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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., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Purpose: Despite advances in "noninvasive" localization techniques, many patients with medically intractable epilepsy require the placement of subdural (subdural grid electrode, SDE) and/or depth electrodes for the identification and definition of extent of the epileptic region. This study investigates the trends in longitudinal seizure outcome and its predictors in this group., Methods: We reviewed the medical records, and electroencephalography (EEG) data of 414 consecutive patients who underwent intracranial electrode placement (SDE and/or depth electrodes) at Cleveland Clinic Epilepsy Center between 1998 and 2008. A favorable outcome was defined as complete seizure freedom, discounting any auras or seizures that occurred within the first postoperative week. Survival curves were constructed, and Cox proportional hazard modeling was used to identify outcome predictors., Key Findings: The estimated probability of complete seizure freedom was 61% (95% confidence interval [CI] 58-64%) at one postoperative year, 47% (95% CI 44-50%) at 3 years, 42% (95% CI 39-45%) at 5 years, and 33% (95% CI 28-38%) at 10 years. Half of all seizure recurrences occurred within the first two postoperative months. Subsequently, the rate of seizure freedom declined by 4-5% every 2-3 years. After multivariate analysis, two independent predictors of seizure recurrence were identified: (1) prior resective surgery (p ≤ 0.002), mostly in patients with temporal lobe resections, and (2) sublobar or multilobar resection (p ≤ 0.02), mostly in patients following frontal lobe resections., Significance: Favorable seizure outcomes are possible in the complex epilepsy population requiring invasive EEG studies. We propose that mislocalization of the epileptogenic zone or its incomplete resection account for early postoperative recurrences, whereas epileptogenesis may lead to later relapses., (Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.)

Purpose: Outcomes following unilobar surgeries for refractory epilepsy have been well described. However, little is known about long-term seizure outcomes following multilobar resections. The aim of the current study was to identify long-term seizure control and predictors of seizure recurrence in this patient population., Methods: Records of patients who underwent multilobar epilepsy surgery at the Cleveland Clinic between 1994 and 2010 were retrospectively reviewed. A postoperative follow-up of at least 6 months was required. Patients were classified as seizure free if they achieved an Engel class I at last follow-up. Long-term chances of seizure freedom were illustrated using a survival analysis, and predictors of recurrence were identified using Cox proportional hazard modeling., Key Findings: Sixty-three patients with medically intractable epilepsy underwent multilobar surgical resections during the study period (mean follow-up of 4.6 years). Predominant resection types included extended occipital (temporoparietooccipital, parietooccipital, temporooccipital: 57%), frontotemporal (21%), and temporoparietal (17%). Mean age at surgery was 21.4 years and mean age at seizure onset was 10.1 years. Fifty-six percent of the patients underwent extraoperative invasive electroencephalography (EEG) evaluations. At 6 postoperative months, 71% (95% confidence interval (CI) 65-77) were seizure-free (SF), 64% (CI 58-70) were SF at 1 year, 52% (CI 46-59) were SF at 5 years, and 41% (CI 32-50) remained SF at 10 years. Forty-one patients had at least one breakthrough seizure after surgery (median timing of recurrence 6.1 months), with an Engel class 1 achieved again by last follow-up in 12 of these 41 cases. Nine patients required a reoperation. Patients who underwent extended occipital/posterior quadrant resections had more favorable outcomes as compared to the other groups. With multivariate analysis, the type of resection (p = 0.03), preoperative auras (p = 0.03), an incomplete resection (0.03), and the presence of postoperative spikes (p = 0.0003) correlated with seizure recurrence. The risk of seizure recurrence for an incomplete resection was 2.3 (CI 1.53-3.36), preoperative aura 2.3 (CI 1.34-3.87), and postoperative spikes on surface EEG 2.5 (CI 1.29-4.71)., Significance: A favorable outcome can be achieved in 41% of patients undergoing multilobar resections for epilepsy surgery at 10 years of follow-up. Close to one-third of patients who have breakthrough seizures after surgery are able to regain seizure freedom by last follow-up. Predictors of recurrence include resection type (frontotemporal and parietotemporal resections did worse), presence of preoperative aura, an incomplete surgical resection, and the presence of postoperative interictal discharges on EEG., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: Up to one-half of epilepsy surgery patients will have at least one seizure after surgery. We aim to characterize the prognosis following a first postoperative seizure, and provide criteria allowing early identification of recurrent refractory epilepsy., Methods: Analyzing 915 epilepsy surgery patients operated on between 1990 and 2007, we studied 276 who had >or=1 seizure beyond the immediate postoperative period. The probability of subsequent seizures was calculated using survival analysis. Patients were divided into seizure-free (no seizures for >or=1 year) and refractory (persistent seizures) and analyzed using multivariate regression analysis., Results: After a first seizure, 50% had a recurrence within 1 month and 77% within a year before the risk slowed down to additional 2-3% increments every two subsequent years. After a second seizure, 50% had a recurrence within 2 weeks, 78% within 2 months, and 83% within 6 months. Having both the first and second seizures within six postoperative months [odds ratio (OR) 4.04; 95% confidence interval (CI) 2.05-8.40; p = 0.0001], an unprovoked initial recurrence (OR 3.92; 95% CI 2.13-7.30; p < 0.0001), and ipsilateral spikes on a 6-months postoperative electroencephalography (EEG) (OR 2.05; 95% CI 1.10-3.88; p = 0.025) predicted a poorer outcome, with 95% of patients who had all three risk factors becoming refractory. All patients with cryptogenic epilepsy and recurrent seizures developed refractoriness., Discussion: Seizures will recur in most patients who present with their first postoperative event, with one-third eventually regaining seizure-freedom. Etiology and early and unprovoked postoperative seizures with epileptiform activity on EEG at six postoperative months may predict recurrent medical refractoriness.

