Infants with Sturge-Weber syndrome (SWS) are considered for surgery if they develop seizures and the seizures prove medically refractory. The authors report on 2 infants (15 and 19 months old) with SWS who underwent scalp video electroencephalography (EEG) and subsequent functional hemispherotomy for intractable partial motor seizures due to extensive left hemispheric angiomatosis. They presented with similar interictal and ictal EEG findings. Ictal EEG showed abrupt high-amplitude delta slow waves, without evolution on the contralateral hemisphere before the build-up of ictal EEG changes on the lesional hemisphere. The patients became seizure free after hemispherotomy. The ictal contralateral slow waves were not a sign of an ictal hemisphere and may indicate prominent ischemic changes resulting from a steal phenomenon of hemispheric angiomatosis during seizure.
Chusak Limotai, Cristina Y. Go, Shiro Baba, Kazuo Okanari, Ayako Ochi, James T. Rutka, O. Carter Snead III and Hiroshi Otsubo
Jeffrey P. Blount, Wayne Langburt, Hiroshi Otsubo, Shiro Chitoku, Ayako Ochi, Shelly Weiss, O. Carter Snead and James T. Rutka
Object. The technique involved in multiple subpial transections (MSTs) allows the surgeon treating patients with epilepsy the capability to make disconnective lesions in epileptogenic regions of eloquent cortex. Although there have been increasing numbers of reports in adults of the efficacy and relative safety of this technique, there are relatively few such reports in children. The authors present their experience in 30 children who underwent MSTs during the surgical management of the seizure disorder.
Methods. Thirty consecutive children who underwent MSTs with or without cortical excision form the basis of this retrospective review. An analysis of neurological adverse effects following MSTs and seizure outcome was performed.
Between 1996 and 2000, MSTs were performed either as stand-alone therapy (four patients) or in conjunction with planned cortical excisions (26 patients). Twenty-three children underwent invasive monitoring after placement of subdural grid electrodes, and in seven intraoperative electrocorticography alone was performed. The mean follow-up period for the group was 3.5 years (minimum 30 months in all cases). All 20 patients in whom MSTs were performed in the primary motor cortex experienced transient hemiparesis (mild in 12 and moderate in eight) lasting up to 6 weeks; however, no patient suffered a permanent motor deficit in the long-term follow-up period. In 26 patients who underwent cortical resections followed by MSTs, 12 (46%) were seizure free (Engel Class I) following surgery. Eleven patients (42%) (Engel Classes II and III) continued to suffer seizures but improvement in seizure control was adequate following surgery. In the 23 patients in whom subdural grids were placed to capture the ictal onset zone by invasive video-electroencephalography, MSTs comprised a mean of 37% of the surgically treated area under the grid.
Conclusions. The results of this series demonstrate that MSTs can be performed with acceptable morbidity in children undergoing epilepsy surgery. The precise role of MSTs in controlling seizure frequency and outcome, especially when combined with planned cortical resections, awaits further study.
Gregory W. Albert, George M. Ibrahim, Hiroshi Otsubo, Ayako Ochi, Cristina Y. Go, O. Carter Snead III, James M. Drake and James T. Rutka
Resective surgery is increasingly used in the management of pediatric epilepsy. Frequently, invasive monitoring with subdural electrodes is required to adequately map the epileptogenic focus. The risks of invasive monitoring include the need for 2 operations, infection, and CSF leak. The aim of this study was to evaluate the feasibility and outcomes of resective epilepsy surgery guided by magnetoencephalography (MEG) in children who would have otherwise been candidates for electrode implantation.
The authors reviewed the records of patients undergoing resective epilepsy surgery at the Hospital for Sick Children between 2001 and 2010. They identified cases in which resections were based on MEG data and no intracranial recordings were performed. Each patient's chart was reviewed for presentation, MRI findings, MEG findings, surgical procedure, pathology, and surgical outcome.
Sixteen patients qualified for the study. All patients had localized spike clusters on MEG and most had abnormal findings on MRI. Resection was carried out in each case based on the MEG data linked to neuronavigation and supplemented with intraoperative neuromonitoring. Overall, 62.5% of patients were seizure free following surgery, and 20% of patients experienced an improvement in seizures without attaining seizure freedom. In 2 cases, additional surgery was performed subsequently with intracranial monitoring in attempts to obtain seizure control.
MEG is a viable alternative to invasive monitoring with intracranial electrodes for planning of resective surgery in carefully selected pediatric patients with localization-related epilepsy. Good candidates for this approach include patients who have a well-delineated, localized spike cluster on MEG that is concordant with findings of other preoperative evaluations and patients with prior brain pathologies that make the implantation of subdural and depth electrodes somewhat problematic.
