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+++++++++++++++++ +++ +++ +++++++++++++++++ PEDIATRICS + + Curing Epilepsy in Children Before the Die Is Cast By Ingrid Tuxhorn, MD E PILEPSY IS A lifespan chronic disorder affecting approximately 3-5% of people. Children are at a particularly high risk of being affected. While many patients with epilepsy will do well with medical management controlling their seizures and are able to lead a full unrestricted life, epilepsy can be a devastating disease impacting safety, behavior, development, academics and social relationships.1 A detailed knowledge of how epilepsy manifests in infants, toddlers, schoolaged children and adolescents is needed to facilitate accurate diagnosis, mitigate seizures and risks for neurocognitive and behavioral disorders before the die is cast. Neonatal seizures are usually caused by hypoxic ischemic brain injury related to intrauterine, birth or perinatal factors. The neonatal brain is a still developing organ with neurons being born and circuits being formed. Seizures in the newborn therefore may remain subclini- cal only visible as an EEG discharge, or manifest with fragmented motor or behavioral activity, and propagate poorly in the EEG.2 Video EEG monitoring is needed to differentiate normal from abnormal movements and measure the EEG seizures "under the tip of the ice berg". There is evidence that this will affect long term neurologic outcome of the newborn. Effective drug choices to treat seizures depend on the seizure type, syndrome and etiology. Vigabatrin has become the drug of choice in infantile spasms due to tuberous sclerosis, ACTH is widely the first line drug in infantile spasms of known or unknown causes. When treatment is delayed, previously normal infants are at high risk for loosing visual behavior and regressing irreversibly in all areas of development. Early referral for vEEG and a neurologic assessment is essential to result in remissions.3 Felbamate is highly effective for drop attacks seen in Lennox Gastaut Syndrome which may be quite refractory to multiple drugs. Ethosuccimide has become the drug of choice to treat absences which usually manifest in the preschooler and may go unnoticed till a grand mal seizure declares the seizure disorder. Academic decline is also frequently seen due to tens of seizures per day interfering with concentration and learning. The ketogenic diet has shown remarkable effectiveness in myoclonic seizures starting in infants and tod- dlers associated with sodium channel mutations or other genetic generalized epilepsies such as Doose Syndrome and Dravet Syndrome. Epilepsy due to glucose transporter deficiency is highly responsive to the ketogenic diet as ketones can become the major source of neuronal energy.4 Surgical treatment options for epilepsy have moved to the forefront in recent years as well-selected patients may be cured with minimal risks. A structural lesion causing medically refractory epilepsy – stroke, malformation, AVM, hippocampal sclerosis should be evalu- ated early for surgical remediability. The noxious effects of seizures on the healthy developing brain may impact more that the risks of surgical resection of brain tissue and plasticity will allow sub- stantial regain of function after surgery. Technologic advances in high resolution imaging, metabolic imaging with PET and SPECT, and the array if invasive electrode applications (subdu- ral, stereotactic depth electrodes) that may be used safely in children of all ages has put the early option of a surgical cure out there. Early referral even in infancy to a specialized pediatric epilepsy center is of prime importance. Cure rates are 60-80% in well selected patients.4 Specialized EEG monitoring and electrical cortical stimulation will define the epicenter of the seizure and help minimize risk of damaging functioning cortex.5 Dr. Ingrid Tuxhorn is chief, division of Pediatric Epilepsy, and Professor, Department of Pediatrics at Case Western Reserve University and University Hospitals Rainbow Babies & Children's Hospital. s References 1. Gergory Holmes. Consequences of Epilepsy through the ages: When is the die cast? Epilepsy Currents 12(4):4-22 2. Elia Pestana Knight and Ingrid E.B. Tuxhorn. Neonatal Seizures and Infantile Onset Epilepsies. In: Shorvon and Lhatoo – the Epilepsies Oxford Press 2013, Chapter 14. In press. 3. Ingrid Tuxhorn. Epileptic spasms. In: Wyllie's Treatment of Epilepsy. Principles and Practice. Pages 216-228. Wolters Kluwer 5th edition 2011 4. John Freeman, Jennifer Freeman, Millicent T Kelly. The Ketogenic Diet: A Treatment for Epilepsy. Demos 2000 5. I. Tuxhorn Electrical stimulation. In: O Catalepe and George I Jallo. Pediatric Epilepsy Surgery. Preoperative Assessment and Surgical Treatment. Thieme, New York. 2010 NOVEMBER-DECEMBER 2012 s MDNEWS.COM s MD NEWS Cleveland/Akron/Canton | 33