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.

Purpose: A few reports have described focal electroencephalographic or clinical features or both of juvenile myoclonic epilepsy (JME), but without video-EEG documentation. We examined focal clinical and EEG features in patients with JME who underwent video-EEG monitoring., Methods: Twenty-six patients (nine males and 17 females) who had seizures recorded during video-EEG monitoring were included. Age at seizure onset was 0 to 22 years (mean, 12.3 years), and age at monitoring was 12 to 44 years (mean, 26.5 years). In one patient with left parietooccipital epilepsy, primary generalized tonic-clonic seizures developed after resection of the parietal tumor. Two patients had both temporal lobe epilepsy and JME. Videotaped seizures in each patient were analyzed. Interictal and ictal EEG also were analyzed for any focal features., Results: Focal semiologic features were observed in 12 (46%) of 26 patients. Six patients had focal myoclonic seizures, and two had Figure 4 sign: one with version to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs were generalized at onset but with a lateralized evolution over the right hemisphere. The patient who had both JME and left parietooccipital epilepsy, right arm clonic seizure, and Figure 4 sign was seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves, and another had run of spikes in the right frontal region., Conclusions: Fourteen (54%) of 26 patients with JME exhibited focal semiologic or electroencephalographic features or both. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate antiepileptic drug regimen.

