Disconnection Surgery Research Articles

A Primer on Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Medically Refractory Epilepsy.

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

Long-term seizure outcome after epilepsy surgery in patients with mild malformation of cortical development and focal cortical dysplasia.

SummaryFocal cortical dysplasia (FCD) and mild malformation of cortical development (mMCD) are frequent histopathologic diagnoses in patients who undergo surgery for refractory epilepsy. Literature concerning surgical outcome in patients with mMCD, as well as its contrast with FCD, has been scarce. We studied 88 patients with a histopathologic diagnosis of isolated FCD (n = 57) or mMCD (n = 31), revised according to the latest International League Against Epilepsy (ILAE) guidelines, who underwent resective or disconnective surgery. Our findings suggest differences between mMCD and FCD in clinical presentation and surgical outcome after surgery. Patients with mMCD developed seizures later in life, and their lesions had a predilection for location in the temporal lobe and remained undetected by magnetic resonance imaging (MRI) more frequently. A diagnosis of mMCD has a less favorable surgical outcome. Still, 32% of these patients reached continuous seizure freedom (Engel class 1A) at a latest median follow‐up duration of 8 years, compared to 59% in FCD. A histopathologic diagnosis of mMCD, extratemporal surgery, and indication of an incomplete resection each were independent predictors of poor outcome.

Preoperative evaluation and surgical management of infants and toddlers with drug-resistant epilepsy.

OBJECTIVE Despite perioperative risks, epilepsy surgery represents a legitimate curative or palliative treatment approach for children with drug-resistant epilepsy (DRE). Several factors characterizing infants and toddlers with DRE create unique challenges regarding optimal evaluation and management. Epilepsy surgery within children < 3 years of age has received moderate attention in the literature, including mainly case series and retrospective studies. This article presents a systematic literature review and explores multidisciplinary considerations for the preoperative evaluation and surgical management of infants and toddlers with DRE. METHODS The study team conducted a systematic literature review based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, targeting studies that investigated children < 3 years of age undergoing surgical treatment of DRE. Using the PubMed database, investigators selected peer-reviewed articles that reported seizure outcomes with or without developmental outcomes and/or perioperative complications. Studies were eliminated based on the following exclusion criteria: sample size < 5 patients; and inclusion of patients > 3 years of age, when demographic and outcomes data could not be separated from the cohort of patients < 3 years of age. RESULTS The study team identified 20 studies published between January 1990 and May 2017 that satisfied eligibility criteria. All selected studies represented retrospective reviews, observational studies, and uncontrolled case series. The compiled group of studies incorporated 465 patients who underwent resective or disconnective surgery (18 studies, 444 patients) or vagus nerve stimulator insertion (2 studies, 21 patients). Patient age at surgery ranged between 28 days and 36 months, with a mean of 16.8 months (1.4 years). DISCUSSION The study team provided a detailed summary of the literature review, focusing on the etiologies, preoperative evaluation, surgical treatments, seizure and developmental outcomes, and potential for functional recovery of infants and toddlers with DRE. Additionally, the authors discussed special considerations in this vulnerable age group from the perspective of multiple disciplines. CONCLUSIONS While presenting notable challenges, pediatric epilepsy surgery within infants and toddlers (children < 3 years of age) offers significant opportunities for improved seizure frequency, neuro-cognitive development, and quality of life. Successful evaluation and treatment of young children with DRE requires special consideration of multiple aspects related to neurological and physiological immaturity and surgical morbidity.

P90. Super selective amytal injection (Wada-Test) in a 14 year old with left sided parieto-insular epilepsy due to Focal cortical dysplasia

