Intravenous initiation and maintenance of ketogenic diet: Proof of concept in super-refractory status epilepticus

Abstract

Super-refractory status epilepticus (SE) is characterized by unresponsiveness to initial anaesthetic therapy. This definition encompasses a SE that continues or recurs 24 h or more after the onset of anaesthetic therapy and includes those cases in which SE recurs on the reduction or withdrawal of anaesthesia. Treatment is difficult due to the failure of firstand second-line therapy. As no controlled or randomized studies are available on this phenomenon, further therapeutic management is based on clinical reports and expert opinion. The ketogenic diet (KD) is a high-fat, low-carbohydrate and adequate protein diet that has been an established, effective nonpharmacological treatment in the management of refractory epilepsy and severe childhood encephalopathies since the early 1920s. The KD alters the primary cerebral energy metabolism from glucose to ketone bodies and probably has multiple mechanisms of antiepileptic action. Emergency use of KD has been reported predominantly in children with SE. In adults only five case reports on prolonged SE have been published. The KD should probably be tried in all severe cases of super-refractory SE. Patients usually receive a commercial enteral KD preparation (4:1 or 3:1 ketogenic ratio: grams of fat to protein and carbohydrates combined), which is usually administered via a gastric or gastrostomy tube, provided the patient tolerates tube feeds well.