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Ketogenic diet Jason Doescher, MD Pediatric Neurology & Epilepsy Minnesota Epilepsy Group, PA Saint Paul, Minnesota 651.241.5290 Minnesota Epilepsy Group, P.A.
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Ketogenic diet ADK (adenosine kinase stain) Li et al. J Clin Invest. 2008;118(2):571–582. Discussion goals: Why: ketogenic diet in epilepsy therapy What: ketogenic diet mechanism Who: syndrome & seizure types Where does it get you: results How: strategy for tolerance & goals
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Understand pathology / understand diverse therapy Seizure: abnormal neuronal & inter- neuron connections leading to recurrent imbalance in excitation & inhibition altering normal function & signaling Molecular: Genes proteins, neurotransmitters; toxins, electrolytes, energy/metabolism Cellular: Channels, receptors; neurons, astroglia Neuronal networks: Malformations of cortical development, inflammation, neoplasia, mesial temporal sclerosis/gliosis, trauma
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Therapeutic strategy: think pathology & review results Minnesota Epilepsy Group, P.A.
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Newly Diagnosed Epilepsy (n=470) Seizure-free 47% (n=221) Uncontrolled Seizures 53% (n=245) Seizure-free 13% (n=32) Uncontrolled Seizures 40% (n=100) Seizure-free 1% (n=1) Uncontrolled Seizures 39% (n=39) Seizure-free 3% (n=1) Uncontrolled Seizures 36% (n=14) Monotherapy 1st AED Monotherapy 2nd AED Monotherapy 3rd AED Adjunctive Therapy AED efficacy: 64% total seizure free (301/470) Ref: Brodie & Kwan, Neurology Vol 58, Number 8, April 23, 2002 Minnesota Epilepsy Group, P.A.
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Ketogenic diet: place in therapy Anti-epileptic medications provide approximately 60-75% of patient’s with seizure control If patient fails 3 medication trials, only 1-15% will achieve seizure control with additional medication combinations Surgery: 50-90% of appropriate epileptogenic zone resection candidates attain seizure control, but only 5-15% total qualify Minnesota Epilepsy Group, P.A.
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KETOGENIC DIET
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Metabolism map
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Metabolism review: Typical diet: glucose pyruvate acetyl CoA Ketogenic diet: fatty acid acetyl CoA
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Mitochondria uses ‘citric acid cycle’ to make ATP. Acetyl CoA is common substrate of both fatty acid & glucose metabolism. Minnesota Epilepsy Group, P.A.
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Ketogenic diet ‘transition of energy production from carbohydrates to fats’ Effectiveness depends on the degree of carbohydrate restriction & maintenance of ketone level (BHB goal 3-4) Fat: carbohydrate + protein ratio of 3:1 - 4:1 3:1 diet has long-chain saturated fat (87% calories), carbohydrate (6%), protein (7%) Minnesota Epilepsy Group, P.A.
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Ketogenic diet Mechanism of action: metabolism v anti- epileptic properties of ketones/fatty acid metabolites – Ex. Neurosteroids on GABA-a receptors Ref: Benaroch EE. Neurology 2007;68(8):612-614.
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Ketogenic diet: therapy
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Seizure & syndrome types: typically responsive to ketogenic diet Seizure types: Atypical absence (refractory) Atonic – myoclonic atonic * Tonic Myoclonic Syndromes: Myoclonic atonic epilepsy – Doose syndrome Lennox-Gastaut syndrome Glucose transporter deficiency syndrome Pyruvate dehydrogenase deficiency Metabolic/mitochondrial encephalopathies – Caution to exclude fatty oxidation disorder Intractable focal or generalized epilepsy Dravet syndrome Minnesota Epilepsy Group, P.A.
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Ketogenic diet: results
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In 1923, Conklin treated epilepsy by prescribing “rest of gastrointestinal system” for 25 days. He reported 90% ‘success’ rate in children and 50% in adults. In 1921, Wilder introduced the ketogenic diet. Later reported 95% of patients showing improvement through treatment. Minnesota Epilepsy Group, P.A.
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Ketogenic diet: results Maydell (2001 USA): classic diet 4:1 ratio over 12 months, 143 patients – Seizure free: 16% – >90% seizure reduction: 27% – >50% seizure reduction: 38% Kang (2005 Korea): 199 patients – Seizure free: 25% – >50% seizure reduction: 41% Minnesota Epilepsy Group, P.A.
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Ketogenic diet: results In children, composite of many studies… Seizure free at 6 months15% >90% seizure control: 37% 90-50% seizure control: 30% <50% seizure control: 33% Keene 2006 meta-analysis Minnesota Epilepsy Group, P.A.
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EEG : before ketogenic diet
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Normal EEG : after ketogenic diet
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Keys to success: patience, consistency & teamwork Team Dietician Parent/Caregiver Patient – G-Tube possible Physician/Nurse Minnesota Epilepsy Group, P.A.
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Ketogenic Diet: application Careful patient selection: exclude fatty acid oxidation disorder – Lab: Carnitine & acyl-carnitine profiles; urine organic acids Gradual introduction (5-7 days), no fasting, no fluid restriction Ensure energy & growth Follow acidosis, glucose, & electrolytes (Na) Supplements: vitamins (D, B) & minerals (Calcium, Selenium, Zinc) Caution: – Depakote (follow liver function & carnitine stores) – Acetazolamide, topiramate & zonisamide (acidosis & kidney stones) – “Syrups & Elixirs” – sugar/carbohydrates KNOW THE CARBOHYDRATE CONTENT!! ‘2 week honeymoon’ then advance ratio Minnesota Epilepsy Group, P.A.
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Risks & complications Growth delay & reduction Hyperlipidemia Cardiac dysfunction Kidney stones Disordered bone metabolism Bone marrow dysfunction Liver dysfunction Minnesota Epilepsy Group, P.A.
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Alternatives Modified atkins diet Low glycemic index diet Minnesota Epilepsy Group, P.A.
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Ketogenic diet Why: role ketogenic diet in epilepsy therapy – Roughly 1 in 4 patients need new strategy beyond medications & surgery What: ketogenic diet mechanism – Shift in energy metabolism with direct or indirect anti-epileptogenic properties Who: syndrome & seizure types – Select patients & others who fail medication trials or who are not surgical candidates Where does it get you: results – 15% seizure freedom, 30% excellent results, 30% modest improvement How: strategy for tolerance & goals – Patience, consistency & teamwork Minnesota Epilepsy Group, P.A.
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Thank You Jason Doescher, MD Pediatric Neurology & Epilepsy Minnesota Epilepsy Group, PA Saint Paul, Minnesota 651.241.5290 Minnesota Epilepsy Group
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