A not so incidental 'incidentaloma' - pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review.

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental. Surgical resection led to a robust clinical improvement, and pathology revealed a benign ganglioneuroma. This report widens the spectrum of paraneoplastic manifestations of ganglioneuroma, reviews the diagnostic approach to antibody-negative pediatric AE, and raises important clinical considerations regarding benign and incidentally found tumors in the setting of a suspected paraneoplastic neurologic syndrome.

Characterization and Prediction of Short-term Outcomes in Memory After Temporal Lobe Resection in Children With Epilepsy.

BACKGROUND AND OBJECTIVES: The aim of this study was to characterize short-term outcomes in episodic memory, as assessed by the Children's Memory Scale (CMS), after temporal lobe resection in children with epilepsy using empirical methods for assessing cognitive change (i.e., reliable change indices [RCI] and standardized regression-based change scores [SRB]) and develop and internally validate clinically applicable models to predict postoperative memory decline. METHODS: This retrospective cohort study included children aged 6-16 years who underwent resective epilepsy surgery that included the temporal lobe (temporal only: "temporal" and multilobar: "temporal plus") and who completed preoperative and postoperative neuropsychological assessments including the CMS. Change scores on the CMS delayed memory subtests (Faces, Stories, and Word Pairs) were classified as decline, no change, or improvement using epilepsy-specific RCI and SRB. Logistic regression models for predicting postoperative memory decline were developed and internally validated with bootstrapping. RESULTS: Of the 126 children included, most of them demonstrated either no significant change (54%-69%) or improvement (8%-14%) in memory performance using RCI on individual measures at a median of 7 months after surgery. A subset of children (23%-33%) showed postoperative declines. Change distributions obtained using RCI and SRB were not statistically significantly different from each other. Preoperative memory test score, surgery side, surgery extent, and preoperative full-scale IQ were predictors of memory decline. Prediction models for memory decline included subsets of these variables with bias-corrected concordance statistics ranging from 0.70 to 0.75. The models were well calibrated although slightly overestimated the probability of verbal memory decline in high-risk patients. DISCUSSION: This study used empiric methodology to characterize memory outcome in children after temporal lobe resection. Provided online calculator and nomograms may be used by clinicians to estimate the risk of postoperative memory decline for individual patients before surgery.

Scoring Systems for the Evaluation of Hypsarrhythmia.

SUMMARY: Hypsarrhythmia is a well-recognized EEG pattern and it has been long known as a feature of one of the more severe forms of epilepsy early in life with adverse consequences if not recognized and treated promptly. Yet, it has been difficult to objectively quantify its varied manifestations and link them to the clinical severity of epilepsy, implications for treatment, or the overall seizure and developmental outcome of young patients. This is not for want of effort, for there have been several approaches to score hypsarrhythmia ever since its initial recognition as a unique EEG pattern. In this article, we review the different methods proposed to score hypsarrhythmia and highlight the challenges inherent to each one of them.

Mother knows best or does she? Perceptions of the memory abilities of pediatric patients with epilepsy as reported by patients and their parents across time.

PURPOSE: This study compared the self-reported and parent-reported memory of children with epilepsy across time and explored the relationships between these measures of subjective memory and the children's actual performance on objective neuropsychological tests. METHOD: One-hundred and nineteen children with epilepsy who were surgical candidates underwent comprehensive neuropsychological testing that included the Everyday Verbal Memory Questionnaire (EVMQ). Each child's parent and 82 of the children themselves completed the appropriate version of this subjective memory measure. After 9 months, the children returned for a second neuropsychological evaluation with 71 parents and 39 children completing the same questionnaire. Approximately one-third of the children in the study underwent surgery between the two evaluations. Standardized regression-based norms were used to quantify change in cognitive abilities across assessments. RESULTS: Results revealed significant relationships between parent reports and child reports of the children's memory abilities. Parent reports, but not child reports, correlated with the children's objective test scores at baseline. In contrast, children were more attuned to changes in their memory across time. CONCLUSIONS: These findings demonstrate the importance of considering both parent and child perceptions of everyday cognitive functioning when evaluating cognition and cognitive changes over time in pediatric patients with epilepsy.