Introduction The classical Wada-test is performed by injecting a narcotic (usually amobarbital) into the left internal carotid artery (ICA) in order to establish language dominance. In the presurgical workup of epilepsy, it has largely been replaced by less invasive methods such as functional MRI and functional Transcranial Doppler Ultrasound. In exceptional cases, the Wada-test can be performed superselectively: the narcotic is injected into a branch of the middle cerebral artery (MCA). This allows proving or excluding language function in a small brain volume. We document the utility of a superselective Wada test in a 14-year-old girl with a dysplastic lesion in the left supramarginal cortex and dorsal insula. Methods A 14 year old girl presented with pharmacoresistant epilepsy. 3 T MRI revealed a left dorsal insular/supramaginal gyrus FLAIR hyperintensity suspicious of focal cortical dysplasia as the likely epileptogenic lesion. Multimodal presurgical work-up confirmed this. Yet, it was questionable if an extended lesionectomy would harm language function. Left ACI Wada-Test was inconclusive. Therefore, a superselective Wada-Test of an M3 branch of the left MCA/perirolandic artery was performed. Results Following the application of 70 mg Amobarbital, both EEG-slowing in electrodes C3, P3, and T5 and repeat angiography confirmed the correct localization of barbiturate action. During the superselective Wada-test, the patient was able to complete a comprehensive test battery of language functions (counting, naming, reading, calculating, spontaneous speech) without evident dysfunction. Neuronavigation and intraoperative electrophysiological monitoring supported an extended lesionectomy, preserving the white matter adjacent to the pyramidal tract. Early after surgery, the patient suffered from a Gerstmann’s syndrome and slight amnestic aphasia. However, this resolved within the first postoperative weeks. Histopathology confirmed focal cortical dysplasia type IIb. Following surgery, the patient is now seizure free for 18 months under reduced antiepileptic drugs. Her academic performance has improved compared to the preoperative status. Conclusion The Wada-test simulates the effect of resective or disconnective surgery on language functions by temporarily inactivating brain areas. Technical advances in catheter angiography allow injecting the narcotic into single MCA-branches or other arteries of similar diameter, thus simulating the effect of small cortical resections. In selected patients, this method can be a valuable supplement of multimodal presurgical workup, and may obviate the need for intra- or extraoperative cortical mapping.

Disconnection Surgery for Intractable Epilepsy with a Structural Abnormality in the Medial Posterior Cortex

The lesionectomy is a general surgical procedure for treating focal epilepsy resulting from a structural abnormality, but a favorable outcome cannot be achieved in some patients, especially patients whose symptomatogenic zone is located in remote regions. Herein we propose the surgical disconnection of the seizure propagation pathway, which consists of short and long associating fibers linking the epileptogenic zone to the remote symptomatogenic zone, as an effective method of achieving favorable seizure outcomes in patients with posterior cortex epilepsy. Three patients with intractable epilepsy showing a structural abnormality in the medial posterior cortex participated in this study. Their habitual seizures were complex partial seizures stemming from remote symptomatogenic zones. Seizure propagation-related fibers were assumed by non-invasive examination and semiology. Cingulum and superior/inferior longitudinal fasciculus were considered to form main seizure propagation pathway. Based on the preoperative assumption and the intraoperative intracranial electroencephalogram findings, a lesionectomy and corticectomy were performed for 2 patients while a clusterectomy and corticectomy were performed for the remaining patient. The resection area was extended in the direction of the association fibers to disconnect the remote symptomatogenic zone completely from the epileptogenic zone. Engel class I was achieved in all the patients. The current study suggested that assuming the presence of association fibers was an important factor for achieving a favorable outcome in the surgical treatment of posterior cortex epilepsy. Though further study is required, disconnection surgery is recommended as a treatment option for cases in which the epileptogenic zone is located in an eloquent area.

Tracking the Re-organization of Motor Functions After Disconnective Surgery: A Longitudinal fMRI and DTI Study.

Objective: Mechanisms of motor plasticity are critical to maintain motor functions after cerebral damage. This study explores the mechanisms of motor reorganization occurring before and after surgery in four patients with drug-refractory epilepsy candidate to disconnective surgery.Methods: We studied four patients with early damage, who underwent tailored hemispheric surgery in adulthood, removing the cortical motor areas and disconnecting the corticospinal tract (CST) from the affected hemisphere. Motor functions were assessed clinically, with functional MRI (fMRI) tasks of arm and leg movement and Diffusion Tensor Imaging (DTI) before and after surgery with assessments of up to 3 years. Quantifications of fMRI motor activations and DTI fractional anisotropy (FA) color maps were performed to assess the lateralization of motor network. We hypothesized that lateralization of motor circuits assessed preoperatively with fMRI and DTI was useful to evaluate the motor outcome in these patients.Results: In two cases preoperative DTI-tractography did not reconstruct the CST, and FA-maps were strongly asymmetric. In the other two cases, the affected CST appeared reduced compared to the contralateral one, with modest asymmetry in the FA-maps. fMRI showed different degrees of lateralization of the motor network and the SMA of the intact hemisphere was mostly engaged in all cases. After surgery, patients with a strongly lateralized motor network showed a stable performance. By contrast, a patient with a more bilateral pattern showed worsening of the upper limb function. For all cases, fMRI activations shifted to the intact hemisphere. Structural alterations of motor circuits, observed with FA values, continued beyond 1 year after surgery.Conclusion: In our case series fMRI and DTI could track the longitudinal reorganization of motor functions. In these four patients the more the paretic limbs recruited the intact hemisphere in primary motor and associative areas, the greater the chances were of maintaining elementary motor functions after adult surgery. In particular, DTI-tractography and quantification of FA-maps were useful to assess the lateralization of motor network. In these cases reorganization of motor connectivity continued for long time periods after surgery.

