Abstracts

Rationale: The ketogenic diet (KD) is an evidence-based treatment for medication-resistant epilepsy. Super refractory status epilepticus (SRSE) is defined as status epilepticus (SE) that continues or recurs despite 24 hours of high dose suppressive therapy. Research has shown the benefits of the KD to treat SE. There are no known case reports describing the KD as a rescue treatment for SRSE.Methods: We report a boy with Lennox-Gastaut Syndrome of unknown etiology with four events of SRSE in an eight-year period. After failure to respond to standard treatment with high dose suppressive anticonvulsant therapy, he was treated with the classic ketogenic diet at 4:1 ratio with zero grams of carbohydrate.Results: This boy has medication resistant epilepsy with tonic-clonic, myoclonic and absence seizures, which have not been controlled despite trials of multiple anticonvulsant medications alone and in combination. Seizure onset was at 3 years of age. Anticonvulsant medications tried include topamax, levetiracetam, valproate, phenobarbital, zarontin, felbamate, lamotrigine, phenytoin and rufinamida. He developed SRSE on 4 occasions, at ages 6.8, 13.4, 13.7 and 14.4 yrs old. All episode of SRSE did not respond to traditional high dose medical treatments with phenobarbital, valproate, fosphenytoin and midazolam infusions in the ICU. Benzodiazepines produced an electroclinical worsening of seizures. The KD was utilized in all cases of SRSE with good effect. On the first episode of SRSE at age 6 years, the diet was initiated on day 19 of the ICU admission and continued for 15 days. During second episodes the KD was started after 2 days of SRSE and was used for 3 days. For the 3rd and 4th episodes, it was started after 3 days of SRSE and was used for 2 and 5 days respectively. With the first use of the KD, the child reached urine ketones of 16 mmol/L by day 7 of KD, and SE had resolved by day 12. During the 2nd and 4th episodes, he produced urine ketone of 16 mmol/L by day 1 of KD and SE had resolved by day 2. With the 3rd course of treatment, he reached ketone >16 mmol/L by day 1 and seizures stopped the same day. There were no complications during the 2nd, 3rd and 4th episodes, however, with the first course of KD, he experienced diarrhea during the first 6 days of KD. On all occasions, the family elected not to continue the KD following hospital discharge, expressing concerns with the child’s ability to adhere to the diet treatment.Conclusions: We present a case using the KD as an adjunctive rescue therapy for SRSE. The KD may be considered as an abortive therapy for SRSE, even in children who will not continue KD treatment following hospital discharge.