Comparative Analysis of Hemispherotomy in Adults versus Children- A Prospective Observational Series.

BACKGROUND: Hemispherotomy (HS) is an effective treatment for unilateral hemispheric onset epilepsy. There are few publications for HS in adults, and there is no series comparing adults and pediatric patients of HS. OBJECTIVE: To compare the hemispherotomies done in adult patients with pediatric ones in terms of efficacy and safety. METHODS: Data was prospectively collected for HS patients (up to 18 years and more) from Aug 2014 to Aug 2018. Comparison between the groups was made for seizure onset, duration of epilepsy, frequency of seizures, number of drugs, intraoperative blood loss, postoperative seizure control, postoperative stay, postoperative motor functions, and preoperative and postoperative intelligence quotient. Follow-up was one year. RESULTS: A total of 61 pediatric and 11 adults underwent HS. The seizure onset was earlier in children, and the duration of epilepsy was longer in adults. The frequency of seizures per day was more in children being 14.62 ± 26.34 in children, and 7.71 ± 5.21 per day in adults (P - 0.49). The mean number of drugs was similar in the preoperative and postoperative periods in both. Class I seizure outcome was similar in both the groups being 85.24% in children and 90.9% in adults (P - 0.56). Blood loss, postoperative stay, was similar in both the groups. No patient had a new permanent motor deficit. Power worsened transiently in 1 pediatric patient and in 4 adult patients. The visual word reading and object naming improved in both the groups (no intergroup difference), and IQ remained the same in both groups. One adult patient had meningitis, and another had hydrocephalus requiring shunt placement. CONCLUSION: Hemispherotomy is a safe and effective procedure in adults as in children in appropriately selected patients.

Technique of Distraction, Compression, Extension, Reduction to Reduce and Realign Old Displaced Odontoid Fracture From Posterior Approach: A Novel Technique.

OBJECTIVE: Chronic 'displaced' displaced type II fractures, though uncommon, are difficult to manage. They usually require a transoral procedure followed by a posterior instrumented fusion. We describe here, a new method to reduce the fractured displaced odontoid using a posterior cervical approach only. METHODS: Prospective and observational, n = 14 had a 'displaced and irreducible' old fracture dens causing cord compression (type I, 1; type II, 13). They underwent a novel technique to reduce the fracture. The C1 arch was first drilled and removed. The C1 lateral masses on both sides were then drilled completely and a spacer was placed between the occiput and C2 facet. Following this, an intraoperative reducing maneuver was performed, utilizing the spacer as a fulcrum, and then achieving complete reduction and realignment. RESULTS: All patients improved clinically (mean Nurick preoperative score: 4.07 ± 0.8; the postoperative score was 1.3 ± 0.4). The mean correction in effective canal diameter was 74.3% ± 9.5% and the mean correction in actual canal diameter was 77% ± 8.7%. Solid bone fusion was demonstrated in 12 patients with at least 1-year follow-up (follow-up range, 12-35 months; mean, 21.8 ± 9.8 months). CONCLUSION: The new described modification of distraction, compression extension, and reduction seems to be effective for 'displaced' chronic fracture dens with cord compression. It avoids additional transoral surgery in these patients.

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children.