Purpose: To report our experience with hemispherectomy in the treatment of catastrophic epilepsy in children younger than 2 years., Methods: In a single-surgeon series, we performed a retrospective analysis of 18 patients with refractory epilepsy undergoing hemispherectomy (22 procedures). Three different surgical techniques were performed: anatomic hemispherectomy, functional hemispherectomy, and modified anatomic hemispherectomy. Pre- and postoperative evaluations included extensive video-EEG monitoring, magnetic resonance imaging, and positron emission tomography scanning. Seizure outcome was correlated with possible variables associated with persistent postoperative seizures. The Generalized Estimation Equation (GEE) and the Barnard's exact test were used as statistical methods., Results: The follow-up was 12-74 months (mean, 34.8 months). Mean weight was 9.3 kg (6-12.3 kg). The population age was 3-22 months (mean, 11.7 months). Thirteen (66%) patients were seizure free, and four patients had >90% reduction of the seizure frequency and intensity. The overall complication rate was 16.7%. No deaths occurred. Twelve (54.5%) of 22 procedures resulted in incomplete disconnection, evidenced on postoperative images. Type of surgical procedure, diagnosis categories, persistence of insular cortex, and bilateral interictal epileptiform activity were not associated with persistent seizures after surgery. Incomplete disconnection was the only variable statistically associated with persistent seizures after surgery (p<0.05)., Conclusions: Hemispherectomy for seizure control provides excellent and dramatic results with a satisfactory complication rate. Our results support the concept that early surgery should be indicated in highly selected patients with catastrophic epilepsy. Safety factors such as an expert team in the pediatric intensive care unit, neuroanesthesia, and a pediatric epilepsy surgeon familiar with the procedure are mandatory.

Purpose: To identify clinical and EEG correlates of ictal spitting automatisms and to assess their reliability in indicating the hemisphere of seizure onset., Methods: The epilepsy-monitoring database (1994-2002) of the Cleveland Clinic Foundation (CCF) was searched for patients with a definite history of ictal spitting. All available documents of the patients, particularly their original video and EEG data, were reviewed., Results: Twelve (0.3%) of the approximately 4000 patients had a documented history of ictal spitting. In seven of them, 15 seizures with spitting automatisms were recorded. All of them started with an aura or arousal out of sleep. In six of the seven patients (12 of 15 seizures), EEG onset was clearly lateralized to the right, nondominant hemisphere. Spitting occurred at a median time of 21 s after EEG seizure onset. At that time, predominantly fast, high-amplitude theta (5-7 Hz) was seen in the hemisphere of seizure onset, maximum temporal. In all but one of the total 12 patients, the epileptogenic zone was in the temporal lobe. In nine of the 12 patients, seizure onset was in the non-language-dominant hemisphere. Two patients had seizures arising from the language-dominant hemisphere; in another patient, the side of the seizure onset could not be determined., Conclusions: Ictal spitting is an uncommon feature of epileptic seizures. Although the symptomatogenic area is probably outside the temporal lobe, it is most frequently seen in temporal lobe epilepsy of the right, nondominant hemisphere.

Purpose: Hypomotor seizures (characterized by diminished behavioral activity with indeterminate level of consciousness) have been identified as an important seizure type in infants. Our goal was to investigate further the clinical and EEG features of hypomotor seizures., Methods: We retrospectively reviewed 110 hypomotor seizures from 34 patients recorded with video-EEG., Results: Twenty-seven (79%) patients were younger than 48 months, and seven (21%) were aged 4 to 15 years. Seventy-one (64%) seizures had regional or lateralized EEG onset, arising predominantly from temporal or parietal lobe regions. The other 39 (35%) seizures had generalized onset, usually with abrupt onset of diffuse rhythmic high-amplitude theta activity or diffuse electrodecrement and only rarely (two patients) with slow spike-wave complexes or 3-Hz spike-wave complexes. Hypomotor seizures with generalized EEG onset were significantly shorter than those with regional or lateralized onset (p = 0.01, GEE model). Unsustained head or eye movements and subtle mouth automatisms were commonly seen in hypomotor seizures with either focal or generalized onset. Seventeen percent of hypomotor seizures with focal onset evolved to include version of head and eyes or jerking of one arm, whereas 2% of generalized hypomotor seizures evolved to a cluster of spasms., Conclusions: Hypomotor seizures may be either focal or generalized. Regional EEG onsets were most often temporal or parietal, suggesting that focal hypomotor seizures may be a bland form of "complex partial" seizures with no or minimal automatisms, seen predominantly in infants. Generalized hypomotor seizures were rarely associated with an ictal pattern of generalized spike-wave complexes, suggesting a different mechanism from absence seizures seen later in life.

