Improving Outcomes in Pediatric Epilepsy Syndromes
Stop the Status Improving OUtcomes in Pediatric Epilepsy Syndromes
– Seizures and Epilepsy Syndromes
– Seizure Emergencies
– Febrile Seizures
Seizure Epilepsy Syndrome Facts
• 2.5 million Americans have epilepsy (defined as 2 or more unprovoked seizures)
• Children with epilepsy syndromes represent about 15% of the childhood epilepsy population
• About ½ of the epilepsy syndromes in children are considered catastrophic, drug resistant, and prone to status epilepticus
• There are approximately 150,000 cases of status epilepticus (seizure > 30 minutes) each year in the US; most cases occur in children 2 and under and the elderly
• The children with catastrophic epilepsy syndromes often have status epilepticus multiple times per week
Seizure Emergency Definitions
• Status Epilepticus–Operational Definition is seizure duration of 5 minutes or longer. Seizures that last 5 minutes should be treated.
• Convulsive Status Epilepticus occurs when seizure last 30 minutes or longer OR patient has 2 or more seizures without full recovery between seizures.
–Acute Repetitive Seizures aremultiple seizures within 24 hours for adults (12 hours for children) despite optimal therapy and recognizable by the patient’s caregiver as distinguishable from other seizures.
Morbidity and Mortality of Status Epilepticus: Every Minute Counts•For each minute delay from start of convulsive status epilepticus (seizure lasting five minutes) to time patient arrives at emergency department (ED), there is a 5% added cumulative risk that the seizure will last > 60 minutes.
• In one prospective study, arrival at ED 40 minutes after onset of convulsive status epilepticus irrespective of pre-hospital treatment with rectal diazepam was associated with a 4.3x increased risk of seizure lasting > 60 minutes compared to arrival at ED within 10 minutes.
• Delaying treatment with a benzodiazepine causes resistance to benzodiazepine treatment through internalization of benzodiazepine receptors as seizure length increases, making it harder to stop a seizure as time goes on.
Delayed Medical Treatment for Status Epilepticus in the US Leads to Increased Morbidity and Mortality
• Community –based treatment of children with epilepsy syndromes is imperative, as morbidity and mortality from seizures increases proportionately to time lapse from seizure onset
• An 85 minute average time to medical treatment from seizure onset is unacceptable in life-threatening situations such as status epilepticus
• These children are chronically at high risk of death and brain damage
Morbidity of Status Epilepticus in Children
• Association with development of temporal lobe epilepsy
• 20-40% risk of developing epilepsy with status epilepticus as first seizure vs. 10% risk in first seizure lasting < 30 minutes
• Chronic encephalopathy and brain atrophy found in 6-15% of cases
• Focal neurologic signs in 9-11% of cases of children with SE
Morbidity and Mortality of Status Epilepticus in Children
• Retrospective and hospital based study concluded the major determinant of intelligence quotient (IQ) in children with ‘‘generalized idiopathic epilepsy’’ was a history of convulsive status epilepticus
• Prevention of recurrent episodes of SE are critical to long term seizure prevention and cognitive outcome in certain epilepsy syndromes, such as Dravet syndrome. In a study of 31 adults with Dravet syndrome, seizure freedom in adulthood was correlated to < 3 episodes of convulsive status epilepticus as a child
• Occurs in 2-4% of children in the US
• Approximately 25% of initial FS lasts more than 10 minutes and are considered complex
• 30-50% of patients with an initial FS will have a second FS
• Occurrence of a prolonged FS increases the risk of Epilepsy (30 % risk with prolonged seizure vs. 10% risk with seizure< 5 minutes)
• If a child with an initial prolonged FS experiences a second FS it is likely to be prolonged
Morbidity in Febrile Status Epilepticus
• Relationship between prolonged febrile seizures and later temporal lobe epilepsy associated with mesial temporal sclerosis is important, because the incidence of epilepsy following a prolonged febrile seizure may potentially be reduced by stopping the seizure and/or using neuroprotective agents.
Have A Plan
• Have a written plan to treat your child with a benzodiazepine such as rectal diazepam (Diastat) or buccalmidazolam(Epistatus–available in UK) to prevent status epilepticus
• If there are intravenous drugs that worsen seizures in your child (such as phenytoinor fosphenytoinin Dravet syndrome), these should be listed as contraindicated on the written plan
• Don’t wait. The seizure should be stopped in under an hour for best outcome.