Advances and challenges in the delivery of care for children diagnosed with status epilepticus

Administrator: Cigdem Akman, US

Dr Benilda Sanchez-Gan
University of Manilla
beniganmd@gmail.com
Advances in the definition and classification of pediatric status epilepticus
Status epilepticus (SE) is one of the most common medical emergencies seen in the pediatric age groups. In 1981, international league against Epilepsy (ILEA) accepted the definition of SE which described an acute condition of a prolonged seizure (>30 min) or repetitive seizures without regaining mental status.Over the years, our knowledge on SE has expanded from the role of anticonvulsant treatment, acute and long term outcome of patens following SE. A number of animal model of SE also demonstrated structural and cognitive changes which underlined the importance of timely treatment for cessation of seizures. Therefore, a new definition was introduced in 2015 by ILAE which adopted a shorter seizure duration, a seizure longer than 5 min was to be considered for SE.Recently a new proposal has been introduced based on the clinical features of SE which includes seizure semiology, EEG findings, underlying etiology and age of the patients.

Learning objectives:
1. To present and discuss the latest definition of status epilepticus as proposed by the ILAE Commission on Classification and Terminology.
2. To discuss the previous and latest classification of status epilepticus.
4. To present cases of children with status epilepticus and classify their SE based on the latest classification.

Dr. Howard Goodkin
University of Virginia
hpg9v@hscmail.mcc.virginia.edu
Pharmacotherapy For Convulsive Status Epilepticus: Guidelines Proposing a Timeline‐Based Algorithm
The treatment of status epilepticus, a neurological emergency characterized by an abnormally prolonged seizure, was greatly impacted by the discovery of the benzodiazepines in the mid-20th century, and, more recently, by the results of the Established Status Epilepticus Treatment Trial (ESETT). Current treatment algorithms emphasize the need to rapidly treat with the goal of terminating the seizure as quickly as possible in an effort to reduce irreversible, structural or functional, injury to the brain. While early treatment has been long recognized, treatment of status epilepticus in children often remains delayed, and this delay has been associated with a poor short term outcome including a higher frequency of death.

Learning objectives:
1. To make the clinician aware of the human and animal data that supports timely treatment of status epilepticus
2. To assist the clinician by providing the evidence that supports the current treatment algorithm for the treatment of status epilepticus
3. To make clinicians aware of research data that suggests that future treatment of status epilepticus may need to be modified based on etiology

Dr. Seda Kanmaz
Ege University Faculty of Medicine
seda.kanmaz@ege.edu.tr
Clinical outcome and risk factors for recurrence of SE in children: Role of child neurologists to plan of care in the aftermath of SE.
Children diagnosed with Status epilepticus (SE) are at risk for acute complications as well as long term problems including epilepsy, cognitive and behavioral impairment, and focal neurological disorders. Prolonged, refractory SE has a mortality of 30% to 50%, and most survivors have poor functional outcomes. The most important outcome factors are the underlying etiology and the time between the onset of SE and treatment.The risk of SE recurrence is associated with chronic etiology, especially genetic mutations in cases occurring with gross structural cerebral disorders, inborn errors of metabolism, early childhood epileptic encephalopathy, or epilepsy syndromes. Early detection of cases at higher risk of SE recurrence may affect their treatment during follow-up. Multidiciplinary care for management (feeding issues, tracheostomy, rehabilitation medicine) is critical to optimize the functional outcome.

Learning objective
1.To characterize the demographics, outcomes, and prognostic factors in CSE, with particular focus on refractory status epilepcus (RES), managed with a restricted departmental protocol (EPSES®).
2.To learn current guidelines on management and outcome of SE and particularly refractory SE
3.To review steps for longterm care coordination for children presenting with SE: Multidisciplinary approach

Dr. Cigdem Akman
Columbia University Medical Center
cia11@cumc.columbia.edu
Challenging aspect of Status Epilepticus in children: Non-convulsive Status Epilepticus
Nonconvulsive status Epilepticus (NCSE) is a serious condition of prolonged or repetitive seizures with impairment of consciousness and /or behavioral changes without convulsions. In the absence of frank convulsions, NCSE is determined based on the EEG wave patterns and corresponding clinical features of changes in behavior or mental status. Prolonged or repeated short EEG recordings are useful diagnostic tools for the timely management, and monitor the efficacy of treatment.Underlying causes of NCSE is not limited to coma or acute CNS insult (trauma, stroke, CNS infection) and can occur in children with autoimmune encephalopathy, genetic epilepsy or childhood epilepsy syndromes.NCSE a treatable condition, and prognosis depends on the underlying etiology as well as timely treatment. However guidelines for diagnosis and treatment remains not firmly established. The clinical presentation of NCSE in children is diverse and EEG findings are more complex and the underlying etiology varies by age (such as genetic epilepsies.Clinical suspicion and EEG monitoring are essential for earlier diagnosis however in the presence of limited resources, shorter and repeated EEG recording will help us to achieve our goals.

Learning objective:
1.To learn the definition and diagnostic tools for diagnosis of NCSE
2.To review risk factors for NCSE in children
3.To learn treatment approaches and outcome of NCSE in children