Odeya Bennett-Back, Ayako Ochi, Elysa Widjaja, Shohei Nambu, Akio Kamiya, Cristina Go, Sylvester Chuang, James T. Rutka, James Drake, O. Carter Snead III and Hiroshi Otsubo
Porencephalic cyst/encephalomalacia (PC/E) is a brain lesion caused by ischemic insult or hemorrhage. The authors evaluated magnetoencephalography (MEG) spike sources (MEGSS) to localize the epileptogenic zone in children with intractable epilepsy secondary to PC/E.
The authors retrospectively studied 13 children with intractable epilepsy secondary to PC/E (5 girls and 8 boys, age range 1.8–15 years), who underwent prolonged scalp video-electroencephalography (EEG), MRI, and MEG. Interictal MEGSS locations were compared with the ictal and interictal zones as determined from scalp video-EEG.
Magnetic resonance imaging showed PC/E in extratemporal lobes in 3 patients, within the temporal lobe in 2 patients, and in both temporal and extratemporal lobes in 8 patients. Magnetoencephalographic spike sources were asymmetrically clustered at the margin of PC/E in all 13 patients. One cluster of MEGSS was observed in 11 patients, 2 clusters in 1 patient, and 3 clusters in 1 patient. Ictal EEG discharges were lateralized and concordant with MEGSS in 8 patients (62%). Interictal EEG discharges were lateralized and concordant with MEGSS hemisphere in 9 patients (69%). Seven patients underwent lesionectomy in addition to MEGSS clusterectomy with (2 patients) and without (5 patients) intracranial video-EEG. Temporal lobectomy was performed in 1 patient and hemispherectomy in another. Eight of 9 patients achieved seizure freedom following surgery.
Magnetoencephalography delineated the extent of the epileptogenic zone adjacent to PC/E in patients with intractable epilepsy. Complete resection of the MEGSS cluster along with PC/E can provide favorable seizure outcomes.
Çagatay Önal, Hiroshi Otsubo, Takashi Araki, Shiro Chitoku, Ayako Ochi, Shelly Weiss, William Logan, Irene Elliott, O. Carter Snead III and James T. Rutka
Object. This study was performed to evaluate the complications of invasive subdural grid monitoring during epilepsy surgery in children.
Methods. The authors retrospectively reviewed the records of 35 consecutive children with intractable localization-related epilepsy who underwent invasive video electroencephalography (EEG) with subdural grid electrodes at The Hospital for Sick Children between 1996 and 2001. After subdural grid monitoring and identification of the epileptic regions, cortical excisions and/or multiple subpial transections (MSTs) were performed. Complications after these procedures were then categorized as either surgical or neurological.
There were 17 male and 18 female patients whose mean age was 11.7 years. The duration of epilepsy before surgery ranged from 2 to 17 years (mean 8.3 years). Fifteen children (43%) had previously undergone surgical procedures for epilepsy. The number of electrodes on the grids ranged from 40 to 117 (mean 95). During invasive video EEG, cerebrospinal fluid leaks occurred in seven patients. Also, cerebral edema (five patients), subdural hematoma (five patients), and intracerebral hematoma (three patients) were observed on postprocedural imaging studies but did not require surgical intervention. Hypertrophic scars on the scalp were observed in nine patients. There were three infections, including one case of osteomyelitis and two superficial wound infections. Blood loss and the amounts of subsequent transfusions correlated directly with the size and number of electrodes on the grids (p < 0.001). Twenty-eight children derived significant benefit from cortical resections and MSTs, with a more than 50% reduction of seizures and a mean follow-up period of 30 months.
Conclusions. The results of this study indicate that carefully selected pediatric patients with intractable epilepsy can benefit from subdural invasive monitoring procedures that entail definite but acceptable risks.
Cristina V. Torres, Aria Fallah, George M. Ibrahim, Samuel Cheshier, Hiroshi Otsubo, Ayako Ochi, Sylvester Chuang, O. Carter Snead, Stephanie Holowka and James T. Rutka
Hemispherectomy is an established neurosurgical procedure for medication-resistant epilepsy in children. Despite the effectiveness of this technique, there are patients who do not achieve an optimum outcome after surgery; possible causes of suboptimal results include the presence of bilateral independent epileptogenic foci. Magnetoencephalography (MEG) is an emerging tool that has been found to be useful in the management of lesional and nonlesional epilepsy. The authors analyzed the relative contribution of MEG in patient selection for hemispherectomy.
The medical records of children undergoing hemispherectomy at the Hospital for Sick Children were reviewed. Those patients who underwent MEG as part of the presurgical evaluation were selected.
