Monday, August 1, 2016

Ketogenic diet treatment for pediatric super-refractory status epilepticus.

Appavu B, Vanatta L, Condie J, Kerrigan JF, Jarrar R. Ketogenic diet treatment
for pediatric super-refractory status epilepticus. Seizure. 2016 Jul 21;41:62-65.

We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm.
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.
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.
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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From the article:

In our experience, most of our patients (90%) had resolution of SRSE with KD therapy, and the therapy was tolerated well with minimal side effects.

The utilization of KD as a treatment for RSE has been reported. Several groups have reported on children with RSE treated with KD, of whom many have had a beneficial response.  Nine children with RSE secondary to FIRES had been reported, of whom seven had resolution of SE within four days of treatment. With respect to SRSE, a series of 10 adult patients was reported.  9/10 patients had resolution of SE within a median of three days after KD initiation, seizure resolution occurred in 7/10 patients within one week of KD initiation, and the only patient without SRSE resolution failed to reach ketosis. A pediatric series was reported of four children with SRSE treated with KD, of whom all were weaned off anesthesia with variable residual seizure burdens.

Our study is limited by its retrospective nature, a small sample size, the lack of consistent acquisition of serum beta-hydroxybutyrate levels, and the concomitant use of other agents while KD was utilized. In some patients receiving concurrent therapies directed at an underlying diagnosis, resolution of SRSE cannot be directly attributed to the KD. Prospective trials are needed to directly link the effectiveness of KD to SRSE, identify predictors of treatment responsiveness, and determine a dose-responsive relationship of treatment to SRSE. This, however, may be difficult to achieve given how critically ill most of these patients are.

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