Epilepsy with Eyelid myoclonias - A diagnosis concealed in other genetic generalized epilepsies with photoparoxysmal response.

OBJECTIVE: Epilepsy with eyelid myoclonias(EMA) is a genetic generalized epilepsy (GGE) characterized by eyelid myoclonia, eye-closure sensitivity and photosensitivity. Data on EMA patients who specifically present with photoparoxysmal response on EEG is lacking. EMA is an under-recognized syndrome which is frequently misclassified as another GGE. The main objective of our research is to describe the occurrence of EMA versus other GGEs among patients with photoparoxysmal response and evaluate their distinguishing features. METHODS: We retrospectively identified all patients who had photoparoxysmal response on EEGs performed at Cleveland clinic between 01/01/2012 and 12/31/2019. Initial epilepsy diagnosis and clinical data were collected. EEGs were reviewed for eyelid myoclonia and eye-closure-sensitivity which were used as main diagnostic clues for EMA. If clinical criteria was met, diagnosis was revised as EMA. RESULTS: Of 249 patients with photoparoxysmal response, 70(28.1%) met EMA criteria. Sixty-two (88.6%) were females. Mean age of onset of epilepsy was 7 years (+7.9) and 120(48.2%) had other GGEs. Fifty-four (77.1%) patients with EMA were initially classified as another epilepsy. Initial diagnosis included CAE or JME in 40(57.1%) patients with EMA so we compared EMA with these syndromes. Female preponderance, drug refractoriness, older age of onset and generalized myoclonia were more common in EMA than CAE. Earlier age of onset, absence seizures, and lack of generalized myoclonic jerks were more common EMA than JME. SIGNIFICANCE: Our study demonstrates that EMA is under-recognized among GGE patients with photoparoxysmal response. It highlights distinguishing clinical and electrographic features which separate EMA from other GGEs. It emphasizes the diverse treatments utilized and the need for therapeutic options for patients with refractory EMA.

Cognitive outcomes following pediatric epilepsy surgery.

OBJECTIVES: To characterize outcomes following pediatric epilepsy surgery across a broad range of cognitive domains using empirical methods (i.e., reliable change indices: RCIs), compare these outcomes with those based on traditional methods (i.e., standard deviation: SD), and identify factors associated with postoperative cognitive declines and/or improvements. METHODS: This retrospective cohort study included 186 children who underwent surgical resection for treatment of pharmacoresistant epilepsy and who completed pre- and postoperative neuropsychological assessments. Postoperative testing occurred approximately 6.5 months after surgery and included measures of intelligence, attention/working memory, processing speed, language, executive functioning, visuospatial skills, memory, and academic achievement. Change scores for each patient were classified as decline, no change, or improvement using epilepsy-specific RCIs. Chi-square goodness of fit tests were used to compare the distribution of outcomes as classified with RCIs to those obtained using a traditional one SD cutoff. Multinomial regression analyses were conducted to identify factors associated with cognitive decline and/or improvement. RESULTS: While 18% of children demonstrated no postoperative declines or improvements in any cognitive domain, the majority demonstrated relatively focal changes (declines and/or improvements in 1-2 cognitive domains). Rates of postoperative decline and improvement across individual cognitive domains were variable and ranged from 4-35% and 2-31%, respectively. Compared to RCIs, SD methodology often overestimated postoperative improvements and varied with respect to declines. Factors associated with RCI decline or improvement included preoperative performance, age at surgery, surgery site, and postoperative seizures. SIGNIFICANCE: Results suggest substantial variability in individual cognitive outcomes approximately 6.5 months following pediatric epilepsy surgery. The differences in change distributions obtained using epilepsy-specific RCIs versus SDs highlight the need for studies using empiric methodology to study postoperative cognitive change. Variables associated with postoperative cognitive change may be used to develop multivariable prediction models in future studies to aid clinical decision-making and patient counseling.