Thirteen patients were included in the study. Nine patients were boys. The mean age at the time of surgery was 66 months (range 10–149 months). Seizure etiology was Rasmussen encephalitis in 6 patients, hemimegalencephaly in 2 patients, and cortical dysplasia in 4 patients. In 8 patients, video-EEG and MEG results were consistent to localize the primary epileptogenic hemisphere. In 2 patients, video-EEG lateralized the ictal onset, but MEG showed bilateral spikes. Two patients had bilateral video-EEG and MEG spikes. Engel Class I, II, and IV outcomes were seen in 10, 2, and 1 patients, respectively. In 2 of the patients who had an outcome other than Engel Class I, the MEG clusters were concentrated in the disconnected hemisphere. The third patient had bilateral clusters and potentially independent epileptogenic foci from bilateral cortical dysplasia.
The presence of unilateral MEG spike waves correlated with good outcomes following hemispherectomy. In some cases, MEG provides information that differs from that obtained from video-EEG and conventional MR imaging studies. Further studies with a greater number of patients are needed to assess the role of MEG in the preoperative assessment of candidates for hemispherectomy.
Zulma S. Tovar-Spinoza, Ayako Ochi, James T. Rutka, Cristina Go and Hiroshi Otsubo
Epilepsy surgery requires the precise localization of the epileptogenic zone and the anatomical localization of eloquent cortex so that these areas can be preserved during cortical resection. Magnetoencephalography (MEG) is a technique that maps interictal magnetic dipole sources onto MR imaging to produce a magnetic source image. Magneto-encephalographic spike sources can be used to localize the epileptogenic zone and be part of the workup of the patient for epilepsy surgery in conjunction with data derived from an analysis of seizure semiology, scalp video electroencephalography, PET, functional MR imaging, and neuropsychological testing. In addition, magnetoencephalographic spike sources can be linked to neuronavigation platforms for use in the neurosurgical field. Finally, paradigms have been developed so that MEG can be used to identify functional areas of the cerebral cortex including the somatosensory, motor, language, and visual evoked fields.
The authors review the basic principles of MEG and the utility of MEG for presurgical planning as well as intra-operative mapping and discuss future applications of MEG technology.
Erin N. Kiehna, Elysa Widjaja, Stephanie Holowka, O. Carter Snead III, James Drake, Shelly K. Weiss, Ayako Ochi, Eric M. Thompson, Cristina Go, Hiroshi Otsubo, Elizabeth J. Donner and James T. Rutka
Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients. The authors hypothesized that residual fiber connections between the hemispheres could be the underlying cause of recurrent epilepsy in these patients. Diffusion tensor imaging (DTI) was used to test this hypothesis, and to target residual connections at reoperation using neuronavigation.
The authors identified 8 patients with recurrent seizures following hemispherectomy who underwent surgery between 1995 and 2012. Prolonged video electroencephalography recordings documented persistent seizures arising from the affected hemisphere. In all patients, DTI demonstrated residual white matter association fibers connecting the hemispheres. A repeat craniotomy and neuronavigation-guided targeted disconnection of these residual fibers was performed. Engel class was used to determine outcome after surgery at a minimum of 2 years of follow-up.
Two patients underwent initial hemidecortication and 6 had periinsular hemispherotomy as their first procedures at a median age of 9.7 months. Initial pathologies included hemimegalencephaly (n = 4), multilobar cortical dysplasia (n = 3), and Rasmussen's encephalitis (n = 1). The mean duration of seizure freedom for the group after the initial procedure was 32.5 months (range 6–77 months). In all patients, DTI showed limited but definite residual connections between the 2 hemispheres, primarily across the rostrum/genu of the corpus callosum. The median age at reoperation was 6.8 years (range 1.3–14 years). The average time taken for reoperation was 3 hours (range 1.8–4.3 hours), with a mean blood loss of 150 ml (range 50–250 ml). One patient required a blood transfusion. Five patients are seizure free, and the remaining 3 patients are Engel Class II, with a minimum follow-up of 24 months for the group.
Repeat hemispherotomy is an option for consideration in patients with recurrent intractable epilepsy following failed surgery for catastrophic epilepsy. In conjunction with other modalities to establish seizure onset zones, advanced MRI and DTI sequences may be of value in identifying patients with residual connectivity between the affected and unaffected hemispheres. Targeted disconnection of these residual areas of connectivity using neuronavigation may result in improved seizure outcomes, with minimal and acceptable morbidity.