Purpose: Focal cortical dysplasia (CD) is increasingly recognized as a common pathologic substrate of medically intractable epilepsy. As these lesions are often localized in the frontal lobe (therefore in potentially eloquent cortex), an understanding of the functional status of the involved region(s) and of its anatomic and pathologic correlates is of prime importance. The purpose of this study is to assess the function of focal CD in relation to magnetic resonance imaging (MRI) and histopathologic features., Methods: Eight patients operated on for medically intractable epilepsy with histologically proven focal CD involving putative eloquent cortex in the frontal lobe (perirolandic and Broca's areas) were included in the study. Functional regions (motor and language) and epileptogenic areas were assessed by extraoperative electrocorticographic recording and electrical cortical mapping. Cortical functions were correlated with the extent of epileptogenicity on electrocorticographic recordings, MRI features, and histologic characteristics., Results: Language or motor areas were colocalized with epileptogenic regions (n=6 of 8, 75%), but were not mapped in regions of increased signal on fluid-attenuated inversion recovery (FLAIR) MRI (when they were identified) on preoperative MRI (n=5 of 5, 100%). Histologically, balloon cells were almost exclusively found in nonfunctional regions with FLAIR MRI abnormalities. When resected, regions of motor cortex were characterized by cortical dyslamination, columnar disorganization, and dysmorphic neurons, but were devoid of balloon cells., Conclusions: We found an absence of language or motor functions in perirolandic and Broca's areas that showed decreased epileptogenicity, histopathological evidence of CD with balloon cells and FLAIR MRI signal increase. Language and motor functions were present in epileptogenic and dysplastic areas with no balloon cells and no FLAIR signal abnormalities. These findings have implications on options for epilepsy surgery in patients with CD.

Purpose: We sought to determine whether early age at seizure onset is a risk factor for mental retardation, independent of etiology. Assessment of risk for mental retardation with continued uncontrolled seizures plays a role in considerations of timing for epilepsy surgery. Previous studies have indicated that onset of seizures in the first years of life may be a risk factor for mental retardation, but the etiologies of the epilepsies were not included in the analyses., Methods: Intellectual function was assessed at ages 2-20 years during presurgical evaluation in 100 patients with intractable epilepsy due to focal lesions limited to part of one lobe of the brain. Mental retardation (MR) was defined as Full-Scale Intelligence Quotient (FSIQ) < or =70. The age at seizure onset and the seizure frequency were obtained retrospectively., Results: Younger ages at seizure onset were associated with lower FSIQ scores, and mean FSIQ was also significantly lower for patients with onset of epilepsy at < or =24 months of age (74.0 +/- 21.5) versus that in patients with onset of epilepsy later in life (87.8 +/- 18.8; p = 0.005). The frequency of patients with MR was significantly higher for patients with seizure onset at < or =24 months of age (15 of 33, 46%) than for patients with seizure onset later in life (eight of 67, 12%; p < 0.001). This difference persisted within etiologic subgroups. For patients with focal malformation of cortical development, MR was seen in eight (50%) of 16 patients with seizure onset at < or =24 months versus two (10%) of 20 patients with seizure onset at >24 months (p < 0.001); for patients with tumor, MR was seen in four (50%) of eight patients with seizure onset at < or =24 months versus four (13%) of 30 patients with seizure onset at >24 months (p = 0.003); and for patients with hippocampal sclerosis, MR was seen in two (28%) of seven patients with seizure onset at < or =24 months versus none of 30 patients with seizure onset at >24 months (NS). Within the subgroup with daily seizures, MR was present in 13 (65%) of 20 patients with seizure onset at < or =24 months versus five (17%) of 29 patients with seizure onset later in life (p = 0.001)., Conclusions: These results indicate that onset of intractable epilepsy within the first 24 months of life is a significant risk factor for MR, especially if seizures occur daily. The risk based on early age at seizure onset appeared independent of etiology and persisted within subgroups of patients with focal malformation of cortical development, tumor, or hippocampal sclerosis. Prospective studies will be important to clarify whether early surgical intervention may reduce the risk for subsequent MR in carefully selected infants.