The Efficacy and Use of a Pocket Card Algorithm in Status Epilepticus Treatment.

OBJECTIVE: To determine whether a pocket card treatment algorithm improves the early treatment of status epilepticus and to assess its utilization and retention in clinical practice. METHODS: Multidisciplinary care teams participated in video-recorded status epilepticus simulation sessions from 2015 to 2019. In this longitudinal cohort study, we examined the sessions recorded before and after introducing an internally developed, guideline-derived pocket card to determine differences in the adequacy or timeliness of rescue benzodiazepine. Simulation participants were queried 9 months later for submission of a differentiating identification number on each card to assess ongoing availability and utilization. RESULTS: Forty-four teams were included (22 before and 22 after the introduction of the pocket card). The time to rescue therapy was shorter for teams with the pocket card available (84 seconds [64-132]) compared with teams before introduction (144 seconds [100-162]) (U = 94; median difference = -46.9, 95% confidence interval [CI]: -75.9 to -21.9). The adequate dosing did not differ with card availability (odds ratio 1.48, 95% CI: 0.43-5.1). At the 9-month follow-up, 32 participants (65%) completed the survey, with 26 (81%) self-reporting having the pocket card available and 11 (34%) confirming ready access with the identification number. All identification numbers submitted corresponded to the hard copy laminated pocket card, and none to the electronic version. CONCLUSIONS: A pocket card is a feasible, effective, and worthwhile educational tool to improve the implementation of updated guidelines for the treatment of status epilepticus.

Cognitive outcomes following frontal lobe resection for treatment of epilepsy in children and adolescents.

OBJECTIVE: To use reliable change indices (RCIs) developed specifically for pediatric patients with epilepsy to examine cognitive outcomes after frontal lobe resection for pharmacoresistant epilepsy. METHODS: Forty-one pediatric patients (25 male, Mage = 10 years) completed comprehensive neuropsychological evaluations before and an average of 6.5 months after frontal lobe resections for treatment of epilepsy. Evaluations included tests of intelligence, attention/working memory, processing speed, language, visuospatial skills, executive function, and episodic memory. Practice effect-adjusted RCIs were used to determine clinically significant postoperative cognitive change. Demographic, disease, and surgical variables were examined to identify factors associated with postoperative cognitive decline or improvement. RESULTS: Within each cognitive domain, there was a large proportion of patients (51-84%) who did not exhibit significant cognitive change. In terms of overall cognitive profile, 44% demonstrated improvement in at least one domain and 69% declined in at least one domain. Postoperative cognitive improvement occurred most commonly in the domain of processing speed, whereas postoperative cognitive decline occurred most frequently in the domain of visuospatial skills. Younger age at surgery was associated with cognitive improvement. Older age at seizure onset and higher baseline cognitive performance were associated with cognitive decline. SIGNIFICANCE: Approximately 6.5 months after frontal lobe resection, only 15% of our sample showed stable performance across all cognitive domains. Seventeen percent of patients showed improvements without declines, 42% showed declines without improvements, and 27% showed a mix of improvements and declines across different cognitive domains. Age and baseline abilities were associated with postoperative cognitive change on multiple measures. With 1 in 8 children demonstrating postoperative decline across three or more domains, further research is needed to identify factors associated with cognitive decline in order to inform clinical decision-making and patient/family counseling.

Surgical candidates in children with epileptic spasms can be selected without invasive monitoring: A report of 70 cases.