Ictal and interictal MEG in pediatric patients with tuberous sclerosis and drug resistant epilepsy

PurposeDrug resistant epilepsy (DRE) is common in patients with tuberous sclerosis (TS). Interictal MEG has been shown as a valuable instrument in the presurgical workup. The goal of our study was to evaluate the role of ictal MEG in epileptogenic tuber selection, especially in patients with multiple irritative zones. MethodsThe clinical and MEG data of 23 patients with TS and DRE from two medical/research centers were reviewed. Seven pediatric patients, who had seizures during MEG recording and underwent resection or disconnection surgery, were included into the study. Cortical sources of ictal and interictal epileptiform MEG discharges were compared with epileptogenic zone location in six patients with favorable surgery outcome. ResultsIn patients who improved substantially after surgery all resected and several other tubers demonstrated epileptiform activity on interictal MEG. Ictal MEG provided crucial information about lobar location of the seizure onset zone (SOZ) in two cases, and in the other four it confirmed the SOZ location derived from the interictal data. In one case, ictal MEG findings were unreliable. In one patient, who did not benefit from surgical treatment, the resected tubers did not overlap with interictal and ictal MEG sources. ConclusionThe combination of interictal and ictal MEG is a valuable tool for identification of the epileptogenic tuber/tubers in presurgical work-up in patients with TS.

The Wada test might predict postoperative fine finger motor deficit after hemispherotomy

IntroductionCerebral hemispherotomy is a surgical method with a high rate of seizure reduction in patients with intractable epilepsy. However, there is a probability of postoperative motor deficits. The objective of this study was to investigate whether the Wada test can help predict motor function outcomes after hemispherotomy and, therefore, may be useful in decision-making and patient selection. Patients and methodA total of 13 patients with hemispherical intractable epilepsy underwent hemispherical disconnection surgeries. Six of them underwent the Wada test to evaluate motor function and language function followed by peri-insula hemispherotomy. The patients’ age ranged from 11 to 45years (mean 27years). ResultsThree of six patients had reduced dexterity on the Wada test. The finger motor function in the other patients did not change on the Wada test. Postoperatively, all patients who had decreased fine motor movement on the Wada test showed postoperative clumsiness of their hands and fingers. ConclusionsThe Wada test might predict postoperative fine finger motor deficit after hemispherotomy. This study showed that gross motor function was compensated in the ipsilateral hemisphere, whereas fine finger motor movement function remained in the contralateral frontal cortex.

Epilepsy surgery in children.

Approximately 60% of all patients with epilepsy suffer from focal epilepsy syndromes. In approximately 15% of these patients, the seizures are not adequately controlled with anticonvulsive drugs, and such patients are potential candidates for surgical treatment and majority are children. Epilepsy surgery in children, who have been carefully chosen, can result in either seizure freedom or a marked (>90%) reduction in seizures in approximately two-third of children with intractable seizures. In the multimodality presurgical evaluation approach, sufficient concordance should be established among various independent investigations, thus identifying the location and extent of the epileptogenic zone with a high degree of confidence. Early surgery improves the quality of life and cognitive and developmental outcome of the child. Surgically remediable epilepsies in children should be identified early and include temporal lobe epilepsy with focal lesions, lesional extratemporal epilepsies, hemispherical epilepsies, and gelastic epilepsy with hypothalamic hamartoma, and can be treated by resective or disconnection surgery. Palliative procedures include corpus callosotomy and vagal nerve stimulation for children with diffuse and multifocal epilepsies, who are not candidates for resective surgery. Deep brain stimulation in patients with epilepsy is still under evaluation. For children with "surgically remedial epilepsy," surgery should be offered as a procedure of choice rather than as a treatment of last resort.