OBJECTIVE: Dystonias are relatively rare disorders characterized by sustained or intermittent muscle contractions causing abnormal movements or postures. Generalized dystonia is a therapeutic challenge because medications are unable to control dystonia adequately in most patients. These patients may be candidates for surgical therapy. The commonly used surgical procedures in these patients are pallidotomy and deep brain stimulation. Limited studies are available on the role of pallidotomy in children with acquired/heredodegenerative generalized dystonia. The objective of this study was to describe the authors' experience with bilateral pallidotomy in this group of patients. METHODS: The authors retrospectively reviewed all pediatric patients (less than 18 years of age) with acquired/heredodegenerative generalized dystonia who underwent bilateral simultaneous pallidotomy at their center between January 2014 and January 2021. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores before and after surgery were recorded. Complications arising after the procedure were recorded as well. RESULTS: Ten patients (8 male and 2 female) with a mean (range) age of 11.1 (5-17) years were included in this study. The mean duration between disease onset and surgical intervention was 3.9 years. Two patients presented in status dystonicus. The mean ± SD (range) preoperative BFMDRS score of the patients without status dystonicus (n = 8) was 80 ± 18.9 (59.5-108). The mean ± SD BFMDRS score at the time of discharge from the hospital after surgery was 58.8 ± 37.9. Three patients had more than 20% change in BFMDRS score at the time of discharge from the hospital. The mean improvement was 25.5% at the end of 1 year. Of 5 surviving patients in the non-status dystonicus group, 3 patients had more than 40% change in BFMDRS score while the other 2 patients developed recurrence at the last follow-up (4.5 years). Status dystonicus abated after bilateral pallidotomy in both patients. Permanent bulbar complications were seen in 2 patients. CONCLUSIONS: Bilateral pallidotomy may result in clinically significant improvement in children with acquired/heredodegenerative generalized dystonia, although the benefits should be closely weighed against the risk of irreversible bulbar dysfunction. It is a viable option for children in resource-limited settings.

Long-Term Functional Outcome Following Left Hemispherotomy in Adults and Pediatric Participants with Fmri Analysis.

Background and Objective: Hemispherotomy surgery in adults is shrouded in doubts regarding the functional outcome. The age at surgery alone should not be the deciding factor for surgery. Language paradigms were used in functional magnetic resonance imaging (fMRI) to confirm the role played by the age at the onset of seizures to predict the postoperative functional outcome. The objective of the study was to formulate an optimal strategy for patient selection for the left-sided hemispherotomy in adults, based on functional outcome analysis. Materials and Methods: A retrospective analysis of 20 participants (age at surgery 1-26 years) who underwent left hemispherotomy (over a 5-year period) was conducted. The language and motor functional assessments of 18 participants (13 pediatric and five adult participants; attrition of participants- two) were recorded at presentation and during follow-up visits. After approval was obtained from the Institutional Ethics Committee, 13 cooperative participants (eight pediatric and five adult participants) underwent language fMRI. Motor fMRI with both active and passive paradigms was done in 16 participants. Results: All 18 participants with a mean follow-up of 24 months had class I seizure-free outcome. Of these 18, five were adults (mean age = 21 years, range: 18-22 years) and 13 were in the pediatric age group (mean age = 8 years, range: 2-15 years). Postoperatively, four adults retained both verbal fluency and language comprehension at a mean follow-up period of 38 months (range: 24-48 months). Their pre- and post-op language fMRI showed word generation and regional activations for semantic comprehension in the right hemisphere. The motor area activations were seen in the right hemisphere in two and in the left hemisphere in two participants. Among the pediatric participants, four (group I [n = 4/13]) who had good language outcome showed activations in the right hemisphere. In two participants (group II [n = 2/13]) who deteriorated postoperatively, the activations were in the left hemisphere. Five participants (group III [n = 5/13]) who retained the telegraphic language postoperatively had bilateral activations of semantic comprehension areas in fMRI. All 13 pediatric participants had motor area activations seen in the left hemisphere, similar to controls. Conclusion: Left hemispherotomy can be advised to adults with comparably good postoperative language and motor outcome as in the pediatric age group, provided the weakness is acquired perinatally or below the age of 7 years. The fMRI is a valuable tool to aid in patient selection.

Feasibility of Tailored Unilateral Disconnection vs Callosotomy for Refractory Epilepsy in Patients with Bilateral Parieto-Occipital Gliosis Following Perinatal Insult.