Purpose: Human cortical dysplasia (CD) is a frequent cause of medically intractable focal epilepsy. The neurotransmitter mechanisms of epileptogenicity in these lesions have been attributed to changes in various glutamate receptor subtypes. Increased N-methyl-D-aspartate (NMDA) receptor (NR) 2A/B coassembled with NR1 subunits has been shown in focal epileptic CD. The purpose of this study is to correlate in situ CD epileptogenicity and the expression of various glutamate receptor subtypes., Methods: The histopathological, morphological, and immunocytochemical findings in cortical tissue resected from five patients with medically intractable epilepsy and CD were correlated with electroencephalographic data recorded from subdural grids. The NMDA antibodies identified subunits NR1 (splicing variants 1a, 1b, 2a, and 2b) and NR2A/B., Results: Epileptogenic specimens displayed the following common features: (a) widespread histological abnormalities of horizontal and columnar dyslamination, neurons with inverted polarity, and more extensive dendritic changes; (b) significantly higher NR2A/B immunoreactivity in both the dysplastic somata and all their dendritic processes; and (c) no statistically significant change in NR1 subunit expression but a more pronounced staining of the apical dendrites in highly epileptogenic cortex. These abnormalities were either absent or minimal in resected specimens that did not show evidence of severe in vivo epileptogenicity., Conclusion: These studies provide direct evidence for a major contribution of the NR2A/B subunit in CD-induced epileptogenicity.

Purpose: Identification of risk factors for unsuccessful testing during intracarotid amobarbital procedure (IAP) in preadolescent children., Methods: A pediatric IAP protocol was attempted in 42 candidates for epilepsy surgery (5-12 years old; mean, 10 years) based on the ability to pass a practice test. Language dominance was defined by marked asymmetry until first verbal response and paraphasic errors. Intact hemispheric memory was defined by recall of >/=60% of test items. The odds ratios of baseline variables (age at IAP, Full-Scale IQ, side of disease, age at seizure onset, amobarbital dose) were calculated for various IAP outcomes., Results: IAPs were accomplished in 40 children. Language dominance was established in 25 (62.5%) of 40 patients: all 21 focal resection candidates were left language dominant; four hemispherectomy candidates had intact language after injection of the damaged hemisphere. In 12 (30%) of 40 patients, language testing failed because of agitation or obtundation. Compared with the 21 children with language dominance established by bilateral IAP, these 12 children had lower mean Full-Scale IQ (66.4 vs. 87.9; p = 0.014), and more frequently, the epileptogenic lesion in the left hemisphere (presumed dominant by right-handedness; 78 vs. 33%; p = 0.04). Excluding hemispherectomy candidates (intentionally only one injection), memory testing could not be completed in 13 (36%) of 36 children because of obtundation or agitation. These children were significantly younger than the 23 (64%) of 36 with successful bilateral memory testing (mean age, 107.6 vs. 128.7 months; p = 0. 006). The eight (25%) of 32 children with failing retention scores after ipsilateral injection had lower Full-Scale IQ than did the 24 (75%) patients who passed (mean, 59.6 vs. 81.7 months; p = 0.03)., Conclusions: The IAP successfully established hemispheric language dominance and memory representation for just under two thirds of the preselected preadolescent children. Risk factors for unsuccessful testing included low Full-Scale IQ (especially <80), young age (especially <10 years), and seizures arising from the left hemisphere presumed dominant by right-handedness.

