Ketogenic diet in pediatric super refractory status epilepticus- short- and long-term outcomes; 15 years single center experience
Abstract number :
2.256
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2025
Submission ID :
388
Source :
www.aesnet.org
Presentation date :
12/7/2025 12:00:00 AM
Published date :
Authors :
Presenting Author: Raid Hommady, MD – University of Toronto, The Hospital for Sick Children
Karen Owusu, NP – University of Toronto, The Hospital for Sick Children
Helen Lowe, RD – University of Toronto, The Hospital for Sick Children
Jolynn Dickson, RD – University of Toronto, The Hospital for Sick Children
Jeff Kobayashi, MD – University of Toronto, The Hospital for Sick Children
Suvasini Sharma, MD – University of Toronto, The Hospital for Sick Children
Rationale: Ketogenic diet (KD) is increasingly utilized in the ICU for patients with super refractory status epilepticus (SRSE). A subset of these patients have new onset refractory status epilepticus (NORSE) or Febrile infections related epilepsy syndrome (FIRES). These conditions are medically challenging and difficult to treat with high morbidity and mortality. There is still a paucity of evidence-based treatments which is limited to case series. There is a lack of data on the long-term outcomes of children who started on the KD for SRSE.
Methods: This was a retrospective cohort study which included all patients diagnosed with SRSE and initiated on KD from 2009 to 2024 at the Hospital for Sick Children. Short term outcomes included electroclinical seizure cessation, adverse events of the KD, and functional status at time of hospital discharge, while long term comes included duration of continuation of the KD post discharge, seizure control status and functional outcomes at the last follow up. Functional status was determined by the modified Rankin scale score (mRS).
Results: A total of 31 patients (17 girls) with SRSE were initiated on KD during the study period. The median age at SRSE onset was 6.8 years (IQR 3-11 years) with 6 patients being less than 12 months of age. Twenty-three (74%) had typical developmental status prior to SRSE onset. Nine (29%) had prior diagnosis of epilepsy before SRSE onset. 21 (67%) had NORSE, and 14 of these had FIRES. KD was effective in achieving electroclinical seizure cessation in 17/31 (54%) patients. The median duration to achieve seizure resolution was 7.4 days (IQR 4-8 days) with 2.1 days (IQR 1-2.5days) time to achieve ketosis. Weight loss (61%) and hypoglycemia (45%) were the most common adverse events. 45% were seizure free at time of hospital discharge and 28% had partial seizure control with less than one motor seizure per week. 18 (58%) patients continued the KD post discharge with a median duration of 1.9 years (IQR 6 months-3.25 years). At the last follow up (median duration of 3.1years (IQR 1.5-5years), 7 patients were seizure free, eight had partially controlled seizures. Nine patients had normal to only slight disability (0-2 Modified Rankin Score (MRS)).
Conclusions: This is the largest single center study of children with SRSE treated with KD till date. In this study, initiation of KD therapy for patients with SRSE in the ICU was found to be safe, tolerable, and effective in the management of SRSE. Two thirds of our patients continued the diet post discharge. Long term outcomes were promising with 50% of children having seizure freedom and normal functional status/ mild disability.
Funding: None
Clinical Epilepsy