Prasanna Jayakar

Proposed criteria for referral and evaluation of children for epilepsy surgery : Recommendations of the subcommission for pediatric epilepsy surgery

Summary:  The Commission on Neurosurgery of the International League Against Epilepsy (ILAE) formed the Pediatric Epilepsy Surgery Subcommission in 1998 and charged it with formulating guidelines and recommendations for epilepsy surgery in childhood. Also endorsed by the Commission on Paediatrics, the following document is the consensus agreement after a meeting of 32 individuals from 12 countries in 2003. The panel agreed that insufficient class 1 evidence exists to recommend practice guidelines at this time. Instead, the panel generated criteria concerning the unique features of pediatric epilepsy patients to justify dedicated resources for specialty pediatric surgical centers, suggested guidelines for physicians for when to refer children with refractory epilepsy, and recommendations on presurgical evaluation and postoperative assessments. The panel also outlined areas of agreement and disagreement on which future research and consensus meetings should focus attention to generate practice guidelines and criteria for pediatric epilepsy surgery centers.

Predictors of outcome in pediatric epilepsy surgery

Objective: To determine the correlation between pre- and perioperative variables on the outcome of children undergoing focal resections for medically intractable partial epilepsy. Methods: Retrospective analysis of pre- and perioperative variables in a cohort of 75 patients younger than 12 years of age who underwent excisional surgery and had at least 1 year of follow-up. Outcome, measured by postoperative seizure frequency, was analyzed as a function of age at seizure onset, duration of epilepsy, presence of cognitive impairment, lobe of seizure origin, presence of a lesion, histopathology, and completeness of resection. Completeness of resection was defined on the basis of excising both the entire structural lesion if present and the region revealing prominent interictal and ictal abnormalities on intracranial EEG. Results: Seventy-seven percent of patients had good outcomes (class 1 or 2), and 59% were seizure-free. Lesional status, site of resection, and pathologic diagnoses were not significant predictors of outcome except for in multilobar resection, for which overall outcome was relatively poor (44% class 3 or 4; 22% seizure-free). Completeness of resection was the only significant predictor of good outcome (p < 0.001), with 92% of patients who underwent complete resection of the epileptogenic zone achieving good outcome compared with 50% of patients who had incomplete resections. Conclusion: In this series of pediatric patients, complete resection of the lesion and the electrographically abnormal region was the main determinant of outcome after focal resections. Except for multilobar resections, other factors examined in this study did not significantly influence postoperative seizure prognosis and should not influence candidate selection for the surgical process.

Incomplete resection of focal cortical dysplasia is the main predictor of poor postsurgical outcome

Background: Focal cortical dysplasia (FCD) is recognized as the major cause of focal intractable epilepsy in childhood. Various factors influencing postsurgical seizure outcome in pediatric patients with FCD have been reported. Objective: To analyze different variables in relation to seizure outcome in order to identify prognostic factors for selection of pediatric patients with FCD for epilepsy surgery. Methods: A cohort of 149 patients with histologically confirmed mild malformations of cortical development or FCD with at least 2 years of postoperative follow-up was retrospectively studied; 113 subjects had at least 5 years of postoperative follow-up. Twenty-eight clinical, EEG, MRI, neuropsychological, surgical, and histopathologic parameters were evaluated. Results: The only significant predictor of surgical success was completeness of surgical resection, defined as complete removal of the structural MRI lesion (if present) and the cortical region exhibiting prominent ictal and interictal abnormalities on intracranial EEG. Unfavorable surgical outcomes are mostly caused by overlap of dysplastic and eloquent cortical regions. There were nonsignificant trends toward better outcomes in patients with normal intelligence, after hemispherectomy and with FCD type II. Other factors such as age at seizure onset, duration of epilepsy, seizure frequency, associated pathologies including hippocampal sclerosis, extent of EEG and MRI abnormalities, as well as extent and localization of resections did not influence outcome. Twenty-five percent of patients changed Engels class of seizure outcome after the second postoperative year. Conclusions: The ability to define and fully excise the entire region of dysplastic cortex is the most powerful variable influencing outcome in pediatric patients with focal cortical dysplasia.

Different features of histopathological subtypes of pediatric focal cortical dysplasia

Focal cortical dysplasia (FCD) is the most frequent pathological finding in pediatric epilepsy surgery patients. Several histopathological types of FCD are distinguished. The aim of the study was to define distinctive features of FCD subtypes.

A Safe and Effective Paradigm to Functionally Map the Cortex in Childhood

Conventional cortical stimulation based on pulses of fixed duration briefer than the chronaxie rarely elicit responses in infants and young children. We developed a stimulation paradigm that relies on increments in both stimulus intensity and pulse duration. This approach ensures that stimulation parameters converge to the chronaxie by mathematically minimizing the energy required to elicit a response. In six patients, this paradigm successfully elicited clinical responses and/or afterdischarges at thresholds 5-8 mA below the standard paradigm and at up to 64% lower energy levels. Furthermore, three patients under age 5 years who did not respond to maximal fixed duration stimulation demonstrated afterdischarges and clinical responses when longer pulse durations were utilized. These findings indicate that a paradigm based on dual increments is effective for cortical mapping in children. Furthermore, by ensuring responses at lower energy levels, it may be more efficient for mapping the cortex at all ages.

Localization of seizure foci: pitfalls and caveats.

