Moseley, Brian D.

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.