anatomy and multimodal intraoperative guidance in three steps: (i) parieto-occipital disconnection, (ii) posterior callosotomy, and (iii) temporal disconnection/resection. Pearls and pitfalls of surgical management are discussed. Conclusion: PQD is a less invasive surgical option to typical hemispherotomy and hemispherectomy for selected indications of posterior multilobar epilepsy. [ABSTRACT FROM AUTHOR]