Purpose: Numerous studies have demonstrated changes in cognitive, memory, and language functioning in adults and adolescents after temporal lobectomy, yet little information is available regarding neuropsychological outcome in preadolescent children., Methods: We studied pre- and postoperative neuropsychological test results from 14 children who underwent temporal lobe resection for intractable epilepsy at age 7-12 years (mean 9.4 years)., Results: Thirteen patients (93%) had no seizures or less than one seizure a year at follow-up 23-48 months (mean 34 months) after operation. Postoperative neuropsychological testing was performed 6-9 months (mean 7 months) after surgery in 13 patients and 36 months after the first operation in 1 patient who underwent two-stage resection of a tumor. Verbal, Performance, and Full Scale IQ were initially in the low-average range, with no significant change across the pre- and postoperative evaluations. Immediate verbal memory performance decreased significantly in children who initially performed above the median preoperatively and tended to decrease in children who had left rather than right temporal lobe resection. Significant postoperative decreases in delayed memory scores were independent of preoperative ability or side of resection., Conclusions: Our small study suggests vulnerability to postoperative decline in immediate verbal memory scores in preadolescent children who have higher baseline immediate memory function or undergo left rather than right temporal lobe resection, similar to that observed in adolescents in adults. The entire group exhibited a statistically significant decrease in delayed verbal memory. Study of larger series of patients will be important to clarify further the short- and long-term risks and benefits of temporal lobe resection in childhood.

On the basis of cytoarchitectural and functional studies, the frontal lobe can be subdivided into the primary motor cortex, premotor cortex, prefrontal cortex, and the limbic and paralimbic cortices. However, we are still a long way from clearly identifying individual frontal lobe epilepsies. Instead, we are limited to a discussion of frontal lobe seizures arising from various regions of the frontal lobe. Supplementary motor area epilepsy and perirolandic epilepsy have been quite well defined, in contrast to syndromes involving other regions of the frontal lobe. Recent technological advances in neuroimaging, electroencephalography, magnetoencephalography and detailed videotape analysis of seizure semiology may enable us to delineate these frontal lobe syndromes with better accuracy, thereby improving outcome after epilepsy surgery.

Purpose: Unilateral dystonic limb posturing in partial seizures has been shown to be an accurate lateralizing sign indicating seizure onset in the contralateral hemisphere. However, its clinical utility may be reduced by confusion with other lateralized ictal motor phenomena. In this study, the ictal phenomena of dystonic limb posturing, tonic limb posturing, unilateral immobile limb, and version were distinguished and examined in patients with temporal and extratemporal seizures., Methods: Partial seizures in 54 patients, successfully treated by surgery (34 temporal, 20 extratemporal; 14 frontal, 3 parietal, and 3 occipital), were analyzed blindly by 3 reviewers. Interobserver agreement was tested with kappa indexes and positive predictive value (PPV) was determined for each sign., Results: In patients with temporal lobe epilepsy (TLE), dystonic posturing occurred in 35.3% (kappa 0.78, positive predictive value (PPV) for the sign being contralateral to seizure onset 92%); tonic limb posturing occurred in 17.7% (kappa 0.36, PPV 40%); unilateral immobile limb occurred in 11.8% (kappa 0.23, PPV 100%); and version occurred in 35.3% (kappa 0.77, PPV 100%). In patients with extratemporal epilepsy, dystonic posturing occurred in 20.0% (kappa 0.31, PPV 100%); tonic limb posturing occurred in 15.0% (kappa 0.08, PPV 67%); and version occurred in 40.0% (kappa 0.54, PPV 100%). The higher kappa indexes were significant for dystonic posturing (p < 0.001) and tonic limb posturing (p = 0.032) in TLE. Dystonic posturing (p = 0.034), tonic posturing (p = 0.07), and version (p = 0.0038) occurred earlier in extratemporal seizures than in temporal seizures., Conclusions: Of the limb ictal motor phenomena, only dystonic posturing was accurate and had good interobserver agreement.

