Epilepsy syndromes you should recognize
Studies confirm that surgery has a 67%-90% chance of obtaining complete seizure control without yielding new neurologic deficits. Clinical studies also demonstrate improved quality of life and developmental outcomes.
Patients undergoing epilepsy surgery have less than 5% risk of bleeding, infection, and stroke, and less than 1% chance of death. When comparing these risks with those of continued seizures – depression, anxiety, suicidal ideation, academic failure, poor employment record, and sudden unexpected death in epilepsy or SUDEP – surgery is favorable.
Dravet syndrome
Pediatricians should also not miss Dravet syndrome, or severe myoclonic epilepsy of infancy. This syndrome usually presents at 4-6 months as complex febrile seizures.
Initially, the child’s development and interictal EEGs are normal. However, between age 1 and 4 years, the child develops febrile and afebrile seizures of multiple types, including generalized tonic-clonic, complex partial, tonic/atonic and myoclonic seizures. Interictal EEGs become epileptogenic, with multifocal and generalized discharges. Development falters and a slow but steady cognitive, psychosocial, and motor decline begins; 70% have autistic spectrum disorder.
As they age, patients have pes planus (flat feet) and a stooped ataxic gait. They often have sleep disturbances and cardiac abnormalities. These children have higher risks of SUDEP, and 18% have immunologic disorders. This epilepsy syndrome is difficult to control, but seizure management is typically optimized with a combination of antiepileptic medications: valproate, topiramate, clobazam, and ketogenic diet. Some common antiepileptic medications will worsen Dravet syndrome seizures: phenytoin, carbamazepine, oxcarbazepine, and lamotrigine.
Dr. Zupanc is director of the Pediatric Comprehensive Epilepsy Program at Children’s Hospital of Orange County in Orange, California, and is professor of neurology and pediatrics at the University of California, Irvine. Dr. Zupanc disclosed she is on the advisory board and speakers bureau for Lundbeck and is a consultant to Questcor.