Seizure Freedom after Leucotomy in a Childhood Refractory Epilepsy Associated with Schizencephaly and Multifocal Cortical Malformation

Schizencephaly associated with extensive malformation of cortical developments frequently cause medically intractable epilepsy in pediatric patients. Because of the extent of the structural abnormality, which is often bihemispheric and multifocal, epileptic surgery in these group of patients is typically not considered or indicated. We report a 10-year-old boy with medically intractable epilepsy and multifocal epileptiform discharges associated with bilateral malformation of cortical developments, schizencephaly, and agenesis of the corpus callosum, initially thought to be not a candidate for epilepsy surgery. All of the seizures localized to the right posterior head region. Seizure freedom was achieved after a right temporoparietooccipital (TPO) disconnection. He was followed postoperatively for 22 months at which time he remained on oxcarbazepine monotherapy. We emphasize that medically intractable epileptic patients with unilateral schizencephaly, bilateral colpocephaly, and multifocal epileptiform discharges must be evaluated with a video electroencephalography (VEEG) to characterize the seizures before ruling out epilepsy surgery as a therapeutic option. TPO disconnection surgery, instead of multilobar resections, can result in favorable seizure outcome in patients with localized seizure onset in the posterior head region.

Less invasive disconnection surgery using advanced image guidance for wide spread cortical malformations

Less invasive disconnection surgery using advanced image guidance for wide spread cortical malformations

Impact of intraoperative MRI on outcomes in epilepsy surgery: preliminary experience of two years

Purpose. To determine the impact of intraoperative magnetic resonance imaging (iMRI) in epilepsy surgeries on the extent of surgical resection and seizure outcome along with its feasibility and limitations. Methods. Patients with pharmacoresistant epilepsy (PRE), who underwent surgeries in operating theater equipped with high-field 1.5-Tesla MRI, were evaluated for extent of resection, operative time, scanning time, pathologies, resultant extra resection, and seizure outcomes. Results. Thirty-nine patients with mean age of 18 (range: 3–65) years with PRE underwent surgical intervention. Mean duration of epilepsy was 10.2 years. Surgical interventions included tumor resection (31%), resection of focal cortical dysplasia (28%), mesial temporal lobe surgeries (18%), and disconnection surgeries (23%). iMRI alone, apart from navigation and electrophysiology, improved resection rates in 13% (5 out of 39) of these patients. In lesional group, iMRI modified operative strategy resulting in increased resections in 21% (5/23) patients. Complete resection was observed in 87% of patients. iMRI scanning time constituted 25% (mean: 72 ± 21 min) of time spent under anesthesia by the patient. Major and minor complications were observed in 2.5% and 7.5% of patients, respectively. The mean follow-up was 14 months. Favorable postoperative seizure control (Engel Classes I and II) was achieved in 85% and complete seizure freedom was achieved in 77% of patients (Engel Class IA) at 1-year follow-up. Conclusions. iMRI increases the extent of resection mainly in lesional epilepsy surgeries translating into good seizure outcomes but not found to be much beneficial in prototype mesial temporal sclerosis surgeries and disconnection surgeries.

Endoscopic disconnection of hypothalamic hamartomas: safety and feasibility of robot-assisted, thulium laser–based procedures

