Oral Dexamethasone Pulse Therapy in Children with Intractable Epilepsy and Epileptic Encephalopathy During the COVID-19 Pandemic
Abstract number :
3.214
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2022
Submission ID :
2204326
Source :
www.aesnet.org
Presentation date :
12/5/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:24 AM
Authors :
Christine Tran, MD – Magnificent Minds Neurology Center; Deborah Holder, MD – Pediatric Neurology, Epilepsy – Children's Hospital Los Angeles; Jonathan Santoro, MD – Pediatric Neurology – Children's Hospital Los Angeles
Rationale: Multiple therapies exist for treatment of intractable pediatric epilepsy including traditional anti-seizure medications, steroids, intravenous immunoglobulin therapy, ketogenic diet, neuromodulation and surgery. Intravenous steroid therapy have been used safely and effectively intractable epilepsy. However, this intervention requires hospital-based care which was problematic during the height of the COVID-19 pandemic. In this study, we evaluated the safety and efficacy of home-based oral dexamethasone pulse therapy in the treatment of intractable epilepsy and epileptic encephalopathy.
Methods: A single center retrospective review was performed following IRB approval. Patients aged 1 year to 25 years with a diagnosis of pediatric onset medically refractory epilepsy or epileptic encephalopathy were reviewed for use of oral dexamethasone between March 15, 2020 and December 31, 2021. All patients received dexamethasone 20 mg/m2/day divided three times per day for three days (maximum 8 mg three times per day). The seizure frequency, percentage seizure reduction compared to baseline, number of anti-seizure medications before and after steroid pulse, weight and blood pressure were evaluated to assess efficacy and adverse effects.
Results: Twenty-four patients aged 1 to 25 (mean 11, SD 5.2) years were included. There was equal distribution between males and females. Etiologies comprised of Lennox-Gastaut syndrome (n=3), Landau Kleffner syndrome (n =4), Dravet syndrome (n=2), infantile spasm (n=1), GLUT1 deficiency syndrome, KCNT1-related epilepsy (n=2), GRIN2B-related neurodevelopmental disorder (n=2), Sturge Weber syndrome (n=1), Down syndrome (n=1), Hashimoto encephalopathy (n=1), hypoxic ischemic encephalopathy (n=1), brain tumor (n=1), traumatic brain injury (n=1) and unknown (n=3). Patients in this cohort had abnormalities in the following genes or chromosome: SCN1A (n=2), KCNT1 (n=2), GRIN2B (n=2), SLC2A1 (n=1) and 18p (n=1). After therapeutic intervention, 16 of 24 patients (66%) showed a reduction in seizure frequency (mean percent seizure reduction was 45%). Of this cohort, 12 patients (50%) showed a reduction in seizure frequency of at least 50% or greater and 5 patients (21%) became seizure free at follow up. Four patients (17%) reported cognitive and language improvement. Prior to steroid pulse, the mean number of anti-seizure medications taken was 2.7 and the mean number of anti-seizure medications taken post-steroid pulse was 2.2. The mean and median weight change after steroid pulse were +2.79 kg and +2.22 kg, respectively. The mean and median blood pressure percentile change after steroid pulse were +3.9% and +7.5%, respectively. No adverse events were reported.
Conclusions: This study showed that oral dexamethasone pulse therapy can be an effective alternative to IV methylprednisolone in children with intractable epilepsy or epileptic encephalopathy and can be safely completed at home. Further investigation into dexamethasone as adjunct therapy in children with medically refractory epilepsy is warranted.
Funding: None
Clinical Epilepsy