Humans ; Drug Resistant Epilepsy ; Neurodevelopmental Disorders/genetics*

Epilepsy is one of the world's most common neurologic disorders and epilepsy surgery is a major treatment option for intractable epilepsy in pediatric patients. Understanding the sequential steps of epilepsy surgery is important for the selection of appropriate candidate for surgery. Surgical methods in epilepsy surgery should be decided according to the results of the presurgical evaluation and they can be categorized into resective surgery and disconnective surgery. The implantation of neuromodulation is also a potential surgical approach. Here, we reviewed the current state of presurgical assessment for intractable epilepsy in pediatric patients, novel surgical procedures, and associated outcome data.
Child ; Drug Resistant Epilepsy ; Epilepsy* ; Humans ; Nervous System Diseases

Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development.
Brain ; Cerebral Cortex ; Classification ; Drug Resistant Epilepsy ; Epilepsy ; Humans ; Magnetic Resonance Imaging ; Malformations of Cortical Development* ; Neurogenesis

Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development.
Brain ; Cerebral Cortex ; Classification ; Drug Resistant Epilepsy ; Epilepsy ; Humans ; Magnetic Resonance Imaging ; Malformations of Cortical Development* ; Neurogenesis

Temporal lobe epilepsy, with a variety of etiological, symptomatic, electrophysiological characteristics, has the highest incidence among all focal epilepsy, and a high rate of progression to refractory epilepsy. Surgery is an effective treatment, but traditional methods are usually difficult to accurately locate the epileptogenic zone, which may be resolved by stereotactic-electroencephalogram(SEEG) technique. Radiofrequency thermocoagulation and MRI-guided laser interstitial thermal therapy based on SEEG provide a new accurate and minimally invasive choice for refractory epilepsy patients with high surgical risk and difficulty.
Drug Resistant Epilepsy/surgery* ; Electrocoagulation/methods* ; Electroencephalography ; Epilepsy, Temporal Lobe/surgery* ; Humans ; Stereotaxic Techniques

An uncommon female-limited intractable epilepsy, protocadherin (PCDH) 19-related epilepsy, is characterized by mutations in the PCDH 19 gene, located on chromosome X. Clinical symptoms include early onset, fever sensitivity, focal seizures and psychomotor retardation. PCDH 19-related epilepsy is unresponsive to conventional antiepileptic drugs (AEDs), but corticosteroid is reported to be effective in a few cases. We report a case of a 25-month-old girl who was admitted to our hospital due to developmental regression, accompanied by aggravated seizures with fever. Although several conventional AEDs were administered, the frequency and severity of seizures increased with mild fever, and the symptoms did not improve. Considering possible immune, and inflammatory involvement in seizure generation, the patient was administered corticosteroid treatment during the acute phase. Corticosteroid dramatically improved seizures and her development gradually. The patient was finally diagnosed with PCDH 19-related epilepsy in genomic evaluation. We observed the effect of corticosteroid on intractable epilepsy in patient with PCDH 19 mutation. If a female patient whose seizures are resistant to conventional AEDs or easily provoked by mild fever, has developmental delay or developmental regression, this may be an important clinical clue to the early diagnosis of PCDH 19-related epilepsy.
Adrenal Cortex Hormones ; Anticonvulsants ; Child, Preschool ; Drug Resistant Epilepsy ; Early Diagnosis ; Epilepsy* ; Female ; Fever ; Humans ; Seizures

Epilepsy is one of the most common chronic neurological disorder affecting 6–7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.
Adult ; Child ; Drug Resistant Epilepsy ; Epilepsy ; Humans ; Nervous System Diseases ; Plastics ; Quality of Life ; Seizures

This study investigates the sensitivity and specificity of predicting epileptic seizures from intracranial electroencephalography (iEEG). A monitoring system is studied to generate an alarm upon detecting a precursor of an epileptic seizure. The iEEG traces of ten patients suffering from medically intractable epilepsy were used to build a prediction model. From the iEEG recording of each patient, power spectral densities were calculated and classified using support vector machines. The prediction results varied across patients. For seven patients, seizures were predicted with 100% sensitivity without any false alarms. One patient showed good sensitivity but lower specificity, and the other two patients showed lower sensitivity and specificity. Predictive analytics based on the spectral feature of iEEG performs well for some patients but not all. This result highlights the need for patient-specific prediction models and algorithms.
Drug Resistant Epilepsy ; Electrocorticography* ; Electroencephalography ; Epilepsy* ; Humans ; Seizures ; Sensitivity and Specificity ; Support Vector Machine

BACKGROUND AND PURPOSE: Perampanel is the first α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA)-receptor antagonist developed to treat epilepsy. The effects of either rapid or slow dose titration on adverse events remain to be elucidated. METHODS: Eighty-five patients received perampanel between March 2016 and August 2016. Patients were divided into two groups according to their dosing schedule: rapid dose titration (2-mg increments at intervals of 1 to 2 weeks) and slow dose titration (2-mg increments at intervals of at least 3 weeks). Seizure frequency and adverse events were analyzed over 3 months. RESULTS: Adverse events were reported by 47 (58%) of the 81 patients analyzed, with 12 (15%) patients discontinuing perampanel due to adverse events. Common adverse events included dizziness (n=30, 37%), aggressive mood and behavior (n=19, 24%), gait disturbance (n=16, 20%), and sleep problems (n=10, 12.4%). The overall adverse events were similar in the slow-titration group (38 of 61 patients) and the rapid-titration group (8 of 20 patients, p=0.081). However, none of the 20 patients in the slow-titration group experienced gait disturbance, compared with 16 of the 61 patients in the rapid-titration group (p=0.009), while appetite change was experienced by 4 patients in the slow-titration group but only 1 in the rapid-titration group (p=0.003). No relationship was noted between adverse events and the maximum dose of perampanel (p=0.116). Sex differences were observed, with the response to perampanel being better and the rate of adverse events being higher in females (p=0.015 and p=0.046, respectively). CONCLUSIONS: Slow titration of perampanel may reduce perampanel-related adverse events.
Appetite ; Appointments and Schedules ; Dizziness ; Drug Resistant Epilepsy* ; Epilepsy ; Female ; Gait ; Humans ; Seizures ; Sex Characteristics

Drug-refractory epilepsy (DRE) may be treated by surgical intervention. Intracranial EEG has been widely used to localize the epileptogenic zone (EZ). Most studies of epileptic network focus on the features of EZ nodes, such as centrality and degrees. It is difficult to apply those features to the treatment of individual patients. In this study, we proposed a spatial neighbor expansion approach for EZ localization based on a neural computational model and epileptic network reconstruction. The virtual resection method was also used to validate the effectiveness of our approach. The electrocorticography (ECoG) data from 11 patients with DRE were analyzed in this study. Both interictal data and surgical resection regions were used. The results showed that the rate of consistency between the localized regions and the surgical resections in patients with good outcomes was higher than that in patients with poor outcomes. The average deviation distance of the localized region for patients with good outcomes and poor outcomes were 15 mm and 36 mm, respectively. Outcome prediction showed that the patients with poor outcomes could be improved when the brain regions localized by the proposed approach were treated. This study provides a quantitative analysis tool for patient-specific measures for potential surgical treatment of epilepsy.
Humans ; Epilepsy/surgery* ; Brain/surgery* ; Electrocorticography/methods* ; Drug Resistant Epilepsy/surgery* ; Brain Mapping/methods* ; Electroencephalography/methods*