Hypothalamic hamartomas (HH) may induce drug-resistant epilepsy (DRE), thereby requiring surgical treatment. Conventionally, treatment is aimed at removing the lesion, but a disconnection procedure has been shown to be safer and at least as effective. The thulium laser (Revolix) has been recently introduced in urological endoscopy because of its ability to deliver a smooth cut with good control of the extent of tissue damage. The authors sought to analyze the safety and efficacy of the thulium 2-μm laser applied through navigated, robot-assisted endoscopy in disconnection surgery for HHs. Twenty patients with HH who were drug resistant were treated during a 12-month period. Conventional disconnection by monopolar coagulation (endoscopic electrode) was performed in 13 patients, and thulium laser disconnection was performed in the remaining 7 patients. The endoscope was inserted into the ventricle contralateral to the attachment of the HH on the third ventricular wall. Results in terms of safety, efficacy, and ease of use of the instrument were analyzed. All 20 patients achieved a satisfactory postoperative Engel score (Classes I-III). At 12 months, the Engel class was I or II in 8 of 13 patients (61.5%) who underwent monopolar coagulation and in 6 of 7 patients (85.7%) who underwent laser disconnection (p = 0.04). Seven of 13 patients (53.8%) who underwent monopolar coagulator disconnection and 2 of 7 patients (28.6%) who underwent laser disconnection had immediate postoperative complications. At the 3-month follow-up, only 2 patients (15.4%) treated by coagulation still experienced mild surgery-related recent memory deficits. No complications persisted at the 12-month follow-up. The disconnection procedure is a safe and effective treatment strategy to treat drug-resistant epilepsy in patients with HHs. With the limitations of initial experience and a short-term follow-up, it appears that the thulium 2-μm laser has the technical features to replace the standard coagulation in this procedure.

Perioperative anesthetic implications of epilepsy surgery: a retrospective analysis.

Drug-resistant epilepsy (DRE) occurs in about 30% of individuals with epilepsy. For seizure control, a wide range of surgical procedures are performed, depending on the underlying pathology. To address the anesthetic and perioperative concerns in these patients, we analyzed the data of persons with DRE who underwent epilepsy surgery at our institute. A retrospective analysis of patients who underwent epilepsy surgery from 2005-2010 was performed. For data collection and analysis, patients were divided into three groups: Group I (temporal lobe epilepsy), Group II (extratemporal lobe epilepsy), and Group III (multilobar epilepsy and others). A total of 241 surgical procedures were performed on 235 persons with DRE. The procedures included temporal (149) and extratemporal (47) lobe resection, hemispherotomy (31), corpus callosotomy (5), vagus nerve stimulation (3), and implantation of invasive cerebral electrodes (6). General anesthesia was the more common anesthetic technique; awake craniotomy was performed in only five cases. Intraoperative neuromonitoring was used most frequently in Group II. Patients in Group III had the longest intraoperative course and the greatest blood loss. The overall incidence of postoperative mechanical ventilation was 17.84%, with 53.84% of patients in Group III alone. At one-year follow-up, a good outcome was seen in 78% of temporal lobe resection, 55% of extratemporal cortical resection, 82% of hemispherotomy, and 80% of corpus callosotomy procedures. Careful preoperative selection and meticulous perioperative management are the most significant factors for success of epilepsy surgery. Although temporal and extratemporal lobe surgeries have a fairly stable perioperative course, multilobar epilepsy requiring disconnective surgery poses a greater challenge.

Disconnective surgery in posterior quadrantic epilepsy: a series of 12 paediatric patients.

To assess the surgical outcomes of temporo-parieto-occipital (TPO) and parieto-occipital (PO) disconnection surgery for children with intractable posterior quadrantic epilepsy and a unilateral posterior quadrant lesion based on MRI and functional imaging abnormality in the TPO region on one side. A retrospective review of data of 12 children who underwent TPO or PO disconnective surgery was carried out from September 2009 to September 2012. Three-dimensional surface reconstructions of MRI scans and intraoperative electrophysiological monitoring were used during surgery. Drugs were not discontinued after surgery in any patient. The affected hemisphere was the left in seven patients and the right in five patients. The mean ages at seizure onset and at surgery were four years and 12.3 years, respectively. At the time of surgery, 3 children had atonic seizures, 4 had symptomatic epilepsy with focal seizures and alteration of conscioussness, 4 had secondarily generalised seizures, and 1 child had spasms and tonic seizures. All patients had developmental delay. A pure TPO disconnection was performed in 11 patients and a PO disconnection was performed in the remaining patient. On pathological examination, 3 patients were shown to have focal cortical dysplasia (FCD) Ib, 2 with FCD IIa, 5 with FCD IIb, 1 with gliosis, and 1 with gliosis plus FCD IIa. Following surgery, 2 patients had oedema; 1 required another operation to resect the occipital lobe. At a mean follow-up of 34.5 months, 9 patients (75%) were classified as Engel class I, 2 as Engel Class II, and 1 as Engel class III. All 12 children had contralateral hemianopia postoperatively and improvement in median IQ (p=0.04) was reported three months postoperatively. With respect to the limits of a retrospective and relatively small sample size series TPO and PO disconnection are safe and effective motor-sparing epilepsy surgical procedures in selected patients with the epileptiform zone located in the posterior quadrant on one side.