OBJECTIVE: Prior surgical series in children with drug-resistant epileptic spasms have reported use of intracranial EEG monitoring in up to two-third of patients. We report outcome after epilepsy surgery for drug-resistant epileptic spasms in a cohort of children without the use of intracranial EEG monitoring in any of the patients. METHODS: Medical records of all consecutive children aged 5 years or under who had epilepsy surgery for epileptic spasms at Cleveland Clinic between 2000 and 2018 were reviewed. Post-operative seizure outcome and predictors of prognosis of seizure outcome were analyzed. RESULTS: Seventy children with active epileptic spasms underwent surgical resections during the study period. Mean age at seizure onset was 6.8 (+9.31) months and median age at surgery was 18.5 months. An epileptogenic lesion was identified on brain MRI in all patients; 17 (24%) had bilateral abnormalities. Etiologies included malformations of cortical development (58%), perinatal infarct/encephalomalacia (39%), and tumor (3%). None of the patients had intracranial EEG. Surgical procedures included hemispherectomy (44%), lobectomy/ lesionectomy (33%), and multilobar resections (23%). Twelve children needed repeat surgery; six (50%) became seizure free after the second surgery. At six months follow-up, 73% (51/70) were seizure-free since surgery. At a mean follow-up of 4.7 years, 60% (42/70) had Engel 1 outcome. In those with seizure recurrence, 17 (60%) reported improvement. Shorter epilepsy duration (p = 0.05) and lobar or sub-lobar epileptogenic lesions (p = 0.02) predicted favorable seizure outcome at 6 months after surgery. For long term outcome, patients with bilateral abnormalities on MRI (p = 0.001), and multilobar extent on MRI (p = 0.02) were at higher risk for recurrence. SIGNIFICANCE: Children with drug-resistant epileptic spasms secondary to an epileptogenic lesion detected on MRI could be selected for epilepsy surgery without undergoing intracranial EEG monitoring. A surgical selection paradigm without intracranial monitoring may allow early surgery without the risks of invasive monitoring.

Epilepsy surgery near or in eloquent cortex in children-Practice patterns and recommendations for minimizing and reporting deficits.

OBJECTIVE: We aimed to investigate the current practices guiding surgical resection strategies involving epileptogenic zones (EZs) near or in eloquent cortex (EC) at pediatric epilepsy surgery centers worldwide. METHODS: A survey was conducted among 40 respondents from 33 pediatric epilepsy surgery centers worldwide on the weight assigned to diagnostic tests used to define the EZ and EC, how EC is viewed, and how surgeries are planned for foci near or in eloquent cortex. RESULTS: A descriptive analysis was performed that revealed considerable variation in the use of diagnostic tests and resective strategies toward EZ and EC. SIGNIFICANCE: The wide variation in strategies may contribute to undesirable outcomes characterized by poor seizure control with added deficits and underscores the need to establish best practices in pediatric epilepsy surgery. The survey data were used to formulate a set of recommendations to help minimize deficits and to report them consistently.

Antiepileptic drugs--a review.

Epilepsy is a common neurological condition that affects approximately 1% of the general population. In addition, about 10% of the population experiences a seizure sometime during life. The treatment options for epilepsy have come a long way from the bromides to the current era in which we now have multiple treatment modalities, including medications, implantable devices, and surgery. Antiepileptic drugs (AEDs) are the mainstay for treatment of epilepsy with about 70% of children achieving good control with medications alone. The past decade has witnessed the emergence of multiple AEDs-with more than 24 AEDs to choose from presently. The newer drugs provide us with novel mechanisms of action and improved safety profile. This expanded choice of AEDs has made it possible to offer tailored-treatment plans based on unique patient profiles. However, such an ever-increasing choice of medications also poses a challenge for the treating physician as far as choosing the initial drug is concerned-especially because there is limited data comparing the efficacy of one drug to the other. An additional humbling fact remains that, despite an increase in the choice of medications, we are still only able to treat the symptoms of seizures without making any significant progress in reversing or stopping the underlying mechanism of epileptogenesis or in offering neuroprotection from epileptogenesis. Therefore, it is not surprising that, despite the wide array of AED choices, the prevalence of drug-resistant epilepsy has not improved. This article aims at giving a short overview of currently available AEDs.