Background: Patients with perinatal hypoxia (PH) and drug-refractory epilepsy (DRE) often have bilateral parieto-occipital gliosis. Surgical management of such patients is a dilemma. Objective: To identify preoperative determinants for unilateral disconnection vs callosotomy, and analyze the surgical outcome in such patients. Methods and Material: This was a retrospective analysis of patients with DRE and history of PH, with MRI abnormalities restricted to bilateral posterior quadrants. Preoperative semiology, epilepsy duration and seizure frequency were recorded. Based on the concordance between the results of non-invasive tests, patients underwent either posterior quadrant disconnection (PQD) or corpus callosotomy (CC). Preoperative variables were analyzed and corelated to the postoperative seizure freedom. Results: Fourteen patients were identified, 6 underwent PQD and 8 underwent CC. At follow up of 39.17 ± 23.75 months, 66.66% of patients (4/6) in the PQD subgroup had an ILAE Class I outcome. While none in the CC group attained seizure freedom, 87.5% (7/8) had more than 50% reduction in seizure frequency (follow up: 42 ± 27.31 months). Patients with a poor outcome had significantly greater seizure frequency (P = 0.05) and history of drop attacks (P = 0.04) in both the groups. Magnetoencephalography (MEG) accurately localized the epileptogenic zone in all of the patients with good outcome (P = 0.015). Concordance with single photon emission tomography (SPECT) was also a predictor of favorable outcome (P = 0.041). Conclusions: A history of drop attacks with high seizure frequency is associated with poor postoperative seizure outcome. Unilateral PQD is feasible and leads to superior seizure-free outcomes, even in cases with widespread and bilateral imaging and electrical abnormalities, provided the other preoperative investigations are concordant in localizing the epileptogenic zone.

Mini Temporal Craniotomy Using Anatomical Surface Landmarks for Temporal Lobe Epilepsy: Technical Note and Clinical Outcomes.

Background: Patients with temporal lobe epilepsy are subjected to standard temporal lobectomy wherever indicated. This is performed using a reverse question mark flap and a standard frontotemporal craniotomy. We describe the technique of minitemporal craniotomy (3 × 3cms) for temporal lobe epilepsy (TLE) and analyze the clinical outcomes of patients operated using this approach. Objectives: To describe the technique of minitemporal craniotomy for TLE without navigation guidance and to analyze the clinical outcomes of patients operated using this approach. Materials and Method: This was a retrospective analysis of all consecutive TLE cases operated at our institute from 2014 to 2019, via minitemporal craniotomy, using surface landmarks only without navigation guidance. The surgical technique, indications for surgery, and their clinical outcomes were analyzed. Results: A total number of 48 patients underwent surgery for TLE. There were no complications except three patients who had transient hemiparesis. The average duration of hospital stay was 4 days following surgery. Out of 28 patients with mesial temporal sclerosis, 22 (82%) had international league against epilepsy, Class I seizure outcome, 4 (12.5%) had Class II outcome and 2 (5.5%) had Class III outcome. 9 patients with dysembryoplastic neurectodermal tumor (DNET), 4 gangliogliomas, 2 neurocystecercosis (NCC), all had Class I outcome. Out of the five patients with MTS and associated anterior temporal focal cortical dysplasia (FCD), four (80%) had a Class I outcome, whereas one (20%) had Class II outcome. Conclusion: Utilizing surface anatomical landmarks, minitemporal craniotomy can be performed in even peripheral centers without neuronavigation, with good cosmesis, seizure outcomes.

Surgical Track and Scalp Implantation Following Intraventricular Meningiomas Excision: A Report with Review of Literature.

Meningiomas are benign intracranial neoplasms arising from arachnoid cap cells. High grade meningiomas are uncommon and metastasis from these is an extremely rare event. Commonest sites of metastasis from high grade meningiomas include lung, liver, lymph nodes and bone. It is unusual for meningiomas to recur in the surgical track following excision. More so, it is even the rarest phenomenon for a meningioma to implant in subgaleal location. Various mechanisms have been proposed for the scalp implantation vis-à-vis CSF dissemination, direct surgical implantation etc., It may apply to all histological grades of meningiomas. Even the benign tumors have been shown to seed at postoperative scar. This seems to have provoked our interest to review the literature regarding this scalp implantation. We have reviewed all the cases where surgical excision of intracranial meningiomas has led to seeding of surgical track as well as scalp. We have discussed the various genetic aberrations that can guide us regarding the progression of the tumor and prognosis. We also report a case of surgical track and scalp implantation of an atypical intraventricular meningioma following excision.

Endoscopic Hemispherotomy for Nonatrophic Rasmussen's Encephalopathy.

