Wirrell, Elaine

Epileptic spasms, or seizures marked by flexor, extensor, or flexor-extensor spasms, are not always responsive to medical management. The purpose of our study was to evaluate the outcome of epilepsy surgery in children with medically intractable epileptic spasms. We identified 11 children with epileptic spasms who underwent lesionectomy (36%), lobectomy (27%), multi-lobectomy (9%), hemispherectomy (18%), or corpus callosotomy (9%). At the time of surgery, 6 children had developed other concurrent seizure type(s), including simple partial (9%), complex partial (27%), partial undifferentiated (9%), primary generalized tonic clonic (9%), tonic (9%), atonic (27%), and myoclonic (9%) seizures. Six children (55%) were seizure free at last follow-up from initial surgery. Predictors of favorable outcome included lack of focal slowing and the presence of less than 2 interictal epileptiform abnormalities on postoperative electroencephalogram ( P = .035 and .035, respectively). Favorable outcome was significantly associated with parent/caregiver report of improved postoperative developmental outcomes ( P = .026).

The temporal lobe is a common focus for epilepsy. Temporal lobe epilepsy in infants and children differs from the relatively homogeneous syndrome seen in adults in several important clinical and pathological ways. Seizure semiology varies by age, and the ictal EEG pattern may be less clear cut than what is seen in adults. Additionally, the occurrence of intractable seizures in the developing brain may impact neurocognitive function remote from the temporal area. While many children will respond favorably to medical therapy, those with focal imaging abnormalities including cortical dysplasia, hippocampal sclerosis, or low-grade tumors are likely to be intractable. Expedient workup and surgical intervention in these medically intractable cases are needed to maximize long-term developmental outcome.

The role of surgery in continuous spike wave in slow-wave sleep has not been robustly explored. The authors present a case of an 11-year-old boy with refractory partial seizures and continuous spike wave in slow-wave sleep who was treated with an anterior temporal lobectomy. His presurgical brain magnetic resonance imaging revealed right basal ganglia and thalamic infarcts and right mesial temporal sclerosis. Following surgery, he achieved seizure freedom, cessation of continuous spike wave in slow-wave sleep, and improved cognition. This case provides further evidence that epilepsy syndromes with generalized electrographic discharges can be secondary to focal pathology and potentially cured with resective surgery. The normalization of his sleep electroencephalogram following the anterior temporal lobectomy suggests that temporal lobe structures may be involved in the seizure network needed to generate continuous spike wave in slow-wave sleep.

The use of valproate sodium as an antiepileptic is not advised in children with an undiagnosed metabolic condition because of the increased risk of hepatotoxicity and encephalopathy. Here the authors describe a 2.5-year-old girl with a history of developmental delay, failure to thrive, and a seizure disorder whose seizures worsened after the introduction of valproate sodium. This led to a search for an underlying metabolic disorder, and after extensive investigations, a diagnosis of nonketotic hyperglycinemia was made. In this report, the authors discuss the metabolic conditions that can be worsened by valproate sodium. Valproate sodium interferes with the glycine cleavage enzyme synthesis in the mitochondria, hence increasing glycine levels. The increased glycine levels are responsible for worsening of the underlying metabolic condition and increased seizure frequency.

Generalized periodic epileptiform discharges can occur in a variety of epileptic syndromes and herald impending seizures. Such discharges are also extremely rare in children. Cerebral visual impairment can be associated with generalized and partial onset seizures, particularly those involving the occipital lobe. The authors present a case of a 10-month-old boy whose electroencephalogram revealed generalized periodic epileptiform discharges following resolution of status epilepticus. Such discharges warranted further monitoring, during which he experienced 2 additional seizures. He also was discovered to have cerebral visual impairment, which slowly resolved following termination of seizure activity. The child was subsequently found to have a de novo mutation of the sodium channel, voltage-gated, type I, alpha subunit (SCN1A) gene consistent with Dravet syndrome.

The goal of this review is to discuss the comorbidities reported in specific epilepsy syndromes to examine possible underlying causes or associations and to present data on current therapies for these conditions. Comorbid conditions including cognitive impairment, neuropsychiatric problems, and social difficulties are common in children with epilepsy, and often more disabling than the seizures themselves. Biological factors associated with a greater risk of comorbidity in epilepsy include younger age at seizure onset, cognitive impairment, temporal or frontal lobe onset, and intractability. Social factors correlating with greater risk include lower socioeconomic status, lower parental education level, and poorer family function. These comorbid conditions not only have a significant impact on the child but also are a source of increased stress and burden for families. Increased awareness and early diagnosis of these conditions may affect therapeutic intervention and long-term outcome as well as assist in better understanding of potential risk factors and biological mechanisms

Seizures in the newborn period are common and frequently indicate serious underlying brain injury. Although accumulating evidence suggests that they may impair brain development, there are currently no evidence-based guidelines for evaluation and management of neonatal seizures. In this review, we will address some of the current controversies facing child neurologists and neonatologists, including how to define, monitor, and treat neonatal seizures.

The feasibility and clinical utility of early electroencephalogram (within 48 hours) was studied in 127 children (age, 1 month-17 years) referred for a “first seizure.” The electroencephalogram was considered late after 48 hours. Electroencephalogram abnormalities were classified as nonepileptiform or epileptiform. Children were classified as having an “epileptic” or “nonepileptic” event. An early electroencephalogram was obtained in 23 (18%). Late referral (n = 36), weekend event (n = 23), difficulty contacting families (n = 11), parental schedules (n = 9), and laboratory scheduling (n = 11) resulted in late electroencephalograms. All 94 children with an epileptic event had an electroencephalogram, 19 (20%) within 48 hours. Results were abnormal in 9 (47%) early (7 epileptiform, 2 nonepileptiform) and 35 (44%) late (30 epileptiform, 5 nonepileptiform). Increased abnormalities were not seen with early electroencephalography ( P = .50). Early electroencephalograms may not be feasible in the pediatric population and did not show a higher yield of abnormalities.