Unmasking Triggers in the Management and Detection of Status Epilepticus in Lennox-Gastaut Syndrome: Babitha Haridas, MD, MBBS

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"This helps remove a little bit of that element of unpredictability with seizures... giving us an opportunity to act quickly when triggers emerge."

Status epilepticus, also known as a status seizure, is a medical emergency that occurs when a person has a single seizure that lasts longer than 5 minutes, or multiple seizures within a 5-minute period without regaining consciousness between them. For convulsive status epilepticus, patients with short tonic-clonic seizures that only last a couple of minutes often may remain unresponsive for several minutes because of the seizure’s post-ictal state. Over the years, there has been an increased effort to understand to predict and manage status epilepticus across a variety of seizure disorders, including Lennox-Gastaut syndrome (LGS).

A newly conducted retrospective study, using prospective data from the pediatric Status Epilepticus Research Group (pSERG), revealed that patients with LGS represent a sizable fraction of status epilepticus patients, and present frequently with intermittent seizures and delayed treatment, in the setting of seizure triggers. The study, presented at the 2024 American Epilepsy Society (AES) Annual Meeting, held December 6-10, in Los Angeles, California, comprised of 845 patients, 51 of which had a diagnosis of LGS or a history of developmental delays with tonic seizures. Overall, the most common etiology of status epilepticus was a systemic infection (33%; n = 17), change in medication regimen (13%; n = 7), and unknown (19%; n = 10).

In a conversation with NeurologyLive,lead investigator Babitha Haridas, MD, MBBS, discussed the importance of identifying triggers to status epilepticus in this patient population. Haridas, pediatric epileptologist at Johns Hopkins Medicine, emphasized the importance of distinguishing between intermittent and continuous status epilepticus. In addition, she emphasized the need to understand the diverse manifestations of status epilepticus, and how the findings underscore more thorough evaluation in emergency settings and heightened awareness of atypical status epilepticus presentations in LGS.

The Pediatric Epilepsy Research Consortium (PERC) is a network of clinical sites focused on research related to pediatric epilepsy. The consortium conducts multicenter studies aimed at improving understanding, diagnosis, and treatment of epilepsy in children. More information about PERC is available at www.perc-epilepsy.org.

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REFERENCE
1. Haridas B, Loddenkemper T, Tasker R, et al. Management of Status Epilepticus in Lennox Gastaut Syndrome. Presented at: 2024 AES Annual Meeting; December 6-10; Los Angeles, CA. ABSTRACT 2.251