Stereoelectroencephalography in children with cortical dysplasia: technique and results.

The stereoelectroencephalophraphy (SEEG) method was developed in France by Jean Tailarach and Jean Bancaud during the 50s and has been mostly used in France and Italy, as the method of choice for extraoperative invasive mapping in refractory focal epilepsy. Subsequently, for more than 60 years, SEEG has shown to be a valuable tool for preoperative decision-making in focal epilepsy. Nevertheless, there are few reports addressing the utility and safety of the SEEG methodology applied to children and adolescents. In this chapter, we will discuss the current results of SEEG in pediatric patients with difficult to localize epilepsy. Details regarding surgical technique and clinical results will be presented.

Stereoelectroencephalography in children and adolescents with difficult-to-localize refractory focal epilepsy.

BACKGROUND: Although stereoelectroencephalography (SEEG) has been shown to be a valuable tool for preoperative decision making in focal epilepsy, there are few reports addressing the utility and safety of SEEG methodology applied to children and adolescents. OBJECTIVE: To present the results of our early experience using SEEG in pediatric patients with difficult-to-localize epilepsy who were not considered candidates for subdural grid evaluation. METHODS: Thirty children and adolescents with the diagnosis of medically refractory focal epilepsy (not considered ideal candidates for subdural grids and strip placement) underwent SEEG implantation. Demographics, electrophysiological localization of the hypothetical epileptogenic zone, complications, and seizure outcome after resections were analyzed. RESULTS: Eighteen patients (60%) underwent resections after SEEG implantations. In patients who did not undergo resections (12 patients), reasons included failure to localize the epileptogenic zone (4 patients); multifocal epileptogenic zone (4 patients); epileptogenic zone located in eloquent cortex, preventing resection (3 patients); and improvement in seizures after the implantation (1 patient). In patients who subsequently underwent resections, 10 patients (55.5%) were seizure free (Engel class I) and 5 patients (27.7%) experienced seizure improvement (Engel class II or III) at the end of the follow-up period (mean, 25.9 months; range, 12 to 47 months). The complication rate in SEEG implantations was 3%. CONCLUSION: The SEEG methodology is safe and should be considered in children/adolescents with difficult-to-localize epilepsy. When applied to highly complex and difficult-to-localize pediatric patients, SEEG may provide an additional opportunity for seizure freedom in association with a low morbidity rate.

Long-term functional outcomes and their predictors after hemispherectomy in 115 children.

PURPOSE: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems. METHODS: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center. Preoperative clinical, electroencephalography (EEG), imaging, and surgical data were collected. One hundred twenty-five families completed a structured questionnaire to assess the functional status and seizure outcome. Prognostic predictors were examined using a multivariate regression analysis. KEY FINDINGS: At a mean follow-up of 6.05 years after hemispherectomy, 70 patients (56%) were seizure-free and 45 (36%) had seizure recurrence; 10 patients (8%) were free of their preoperative seizures but had new-onset nonepileptic spells and were excluded from further analysis. Of 115, at follow-up (mean age at follow-up 12.7 years, range 2-28 years), 96 patients (83%) walked independently, 10 (8.7%) walked with assistance, and 9 (7.8%) were unable to walk. New visual symptoms that were not present preoperatively were reported only in 28 patients (24%). Eighty patients (70%) had satisfactory spoken language skills but only 44 (42%) of the 105 children older than 6 years had satisfactory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 children aged between 6 and 18 years attended mainstream school without assistance; 48 (59%) were in mainstream school with assistance and the rest were in special school for disabled or home cared. Five (21%) of the 24 patients older than 18 years of age were gainfully employed. Multivariate logistic regression analysis identified the following factors as independently associated with poor functional outcome. (1) Seizure recurrence negatively affected all functional domains--ambulation ability, spoken language and reading skills, and behavior (p < 0.05). (2) Abnormalities in the unoperated hemisphere on magnetic resonance imaging (MRI) (p < 0.05) and preexisting quadriparesis (p < 0.01) correlated with poor motor outcome. (3) Multilobar MRI abnormalities in the contralateral hemisphere (odds ratio [OR] = 13.9, p = 0.001) and young age (indeterminate preoperative language status) at hemispherectomy (OR = 11.1, p = 0.01) also correlated with poor language outcome. (4) Younger age at epilepsy onset correlated with poor reading skills (p = 0.01) but not with spoken language skills. SIGNIFICANCE: This study highlights the long-term functional status of patients after hemispherectomy. The majority of patients were ambulant independently; however, impairments in reading and spoken language were frequent. Seizure recurrence after hemispherectomy and contralateral hemisphere abnormalities on MRI were the major predictors of poor outcome in ambulation, spoken language, and reading abilities. This study will assist in presurgical counseling using simple understandable functional outcome measures and may help in planning early interventions after hemispherectomy to improve functional outcome.

