Nonpharmacologic care for patients with Lennox-Gastaut syndrome: ketogenic diets and vagus nerve stimulation.

Individuals with Lennox-Gastaut syndrome (LGS) often do not respond to or become resistant to pharmacologic treatments. Ketogenic diets (KDs) and vagus nerve stimulation (VNS) are nonpharmacologic treatment options for these intractable patients. The classic KD, a high-fat, low-carbohydrate diet with 90% of calories derived from fat, has been used in the treatment of seizures for >90 years. About half of patients with LGS respond to the KD with a >50% reduction in seizures and some patients may achieve a >90% reduction. Vagus nerve stimulation therapy involves a surgically implanted generator that delivers intermittent electrical stimuli to the brain via an electrode wrapped around the left vagus nerve. It is utilized as adjunctive therapy for patients with drug-resistant epilepsy (including patients with LGS) who are not suitable candidates for resective surgery. Similar to the KD, about half of LGS patients respond to VNS therapy, with a >50% reduction in seizures, and the response may improve over time. Both the KD and VNS are options for patients with LGS.

The onset of pediatric refractory status epilepticus is not distributed uniformly during the day.

PURPOSE: To evaluate whether the onset of pediatric refractory status epilepticus (rSE) is related to time of day. METHOD: We analyzed the time of day for the onset of rSE in this prospective observational study performed from June 2011 to May 2019 in pediatric patients (1 month to 21 years of age). We evaluated the temporal distribution of pediatric rSE utilizing a cosinor analysis. We calculated the midline estimating statistic of rhythm (MESOR) and amplitude. MESOR is the estimated mean number of rSE episodes per hour if they were evenly distributed. Amplitude is the difference between MESOR and maximum rSE episodes/hour, or between MESOR and minimum rSE episodes/hour. We also evaluated the temporal distribution of time to treatment. RESULTS: We analyzed 368 patients (58% males) with a median (p25 - p75) age of 4.2 (1.3-9.7) years. The MESOR was 15.3 (95% CI: 13.9-16.8) and the amplitude was 3.2 (95% CI: 1.1-5.3), p = 0.0024, demonstrating that the distribution is not uniform, but better described as varying throughout the day with a peak in the morning (11am-12 pm) and trough at night (11 pm-12 am). The duration from rSE onset to application of the first non-benzodiazepine antiseizure medication peaked during the early morning (2am-3 am) with a minimum during the afternoon (2 pm-3 pm) (p = 0.0179). CONCLUSIONS: The distribution of rSE onset is not uniform during the day. rSE onset shows a 24-h distribution with a peak in the mid-morning (11am-12 pm) and a trough at night (11 pm-12am).

The ketogenic diet in pharmacoresistant childhood epilepsy.

Available pharmacologic treatments for seizures are limited in their efficacy. For a patient with seizures, pharmacologic treatment with available anticonvulsant medications leads to seizure control in <70% of patients. Surgical resection can lead to control in a select subset of patients but still leaves a significant number of patients with uncontrolled seizures. The ketogenic diet and related diets have proven to be useful in pharmacoresistant childhood epilepsy.