BACKGROUND: Hemispheric disconnection represents a challenging and major epilepsy surgical procedure. This procedure in experienced hands offers excellent results in terms of seizure outcomes, especially for hemispheric pathologies such as Rasmussen's encephalitis, hemispheric dysplasias, hemimegalencephaly. The technique of hemispherotomy has witnessed various modifications over the years, beginning from anatomical hemispherectomy to the current era of minimally invasive functional hemispheric disconnections. OBJECTIVE: This study aimed to describe the technique of performing endoscopic vertical hemispherotomy using interhemispheric corridor developed by the senior author. MATERIALS AND METHODS: A 12-year-old girl with seizure onset at the age of 10 years presented with an aura of fear and nausea followed by tonic deviation of eyes to the right and blinking with speech arrest. There were tonic-clonic movements of the right-sided limbs along with ictal spitting and occasional deviation of the angle of mouth to the right. The patient had loss of awareness for the event along with postictal confusion lasting few minutes. RESULTS: Video electroencephalography (VEEG) revealed left parietocentral and left temporal localization. Serial magnetic resonance imaging (MRI) brain over 3 years revealed progressive left hemispheric changes suggestive of Rasmussen's encephalitis. The patient underwent left-sided endoscopic hemispherotomy. At 2 years follow-up, the patient is seizure-free (ILAE [International League Against Epilepsy] Class 1). CONCLUSION: Endoscopic hemispherotomy using the interhemispheric approach is an elegant, minimally invasive, reproducible, safe, and efficacious technique.

Robotic-Guided Stereoelectroencephalography for Refractory Epilepsy: Technique and Nuances.

BACKGROUND: Stereoelectroencephalography (SEEG) has become an integral part of epilepsy surgery, often used in the localization of the epileptogenic zone. It is an essential modality not only in the evaluation of nonlesional but also lesional drug refractory epilepsy, especially in the presence of anatomo-electro-clinical discordance. OBJECTIVE: To describe our technique and the operative nuances involved in the performance of robotic SEEG placement. METHODS: A 28-year lady with seizure onset at the age of 15 years presented with two types of seizures: one was associated with an aura of chest discomfort, palpitations along with oral and bilateral automatisms. There was associated speech and behavioral arrest along with ictal urinary incontinence. The other type has head turning to the right with secondary generalization lasting up to 1 min. RESULTS: Multimodality investigations showed bilateral temporal origin of seizures. SEEG evaluation revealed left amygdala and anterior temporal neocortical (ATL) origin of seizures. The patient underwent left ATL and amygdalectomy. Histopathology revealed focal cortical dysplasia (FCD type Ib). The patient became seizure free (ILAE Class 1) at 1-year follow up. CONCLUSION: Robotic-guided SEEG is a safe and accurate method of evaluating complex MRI negative epilepsy.

A Scientometric Analysis of the 100 Most Cited Articles on Pallidotomy.

BACKGROUND: Pallidotomy is the oldest stereotactically performed neurosurgical procedure for movement disorders. Consequently, there is a wealth of literature available on the topic. OBJECTIVES: The aim of this analysis was to identify the top-cited articles on pallidotomy in order to discern the origins, spread, the current trends, and the future directions of this surgical procedure. METHODS: We performed a search of the Web of Science database on 19 October 2020 using the keyword "pallidotomy." The top-100 cited articles found were arranged in descending order on the basis of citation count (CC) and citation per year (CY). Relevant conclusions were derived. RESULTS: The 100 top-cited articles were published between 1961 and 2017, in 24 journals. The average CC and CY were 118.1 (range - 856-46) and 5.326 (range - 29.52-2.09), respectively. The 3 most prolific authors were Lang AE (Neurologist - Toronto), Lozano AM (Neurosurgeon - Toronto), and Vitek JL (Neurologist - Atlanta). The Journal of Neurosurgery published the highest number of top-cited articles [Neurology. 1960;10:61-9]. The maximum articles were from the USA. University of Toronto and Emory University were the most productive institutions. CONCLUSIONS: Pallidotomy has gone through several ebbs and flows. Unilateral pallidotomy is currently recommended for the treatment of motor symptoms of Parkinson's disease and dystonia. The need for further research and improved technology to make the technique safer and prove its efficacy is highlighted, especially keeping in mind a large number of populations to which the prohibitively expensive deep brain stimulation is unavailable.