Longitudinal seizure outcome and prognostic predictors after hemispherectomy in 170 children.

OBJECTIVE: Data on longitudinal seizure outcome after hemispherectomy in children are limited. This study explores the posthemispherectomy longitudinal seizure outcome and its predictors. METHODS: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center. Clinical, EEG, imaging, and surgical data were collected. Seizure outcome data were collected via a structured questionnaire by contacting families (n = 125) or from the medical records at last follow-up (n = 58). RESULTS: Of 186 patients, 3 were lost to follow-up; 13 seizure-free patients with new-onset nonepileptic spells were excluded. Perioperative complications were not collected. There was no mortality. At a mean follow-up of 5.3 years (±3.3 years), 112 of 170 children (66%) were seizure-free (Engel class 1a). In 58 patients with seizure recurrence, 8 had late remission and 16 had >90% reduction. Overall, at last follow-up, 136 patients (80%) were either seizure-free or had major improvement. Using survival analysis, the estimated probability of seizure freedom after hemispherectomy was 78% (95% confidence interval [CI] = 75%-81%) at 6 months, 76% (95% CI = 73%-79%) at 1 year, 71% (95% CI = 68%-74%) at 2 years, and 63% (95% CI = 59%-67%) at 5 years. On multivariate analysis, bilateral PET abnormalities (risk ratio = 2.53, 95% CI = 1.02-5.85) and acute postoperative seizures (risk ratio = 7.03, 95% CI = 3.07-15.9) independently predicted seizure recurrence. CONCLUSIONS: The long-term seizure-free rates after hemispherectomy remained stable at 63% at 5 years and beyond. This study will assist in better candidate selection for hemispherectomy, presurgical counseling, and early identification of surgical failures.

Reoperative hemispherectomy for intractable epilepsy: a report of 36 patients.

BACKGROUND: In patients with medically intractable epilepsy and diffuse unilateral hemispheric disease, functional or disconnective hemispherectomy is a widely accepted and successful treatment option. If recurrent seizures develop after disconnective hemispherectomy, management options become more complex and include conversion to anatomic hemispherectomy. OBJECTIVE: To present the outcomes of all patients undergoing reoperative hemispherectomy in 1 institution by 1 surgeon since 1998. METHODS: The medical records, operative reports, and imaging studies for 36 patients undergoing reoperative hemispherectomy for continuing medically intractable epilepsy from 1998 to 2011 at Cleveland Clinic were reviewed. Patient characteristics, cause of seizure, imaging findings, surgery-related complications, and long-term seizure outcomes were evaluated. RESULTS: Patients presented with a variety of seizure origins, including Rasmussen encephalitis, perinatal infarction, cortical dysplasia, and hemimegalencephaly. Overall, 19% of patients were seizure free after conversion to anatomic hemispherectomy, and 45% reported a decrease in seizure frequency by ≥ 90%. An additional 36% reported no improvement. Generalized ictal electroencephalography tended to confer a poorer prognosis, as did cortical dysplasia as the underlying diagnosis. CONCLUSION: The possibility that residual epileptogenic tissue in the operated hemisphere remains connected should be considered after failed functional hemispherectomy because our data suggest that improvement in seizure frequency is possible after reoperative hemispherectomy, although the chance of obtaining seizure freedom is relatively low. The decision to proceed with reoperative hemispherectomy should be made after proper discussion with the patient and family and informed consent is given.