Ichiro Sugiyama, Katsumi Imai, Yu Yamaguchi, Ayako Ochi, Yoko Akizuki, Cristina Go, Tomoyuki Akiyama, O. Carter Snead III, James T. Rutka, James M. Drake, Elysa Widjaja, Sylvester H. Chuang, Doug Cheyne and Hiroshi Otsubo
Magnetoencephalography (MEG) has been typically used to localize epileptic activity by modeling interictal activity as equivalent current dipoles (ECDs). Synthetic aperture magnetometry (SAM) is a recently developed adaptive spatial filtering algorithm for MEG that provides some advantages over the ECD approach. The SAM-kurtosis algorithm (also known as SAM[g2]) additionally provides automated temporal detection of spike sources by using excess kurtosis value (steepness of epileptic spike on virtual sensors). To evaluate the efficacy of the SAM(g2) method, the authors applied it to readings obtained in children with intractable epilepsy secondary to tuberous sclerosis complex (TSC), and compared them to localizations obtained with ECDs.
The authors studied 13 children with TSC (7 girls) whose ages ranged from 13 months to 16.3 years (mean 7.3 years). Video electroencephalography, MR imaging, and MEG studies were analyzed. A single ECD model was applied to localize ECD clusters. The SAM(g2) value was calculated at each SAM(g2) virtual voxel in the patient's MR imaging–defined brain volume. The authors defined the epileptic voxels of SAM(g2) (evSAM[g2]) as those with local peak kurtosis values higher than half of the maximum. A clustering of ECDs had to contain ≥ 6 ECDs within 1 cm of each other, and a grouping of evSAM(g2)s had to contain ≥ 3 evSAM(g2)s within 1 cm of each other. The authors then compared both ECD clusters and evSAM(g2) groups with the resection area and correlated these data with seizure outcome.
Seizures started when patients were between 6 weeks and 8 years of age (median 6 months), and became intractable secondary to multiple tubers in all cases. Ictal onset on scalp video electroencephalography was lateralized in 8 patients (62%). The MEG studies showed multiple ECD clusters in 7 patients (54%). The SAM(g2) method showed multiple groups of epileptic voxels in 8 patients (62%). Colocalization of grouped evSAM(g2) with ECD clusters ranged from 20 to 100%, with a mean of 82%. Eight patients underwent resection of single (1 patient) and multiple (7 patients) lobes, with 6 patients achieving freedom from seizures. Of 8 patients who underwent surgery, in 7 the resection area covered ECD clusters and grouped evSAM(g2)s. In the remaining patient the resection area partially included the ECD cluster and grouped evSAM(g2)s. Six of the 7 patients became seizure free.
The combination of SAM(g2) and ECD analyses succeeded in localizing the complex epileptic zones in children with TSC who had intractable epilepsy secondary to multiple cortical tubers. For the subset of children with TSC who present with early-onset and nonlateralized seizures, MEG studies in which SAM(g2) and ECD are used might identify suitable candidates for resection to control seizures.
Koji Iida, Hiroshi Otsubo, Yuuri Matsumoto, Ayako Ochi, Makoto Oishi, Stephanie Holowka, Elizabeth Pang, Irene Elliott, Shelly K. Weiss, Sylvester H. Chuang, O. Carter Snead III and James T. Rutka
Object. The authors sought to validate magnetoencephalography spike sources (MEGSSs) in neuronavigation during epilepsy surgery in pediatric patients.
Methods. The distributions of MEGSSs in 16 children were defined and classified as clusters (Class I), greater than or equal to 20 MEGSSs with 1 cm or less between MEGSSs; small clusters (Class II), 6 to 19 with 1 cm or less between; and scatters (Class III), less than 6 or greater than 1 cm between spike sources. Using neuronavigation, the MEGSSs were correlated to epileptic zones from intra- and extraoperative electrocorticography (ECoG), surgical procedures, disease entities, and seizure outcomes.
Thirteen patients underwent MEGSSs: nine had clusters; two had small clusters, one with and one without clusters; and three had scatters alone. All 13 had scatters. Clusters localized within and extended from areas of cortical dysplasia and at margins of tumors or cystic lesions. All clusters were colocalized to ECoG-defined epileptic zones. Four of six patients with clusters and/or small clusters underwent complete excisions, and two underwent partial excision with or without multiple subpial transections. In the three patients with scatters alone, ECoG revealed epileptic zones buried within MEGSS areas; these regions of scatters were completely excised and treated with multiple subpial transections. Coexisting scatters were left untreated in nine of 10 patients. Postoperatively, nine of 13 patients were seizure free; the four patients with residual seizures had clusters in unresected eloquent cortex. Three patients in whom no MEGSSs were demonstrated underwent lesionectomies and were seizure free.
Conclusions. Magnetoencephalography spike source clusters indicate an epileptic zone requiring complete excision. Coexisting scatters remote from clusters are nonepileptogenic and do not require excision. Scatters alone, however, should be examined by ECoG; an epileptic zone may exist within these distributions.