Outcome and Complications of MR Guided Focused Ultrasound for Essential Tremor: A Systematic Review and Meta-Analysis.

Background: Magnetic resonance guided focused ultrasound (MRgFUS) is a relatively novel technique to treat essential tremor (ET). The objective of this review was to analyze the efficacy and the safety profile of MRgFUS for ET. Methods: A systematic literature review was done. The post procedure changes in the Clinical Rating Scale for Tremor (CRST) score, hand score, disability and quality of life scores were analyzed. Results: We found 29 studies evaluating 617 patients. DTI based targeting was utilized in six cohorts. A significant difference was observed in the pooled standard mean difference between the pre and postoperative total CRST score (p-value < 0.001 and 0.0002), hand score (p-value 0.03 and 0.02); and the disability at 12 months (p-value 0.01). Head pain and dizziness were the most in procedure complications. The immediate pooled proportion of ataxia was 50%, while it was 20% for sensory complications, which, respectively, declined to 31 and 13% on long term follow up. A significant reduction (p = 0.03) in immediate ataxia related complications was seen with DTI targeting. Conclusion: MRgFUS for ET seems to be an effective procedure for relieving unilateral tremor. Use of DTI based targeting revealed a significant reduction in post procedure ataxia related complications as compared to traditional targeting techniques. Analysis of other complications further revealed a decreasing trend on follow up.

Epilepsy surgery in COVID times-a unique conundrum.

The COVID-19 pandemic has forced hospitals to prioritize admissions. Epilepsy surgeries have been postponed at most centers. As the pandemic continues with no definite end in sight in the near future, the question arises until when such patients should be denied appropriate treatment. A 12-year-old child with left-sided Rasmussen's encephalitis with drug refractory epilepsy (DRE) presented at the height of the pandemic, with worsening of seizure frequency from 4-5/day to 20/day, with new-onset epilepsia partialis continua. She demonstrated features of progressive cognitive decline. The pros and cons of operating during the pandemic were discussed with the parents by a multidisciplinary team. She underwent endoscopic left hemispherotomy. Postoperatively she became seizure free but developed hospital-acquired mild COVID infection for which she was treated accordingly. Chosen cases of severe DRE, as the one illustrated above, who are deemed to benefit from surgery by a multidisciplinary team of physicians, should be re-categorized into the most severe class of patients and scheduled for surgery as soon as possible. The risk benefit ratio of the seizures being mitigated by surgery on one hand and possibility of acquiring COVID infection during hospital stay has to be balanced and a decision made accordingly.

Robotic thermocoagulative hemispherotomy: concept, feasibility, outcomes, and safety of a new "bloodless" technique.

OBJECTIVE: The authors present a new "bloodless" technique for minimally invasive robotic thermocoagulative hemispherotomy (ROTCH). Such a method is being described in the literature for the first time. METHODS: A robotic system was used to plan five sets of different trajectories: anterior disconnection, middle disconnection, posterior disconnection, corpus callosotomy, and temporal stem and amygdalar disconnection. A special technique, called the "X" technique, allowed planar disconnection. Registration was performed with surface landmarks (n = 5) and bone fiducials (n = 1). Coregistration with O-arm images was performed one or two times to confirm the trajectories (once for middle disconnection, and once for disconnection of the temporal stem and amygdala or body of the corpus callosum). Impedance measured before ablation allowed for minor adjustments. Radiofrequency ablation was performed at 75°C-80°C for 60 seconds. Surgical procedures were performed with multiple twist drills. After removal of the electrode, glue was used to prevent CSF leak, and a single stitch was applied. Follow-up CT and MRI were immediately performed. RESULTS: The pathologies included Rasmussen's encephalitis (n = 2), hemispheric cortical dysplasia (n = 2), posttraumatic encephalomalacia (n = 1), and perinatal insult (n = 1). The mean ± SD (range) age was 6.7 ± 3.6 years (5 months to 10.2 years), and the right side was affected in 4 patients. The mean ± SD seizure frequency was 7.4 ± 5.6 seizures per day (1 patient had epilepsia partialis continua). The mean ± SD number of trajectories was 15.3 ± 2.5, and the mean ± SD number of lesions was 108 ± 25.8. The mean ± SD maximum numbers of trajectories and lesions required for middle disconnection were 7.1 ± 1.7 and 57.5 ± 18.4, respectively. All but 1 patient had class 1 outcomes according to the International League Against Epilepsy Outcome Scale at a mean ± SD (range) follow-up of 13.5 ± 1.6 (12-16) months; the remaining patient had a class 2 outcome. The estimated blood loss was < 5 ml for all patients. Complications included repeat surgery (after 2 weeks) for a "skip" area (n = 1) and a small temporal hematoma (n = 1), which resolved. CONCLUSIONS: ROTCH seems to be a safe, feasible, and bloodless procedure, with a very low morbidity rate and promising outcomes.