Outcome of no resection after long-term subdural electroencephalography evaluation in children with epilepsy.

OBJECT: The aim of this study was to identify the reasons for and predictors of no resection of the epileptogenic zone in children with epilepsy who had undergone long-term invasive subdural grid electroencephalography (SDG-EEG) evaluation. METHODS: The authors retrospectively reviewed the consecutive medical records of children (< 19 years of age) who had undergone SDG-EEG evaluation over a 7-year period (1997-2004). To determine the predictors of no resection, the authors obtained the clinical characteristics and imaging and EEG findings of children who had no resection after long-term invasive SDG-EEG evaluation and compared these data with those in a group of children who did undergo resection. They describe the indications for SDG-EEG evaluation and the reasons for no resection in these patients. RESULTS: Of 66 children who underwent SDG-EEG evaluation, 9 (13.6%) did not undergo subsequent resection (no-resection group; 6 males). Of these 9 patients, 6 (66.7%) had normal neurological examinations and 5 (55.6%) had normal findings on brain MR imaging. Scalp video EEG localized epilepsy to the left hemisphere in 6 of the 9 patients and to the right hemisphere in 2; it was nonlocalizable in 1 of the 9 patients. Indications for SDG-EEG in the no-resection group were ictal onset zone (IOZ) localization (9 of 9 patients), motor cortex localization (5 of 9 patients), and language area localization (4 of 9 patients). Reasons for no resection after SDG-EEG evaluation were the lack of a well-defined IOZ in 5 of 9 patients (4 multifocal IOZs and 1 nonlocalizable IOZ) and anticipated new permanent postoperative neurological deficits in 7 of 9 patients (3 motor, 2 language, and 2 motor and language deficits). Comparison with the resection group (57 patients) demonstrated that postictal Todd paralysis in the dominant hand was the only variable seen more commonly (χ(2) = 4.781, p = 0.029) in the no-resection group (2 [22.2%] of 9 vs 2 [3.5%] of 57 patients). The no-resection group had a larger number of SDG electrode contacts (mean 126. 5 ± 26.98) as compared with the resection group (100.56 ± 25.52; p = 0.010). There were no significant differences in the demographic data, seizure characteristics, scalp and invasive EEG findings, and imaging variables between the resection and no-resection groups. CONCLUSIONS: Children who did not undergo resection of the epileptogenic zone after SDG-EEG evaluation were likely to have normal neurological examinations without preexisting neurological deficits, a high probability of a new unacceptable permanent neurological deficit following resection, or multifocal or nonlocalizable IOZs. In comparison with the group that underwent resection after SDG-EEG, a history of Todd paralysis in the dominant hand and arm was the only predictor of no resection following SDG-EEG evaluation. Data in this study will help to better select pediatric patients for SDG-EEG and to counsel families prior to epilepsy surgery.

Epilepsy surgery outcome in coexisting symptomatic refractory focal epilepsy and benign focal epilepsy of childhood.

