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(Abst. 3.335), 2014

Authors: Ara Hall, Megan Gustafson and Lindsey Thompson
Content: Rationale: The ketogenic diet is an effective treatment for the reduction of seizures in infants and children with refractory epilepsy. The safety and tolerability of the ketogenic diet is unknown for infants and neonates being treated for neonatal epileptic encephalopathy and refractory seizures in the neonatal intensive care unit (NICU). Methods: We present three cases of the initiation and use of the ketogenic diet in the NICU. We thoroughly reviewed (with signed parental consent) each patient's chart including medical history, daily progress notes, relevant laboratory data, imaging, diagnostic information and parental and caregiver perception of the complications and benefits noted with ketogenic diet initiation. Results: Each patient failed at least three antiepileptic medications prior to ketogenic diet initiation. Metabolic screening was performed for all patients. Case 1: The ketogenic diet was initiated at 10 weeks of age in a female patient with known SCN2B gene mutation and resulting epileptic encephalopathy. Case 2: The diet was started at 9 weeks of age in a female infant with idiopathic Ohtahara syndrome. Case 3: The diet was initiated on a19 day old male neonate with refractory seizures secondary to multiple structural brain abnormalities. All three patients tolerated a non-fasting induction into ketosis over one to two weeks. Constipation was noted in two cases and successfully treated with additional MCT oil. Diet initiation was slowed for two patients secondary to low blood glucose. In Case 1, the patient required continuous nasogastric feedings due to preexisting hypoglycemia that persisted with ketogenic diet induction. A reduction in seizures was noted in two patients. The infant in Case 3 has not had an improvement in seizure frequency. Improved alertness and awareness were reported in all three cases by caregivers and parents. Two patients were able to be weaned from respiratory support to either nasal cannula or room air after ketogenic diet initiation. Weight loss was initially a problem for the infants in Cases 1 and 2 but resolved with dietary adjustment. Obstacles to diet initiation were identified and included: anxiety of bedside nurses related to lack of familiarity with the ketogenic diet, concern by NICU physicians that use of the ketogenic diet would prevent infants from receiving appropriate nutrition and inappropriate treatment of hypoglycemia. Parents were generally positive in their perceptions related to the process of diet initiation and the outcomes of treatment. Conclusions: These cases demonstrate that of use of the ketogenic diet for treatment of neonatal encephalopathy and refractory epilepsy can be undertaken safely in the NICU and is well tolerated by carefully screened neonates and infants. In addition to improved seizure control, benefits to the infants included improved alertness and decreased need for respiratory support. Careful attention to education of all caregivers must be undertaken prior to and during use of the ketogenic diet.