Hemispherotomy in Rasmussen encephalitis: Long-term outcome in an Italian series of 16 patients

Surgical disconnection of the affected hemisphere is considered the treatment of choice for Rasmussen encephalitis (RE), however few data on long-term outcomes after disconnective surgery are available. We report on long-term seizure, cognitive and motor outcomes after disconnective surgery in 16 (8 M, 8 F) RE patients. Pre- and post-operative evaluations included long-term video-EEG monitoring, MRI, assessment of motor function, and cognitive evaluation. Hemispherotomy, by various techniques was used to obtain functional disconnection of the affected hemisphere. The patients, of median current age 23.5 years, range 12-33, were operated on between 1993 and 2009. Median age at disease onset was 5.8 years (range 3-11.4). Median time from seizure onset to surgery was 3.8 years, range 8 months to 21 years. Post-surgical follow-up was a median of 9.5 years, range 3-20. At surgery all patients were receiving two or more antiepileptic drugs (AEDs). All but three patients were seizure-free at latest follow-up. AEDs had been stopped in ten patients; in the remaining six AEDs were markedly reduced. Postural control improved in all patients. Gain in cognitive functioning was significantly (p=0.002) related to disease duration. The long-term outcomes, in terms of seizure control, motor improvement, and cognitive improvement provide important support for disconnective surgery as first choice treatment for RE.

Long‐term outcome after limited cortical resections in two cases of adult‐onset Rasmussen encephalitis

Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug-refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult-onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases. A PowerPoint slide summarizing this article is available for download in the Supporting Information section http://dx.doi.org/10.1111/epi.12596/supinfo.

Posterior quadrant disconnection surgery for Sturge‐Weber syndrome

Some patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development. Ten patients who were surgically treated using the posterior quadrantectomy (PQT) were enrolled in this study. Surgical outcome was analyzed as seizure-free or not at 2 years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (MDI) and psychomotor developmental index (PDI) in the Bayley Scales of Infant Development II preoperatively, and at 6 and 12 months after the PQT. Eight of 10 patients were seizure-free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI scores before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12 months after the PQT in seizure-free patients were 80.5 and 84.5, respectively (p < 0.01). PDI scores at these postoperative intervals were 87.3 and 86.4, respectively (p < 0.05). Patients with residual seizures did not improve in either MDI or PDI. The PQT achieved good seizure control and improved psychomotor development in patients with SWS. The complete deafferentation of angiomatous areas is required for seizure-free results and psychomotor developmental improvement.

Surgery for childhood epilepsy.

Approximately 60% of all patients with epilepsy suffer from focal epilepsy syndromes. In about 15% of these patients, the seizures are not adequately controlled with antiepileptic drugs; such patients are potential candidates for surgical treatment and the major proportion is in the pediatric group (18 years old or less). Epilepsy surgery in children who have been carefully chosen can result in either seizure freedom or a marked (>90%) reduction in seizures in approximately two-thirds of children with intractable seizures. Advances in structural and functional neuroimaging, neurosurgery, and neuroanaesthesia have improved the outcomes of surgery for children with intractable epilepsy. Early surgery improves the quality of life and cognitive and developmental outcome and allows the child to lead a normal life. Surgically remediable epilepsies should be identified early and include temporal lobe epilepsy with hippocampal sclerosis, lesional temporal and extratemporal epilepsy, hemispherical epilepsy, and gelastic epilepsy with hypothalamic hamartoma. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. Palliative procedures are performed in children with diffuse and multifocal epilepsies who are not candidates for resective surgery. The palliative procedures include corpus callosotomy and vagal nerve stimulation while deep brain stimulation in epilepsy is still under evaluation. For children with “surgically remediable epilepsy,” surgery should be offered as a procedure of choice rather than as a treatment of last resort.

Posterior quadrantic disconnection maintains the activity of isolated temporal-parietal-occipital nerve tissue: neuroprotective measures in the surgical treatment of epilepsy.

Posterior quadrantic disconnection maintains the activity of isolated temporal-parietal-occipital nerve tissue: neuroprotective measures in the surgical treatment of epilepsy.