Epilepsy surgery may successfully treat refractory symptomatic focal epilepsy in patients with coexisting benign focal epileptiform discharges. Reported here is the outcome after resective epilepsy surgery in three children with pharmacoresistant lesional focal epilepsy in whom seizures of benign focal epilepsy of childhood had been recorded. Two patients had left temporal epilepsy due to a malformation of cortical development; one of these had dual pathology, with additional ipsilateral hippocampal sclerosis. One child had catastrophic left hemispheric epilepsy due to left hemimegalencephaly. Frequent, habitual seizures of symptomatic epilepsy resolved after surgery (follow-up duration, 32-55 months); however, rare benign focal seizures of childhood have continued. These cases demonstrate that lesional pharmacoresistant focal epilepsy can be successfully treated with resective epilepsy surgery even when coexisting with benign focal epilepsy of childhood. During postoperative follow-up, careful documentation of breakthrough seizures due to benign focal epilepsy of childhood is important, so that these patients are not labeled as surgical failures.

Surgery for catastrophic epilepsy in infants 6 months of age and younger.

OBJECT: Few data are available concerning efficacy and safety of surgery for catastrophic epilepsy in the first 6 months of life. METHODS: The authors retrospectively analyzed epilepsy surgeries in 15 infants ranging in age from 1.5 to 6 months (median 4 months) and weight from 4 to 10 kg (median 7 kg) who underwent anatomical (4 patients) or functional (7 patients) hemispherectomy, or frontal (1 patient), frontoparietal (2 patients), or parietooccipital (1 patient) resection for life-threatening catastrophic epilepsy due to malformation of cortical development. RESULTS: No patient died. Intraoperative complications included an acute ischemic infarction with hemiparesis in our youngest, smallest infant. The most frequent complication was blood loss requiring transfusion, which was encountered in every case. The estimated blood loss was 3-214% (median 63%) of the total blood volume. At maximum follow-up of 6-121 months (median 60 months), 46% were seizure free. CONCLUSIONS: Epilepsy surgery may be effective in young infants as it is in older children. However, intraoperative blood loss and risk of permanent postoperative neurological deficits present significant challenges.

Contralateral MRI abnormalities in candidates for hemispherectomy for refractory epilepsy.

PURPOSE: To assess the impact of contralateral magnetic resonance imaging (MRI) findings on seizure outcome after hemispherectomy for refractory epilepsy. METHODS: We retrospectively reviewed 110 children, 0.4-18 (median 5.9) years of age, who underwent hemispherectomy for severe refractory epilepsy at Cleveland Clinic Children's Hospital. In children with contralateral (as well as ipsilateral) MRI findings appreciated preoperatively, the decision to proceed to surgery was based on other features concordant with the side with the most severe MRI abnormality, including ipsilateral epileptiform discharges, lateralizing seizure semiology, and side of hemiparesis. RESULTS: We retrospectively observed contralateral MRI abnormalities (predominantly small hemisphere, white matter loss or abnormal signal, or sulcation abnormalities) in 81 patients (74%), including 31 of 43 (72%) with malformations of cortical development (MCD), 31 of 42 (73%) with perinatal injury from infarction or hypoxia, and 15 of 25 (60%) with Rasmussen's encephalitis, Sturge-Weber syndrome, or posttraumatic encephalomalacia. Among 84 children (76%) with lesions that were congenital or acquired pre- or perinatally, 67 (83%) had contralateral MRI abnormalities (p = 0.02). Contralateral findings were subjectively judged to be mild or moderate in 70 (86%). At follow-up 12-84 (median 24) months after surgery, 79% of patients with contralateral MRI abnormalities were seizure-free compared to 83% of patients without contralateral MRI findings, with no differences based on etiology group or type or severity of contralateral MRI abnormality. DISCUSSION: MRI abnormalities, usually mild to moderate in severity, were seen in the contralateral hemisphere in the majority of children who underwent hemispherectomy for refractory epilepsy due to various etiologies, especially those that were congenital or early acquired. The contralateral MRI findings, always much less prominent than those in the ipsilateral hemisphere, did not correlate with seizure outcome and may not contraindicate hemispherectomy in otherwise favorable candidates.