The pitfalls and difficulties in accurately localizing seizure foci are reviewed. Basic issues regarding modeling, volume conduction, inhomogeneities, and corticocortical propagation are discussed, and the limitations of scalp and intracranial recordings are outlined. The ambiguities in interpreting patterns and their significance are highlighted with a concluding commentary on pitfalls in defining the epileptogenic region.

Interictal spike detection using the Walsh transform

The objective of this study was to evaluate the feasibility of using the Walsh transformation to detect interictal spikes in electroencephalogram (EEG) data. Walsh operators were designed to formulate characteristics drawn from experimental observation, as provided by medical experts. The merits of the algorithm are: 1) in decorrelating the data to form an orthogonal basis and 2) simplicity of implementation. EEG recordings were obtained at a sampling frequency of 500 Hz using standard 10-20 electrode placements. Independent sets of EEG data recorded on 18 patients with focal epilepsy were used to train and test the algorithm. Twenty to thirty minutes of recordings were obtained with each subject awake, supine, and at rest. Spikes were annotated independently by two EEG experts. On evaluation, the algorithm identified 110 out of 139 spikes identified by either expert (True Positives=79%) and missed 29 spikes (False Negatives=21%). Evaluation of the algorithm revealed a Precision (Positive Predictive Value) of 85% and a Sensitivity of 79%. The encouraging preliminary results support its further development for prolonged EEG recordings in ambulatory subjects. With these results, the false detection (FD) rate is estimated at 7.2 FD per hour of continuous EEG recording.

Electrical status epilepticus during slow-wave sleep: a review.

Electrical status epilepticus during sleep (ESES) is primarily an EEG-defined syndrome in children characterized by the occurrence of continuous spike and slow waves during non-rapid-eye-movement (non-REM) sleep, the paroxysmal abnormalities being substantially less frequent during the awake state and REM sleep. Etiologically, cases can be divided into symptomatic and cryptogenic varieties. Partial motor seizures, frequently nocturnal, precede the emergence of ESES, whereas absence seizures often occur during the phase of ESES. The emergence of ESES is associated with neuropsychological regression. The characteristic electrographic pattern and epilepsy generally disappear during adolescence and are associated with an improvement in neuropsychological function. However, if the cases reported in the literature are representative, then there is a high probability of considerable residual dysfunction. A number of factors, broadly termed ascertainment biases, likely contribute to the paucity of reports from North America and the greater recognition of the syndrome in Europe and Japan. The current information on ESES is critiqued in this review.

Distinct clinicopathologic subtypes of cortical dysplasia of Taylor

Background: Two pathologic subtypes based on the presence or absence of balloon cells have been described in cortical dysplasia of Taylor (CDT). Objective: To determine whether the pathologic subtype has any distinct clinical or MRI features that are relevant to management. Methods: The histopathologic, clinical, and MRI features of 34 children with CDT who underwent epilepsy surgery at Miami Children’s Hospital from 1990 to 2001 were investigated. Results: Bizarre neuronal cytomegaly was the primary pathologic feature of 15 patients with the dysplasia-only subtype, and 19 cases showed additional characteristics including balloon cells and marked white matter abnormalities. Both groups presented with severe intractable epilepsy of very-early-onset, multiple daily seizures, cognitive disability, and focal neurologic deficits. The dysplasia-only subtype had higher rates of neonatal onset, hemiparesis, and severe mental retardation (p < 0.05). The MRI features of focal cortical thickening with associated cortical T2 signal change showed excellent sensitivity (94%) and reasonable specificity (73%) for the diagnosis of the balloon cell subtype. The overall surgical outcome was 59% seizure freedom at 2 years. Conclusions: Children with cortical dysplasia of Taylor type have in common a very-early-onset, severe epilepsy with neurologic co-morbidity. Patients with the non–balloon cell pathologic subtype have a more severe phenotype. A trend toward a better outcome in the balloon cell group suggests that preoperative identification of these subtypes may impact surgical planning.

Detection of interictal spikes and artifactual data through orthogonal transformations.

This study introduces an integrated algorithm based on the Walsh transform to detect interictal spikes and artifactual data in epileptic patients using recorded EEG data. The algorithm proposes a unique mathematical use of Walsh-transformed EEG signals to identify those criteria that best define the morphologic characteristics of interictal spikes. EEG recordings were accomplished using the 10–20 system interfaced with the Electrical Source Imaging System with 256 channels (ESI-256) for enhanced preprocessing and on-line monitoring and visualization. The merits of the algorithm are: (1) its computational simplicity; (2) its integrated design that identifies and localizes interictal spikes while automatically removing or discarding the presence of different artifacts such as electromyography, electrocardiography, and eye blinks; and (3) its potential implication to other types of EEG analysis, given the mathematical basis of this algorithm, which can be patterned or generalized to other brain dysfunctions. The mathematics that were applied here assumed a dual role, that of transforming EEG signals into mutually independent bases and in ascertaining quantitative measures for those morphologic characteristics deemed important in the identification process of interictal spikes. Clinical experiments involved 31 patients with focal epilepsy. EEG data collected from 10 of these patients were used initially in a training phase to ascertain the reliability of the observable and formulated features that were used in the spike detection process. Three EEG experts annotated spikes independently. On evaluation of the algorithm using the 21 remaining patients in the testing phase revealed a precision (positive predictive value) of 92% and a sensitivity of 82%. Based on the 20- to 30-minute epochs of continuous EEG recording per subject, the false detection rate is estimated at 1.8 per hour of continuous EEG. These are positive results that support further development of this algorithm for prolonged EEG recordings on ambulatory subjects and to serve as a support mechanism to the decisions made by EEG experts.