Is surgery effective for treating hypothalamic hamartoma causing isolated central precocious puberty? A systematic review.

The aim of this review was to determine the role of surgery in treating hypothalamic hamartoma (HH) causing isolated central precocious puberty (CPP). Literature review was done according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Patients with isolated CPP due to HH, managed with surgical resection, were included. We found 33 studies, reporting 103 patients (76 pedunculated, 27 sessile). Patients were considered "cured" if the symptoms of PP had regressed and the hormone profile had normalized after surgery. Indications for surgery included hamartoma deemed surgically resectable (n-12), for the purpose of tissue diagnosis (n-3), partial response/failure of preoperative therapy (n-9), and unable to afford/to avoid long-term medical therapy (n-7). The extent of resection was total (TR) (n-39), near total/subtotal (NTR/STR) (n-20), partial (PR) (n-35), or unspecified (n-9). On follow-up (range: 3 months-16 years), 73.6% (56/76) of patients with pedunculated HH were cured, while 17.1% (13/76) had partial relief. Only 3/27 (11.1%) of patients with sessile HH were cured. All patients with a pedunculated hamartoma who underwent TR (n=36) improved, with 88.88% cured of the symptoms. Surgery had no effect in 17/23 (73.9%) patients with sessile HH who underwent PR. Psychological symptoms improved in 10/11 patients. There was no mortality. Permanent complications, in the form of 3rd nerve palsy, occurred in 3.7% (2/54) of the patients. To conclude, in the current era of availability of GnRH analogs, surgical resection in a subset of patients may be acceptable especially for small pedunculated hamartomas.

The development and efficacy of a mobile phone application to improve medication adherence for persons with epilepsy in limited resource settings: A preliminary study.

OBJECTIVE: Persons with epilepsy (PWE), especially those with limited education backgrounds from developing countries, are challenged by complicated medication regimens, debilitating seizures, and stigmatization in their daily life. Consequently, it is difficult for physicians to ensure medication adherence. This study validates a novel mobile application which was hypothesized to increase medication adherence and self-management skills in PWE. Created by medical professionals, the application included behavioral and educational components and was built to be easy-to-understand for those of socio-economically disadvantaged backgrounds. METHODS: This was a parallel, two-armed randomized controlled trial in which a total of 96 participants were enrolled from a Neurology Outpatient Department into a control standard care group and a mobile application group that used the smartphone application (app) in addition to the standard medical treatment. The app was intuitive and easy to understand for those coming from a socio-economically disadvantaged background. Medication adherence and self-efficacy were assessed with the Morisky Green and Levine Scale (MGLS) and the Epilepsy Self Efficacy Scale (ESES). Patients were reassessed 12 weeks later. Change in seizure frequency following administration of the application was a secondary outcome. RESULTS: In an intent-to-treat analysis, the mobile application interventional group showed over a 60% increase in the proportion of medication adherence (P < 0.0001). The mean self-efficacy score for the mobile application group was increased from 269.5 to 289.75 (P < 0.0001). The control group showed no statistically significant increases in either the proportion adherent or mean self-efficacy scores. SIGNIFICANCE: This study demonstrated the statistically significant performance of a mobile application in improving medication adherence and self-management skills in Indian persons with epilepsy.