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Original Research ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Neurol. | doi: 10.3389/fneur.2019.00423

Ketogenic Diet As a Treatment for Super-refractory Status Epilepticus in Febrile Infection-related Epilepsy Syndrome

 Pan Peng1,  Fei Yin1, 2, Xiaolu Deng1,  Chen Chen1, Fang He1, Xiaole Wang1,  Shiqi Guang1 and Jing Peng1*
  • 1Xiangya Hospital, Central South University, China
  • 2Hunan Rehabilitation Research Center for the Disabled, China

Background: Febrile infection-related epilepsy syndrome (FIRES) is a fatal epileptic encephalopathy associated with super-refractory status epilepticus (SRSE). Several treatment strategies been proposed for this condition although the clinical outcomes are poor. Huge efforts from neurointensivists have been focused on identifying the characteristics of FIRES and treatment to reduce the mortality associated with this condition. However, the role of ketogenic diet (KD) in FIRES is not fully understood.
Methods: We performed a retrospective review of patients who met the diagnostic criteria of FIRES, SRSE and were treated with KD between 2015 and 2018 at the Department of Pediatrics, Xiangya Hospital of Central South University. The following data were recorded: demographic features, clinical presentation, anticonvulsant treatment, timing and duration of KD and follow-up information. Electroencephalography recordings were reviewed and analyzed.
Results: Seven patients with FIRES were put on KD (5 via enteral route, and 2 via intravenous line) for SRSE in the PICU. The median age was 8. Four patients were male and 3 were female. Although patients underwent treatment with a median of 4 antiepileptic drugs and 2 anesthetic agents, the status epilepticus (SE) persisted for 7-31 days before KD initiation. After KD initiation, all patients achieved ketosis and SE disappeared within an average of 5 days (IQR 3.5), although there were minor side effects. In 6 patients, a unique pattern was identified in the EEG recording at the peak period. After initiation of KD, the number of seizures reduced, the duration of seizure shortened, the background recovered and sleep architecture normalized in the EEG recordings. The early initiation of KD (at the onset of SE) in the acute phase of patients decreased the mRS score in the subsequent period (p=0.012, Ρ=0.866).
Conclusions: The characteristic EEG pattern in the acute phase promoted timely diagnosis of FIRES. Our data suggest that KD may be a safe and promising therapy for FIRES with SRSE, and that early initiation of KD produces a favorable prognosis. Therefore, KD should be applied earlier in the course of FIRES. Intravenous KD can be an effective alternative route of administration for patients who may not take KD enterally.

Keywords: Ketogenic diet (KD), febrile infection-related epilepsy syndrome (FIRES), Super-refractory status epilepticus (SRSE), PICU (pediatric intensive care unit), EEG

Received: 12 Dec 2018; Accepted: 08 Apr 2019.

Edited by:

Kette D. Valente, University of São Paulo, Brazil

Reviewed by:

Stéphane Auvin, Hôpital Robert Debré, France
Edoardo Ferlazzo, Università degli studi Magna Græcia di Catanzaro, Italy  

Copyright: © 2019 Peng, Yin, Deng, Chen, He, Wang, Guang and Peng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Jing Peng, Xiangya Hospital, Central South University, Changsha, China,