Purpose: We wished to evaluate functional outcome and parental satisfaction after corpus callosotomy., Methods: We studied a cohort of parents of 17 patients who had received an anterior corpus callosotomy > 7 months earlier (mean 26 months) for severe, intractable seizures. Parental satisfaction with the overall surgical results was the primary outcome measure. In addition, the parents assessed behavior changes that had been emphasized preoperatively as important aspects of their children's function, including alertness and responsiveness, falls and injuries, and special care needs. Degree of seizure reduction and extent of corpus callosum were also measured., Results: The parents of 15 (88%) patients reported satisfaction with the surgical outcome. All parents who expressed satisfaction would recommend callosotomy to another family in a similar situation. The behavior change most closely associated with satisfaction was improved alertness and responsiveness (p < 0.03). Although 9 patients experienced > 80% reduction in targeted seizures, satisfaction was not reported at a greater rate as compared with the 6 patients with 50-80% reduction., Conclusions: Most parents report satisfaction with the outcome of their child's corpus callosotomy, but are influenced by improvement in aspects of function and behavior in addition to seizure reduction.

Purpose: We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5-29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5-24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with nuclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia)., Methods: Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue., Results: At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked "catch-up" developmental progress after class I, II, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia., Conclusions: Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.

To explore the electroclinical features of temporal lobe epilepsy (TLE) in early childhood, we studied results of video-EEG and other tests of 14 children aged 16 months to 12 years selected by seizure-free outcome after temporal lobectomy. Four children had mesiotemporal sclerosis, 1 had cortical dysplasia, and 9 had low-grade temporal neoplasms. The children had complex partial seizures (CPS) with symptomatology similar to that of adults with TLE, including decreased responsiveness and automatisms. Automatisms tended to be simpler in the younger children, typically limited to lip smacking and fumbling hand gestures. Scalp/sphenoidal EEG showed anterior/inferior temporal interictal sharp waves and unilateral temporal seizure onset in the 4 children with mesiotemporal sclerosis and in the child with cortical dysplasia, but EEG findings in 9 children with low-grade temporal tumors were complex, including multifocal interictal sharp waves or poorly localized or falsely lateralized EEG seizure onset. In children without tumors, video-EEG was critical to localization of the epileptogenic zone for resection, but in patients with tumors video-EEG was less localizing and its main value was to confirm that the reported behaviors were epileptic seizures with semiology typical of temporal lobe onset.

The neurocutaneous syndromes are characterized by congenital dysplastic abnormalities involving the skin and nervous system. The commonest neurocutaneous syndromes manifesting epilepsy are tuberous sclerosis and the Sturge-Weber syndrome. Neurofibromatosis and other lesser-known entities, such as epidermal nevus syndrome, are also known to be accompanied by epilepsy. These syndromes are not related to one another. This article reviews what has been learned about the epileptic syndromes in these disorders.

Eighty-eight patients had bilateral intracarotid amobarbital (Wada) testing to determine hemispheric dominance for language in preparation for epilepsy surgery, as well as unilateral extraoperative cortical electrical stimulation using subdural electrode arrays. In none of the patients with left dominance by Wada testing were language areas found with right-sided stimulation, but two patients with right dominance by Wada testing had language areas mapped on the left side. These findings suggest that left dominance by Wada testing is strong evidence for exclusive lateralization of language function in the left hemisphere, but there is concern about the ability of the Wada test to exclude the possibility of some left-sided language function despite apparent right-sided dominance. Patients with left dominance on Wada testing do not need cortical stimulation before extensive right temporal lobectomy, but we believe that patients with right or bilateral dominance on Wada testing should have cortical stimulation for localization of language areas if extensive left or right temporal or frontal resection is planned.

In our patient population that had undergone antero-temporal lobectomy, we found 20 patients with a unilateral sphenoidal/antero-temporal interictal focus. All patients had normal computed tomography (CT) scans. Invasive recordings with subdural electrode arrays placed over and under the temporal lobe were used in every patient. We found that the scalp interictal focus predicted for all patients that both the interictal sharp waves and ictal onset would be mesiobasal/anterotemporal in location on the subdural arrays. Seventy-five percent of these patients had an excellent outcome with temporal lobectomy.