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Ketogenic diet treatment for pediatric super-refractory status epilepticus
Brian Appavu1, Lisa Vanatta1, John Condie1
1Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States.
Insights
Ketogenic diet (KD) therapy effectively resolved super-refractory status epilepticus in most pediatric patients. This dietary intervention showed promise for early treatment with minimal harm.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Metabolic Disorders
Background:
- Super-refractory status epilepticus (SRSE) is a severe neurological emergency in children.
- Current treatment options for SRSE are limited and often associated with significant morbidity.
- The ketogenic diet (KD) is a high-fat, low-carbohydrate diet used to manage epilepsy.
Purpose of the Study:
- To evaluate the efficacy and safety of ketogenic diet (KD) therapy in pediatric patients with super-refractory status epilepticus (SRSE).
- To determine if KD therapy can lead to the resolution of SRSE without causing significant harm.
Main Methods:
- A retrospective review of pediatric patients diagnosed with SRSE who underwent KD therapy between 2011 and 2015.
- Data collected included patient demographics, etiology of SRSE, treatment duration, and outcomes.
- Analysis focused on SRSE resolution, reduction in antiepileptic medications, and adverse events.
Main Results:
- Ten pediatric patients (ages 2-16) with SRSE were identified, with diverse etiologies including autoimmune encephalitis and genetic epilepsies.
- Nine out of ten patients experienced resolution of SRSE within a median of 7 days after initiating KD.
- Most patients were weaned off anesthesia shortly after achieving ketonuria, with only one patient experiencing diet-related side effects.
Conclusions:
- Ketogenic diet (KD) therapy appears to be an effective treatment for super-refractory status epilepticus (SRSE) in pediatric patients.
- KD therapy can be considered as an early therapeutic option for children with SRSE.
- The diet demonstrated a favorable safety profile in this cohort, suggesting its potential for early utilization.
Purpose:
We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm.
Method:
A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015.
Results:
Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation.
Conclusion:
Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus.
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