Abstracts

Rationale: Drugs and other treatment options still leave a significant proportion of people with epilepsy with ongoing seizures. Can an acidosis-sparing euketonemic ketogenic diet (ASEK) better address many of the neurobiological factors that affect the likelihood of seizures occurring? Methods: To describe the clinical history of a person with severe epilepsy and the novel dietary changes to her management leading to dramatic improvement in control of her seizures. Results: RA is a 40 yr old female with subcortical band heterotopia. She had autism and seizures with multiple atypical and morphing seizure types which started when she was 9 months old. She was first hospitalized for status epilepticus (SE) at age 13 and was started on carbamazepine. Her seizures remained refractory on various AED combinations. She had 8 further admissions because of SE, the last SE and hospitalization occurring at age 33. RA started ketogenic dietary treatment at age 27. Her dietary management has evolved over a period of 13 years and she has had only occasional minor break-through seizures in the last 7 years generally associated with viral infections. EEG at age 35 was essentially normal. Primary treatment consists of phenytoin maintained at maximum therapeutic level, high dose levetiracetam, and ketogenic-based diet formulated for: reduction of low-grade metabolic acidosis, caloric and nutritional sufficiency, and protection of mitochondrial and peroxisomal beta-oxidation pathways. RA s CO2 content is 25 Meq/L (normal 22-31), beta hydroxybutyrate is 0.33 mM/L (normal up to 0.3), and triglycerides 0.67 mM/L (normal <1.7). Ancillary treatment consists of managing hormonal and psychological stressors, constipation, and ensuring quality of sleep. RA is in excellent health, and shows cognitive and social rehabilitation indicative of recovery from autistic dysfunction. Conclusions: This case history suggests the potential of a novel euketonemic diet to improve markedly seizure control and quality of life in people with refractory epilepsy. We are aware of a female aged 10 yrs and male aged 15 yrs with Lennox Gastaut syndrome, and a female 15 yrs with subcortical band heterotopia who have shown marked improvement in seizure control following ASEK principles.