Dietary Approaches to Non-Lesional Epilepsy December 8, 2013

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Dietary Approaches to Non-Lesional Epilepsy December 8, 2013 Eric H. Kossoff, MD Johns Hopkins University Baltimore, Maryland American Epilepsy Society Annual Meeting 1

Disclosures Nutricia, Inc. Atkins Nutritionals, Inc., NeuroPace, Inc. Grant Support Scientific Advisory Boards American Epilepsy Society 2013 Annual Meeting 2

Learning Objectives Recognize the different ketogenic diets available today for children and adults with epilepsy. Realize how different dietary therapies today are compared to several decades ago. Understand the true benefits (and risks) of these therapies for patients with non-lesional epilepsy. American Epilepsy Society 2013 Annual Meeting 3

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1921-2000: The Classic Ketogenic Diet Tried for all children, including lesional cases Started in the hospital gradually over 4-5 days after a fast Fluid and calorie-restricted Foods weighed on gram scales precisely Intensive dietitian involvement Minimum 6 months, maximum 2 years 5

Ketogenic Diet Studies Published 180 160 140 120 100 80 60 40 20 0

Masino & Rho 2012 7

Henderson J Child Neurol 2006 8

Randomized Controlled Study Randomize MCT keto diet Subject entry Classical keto diet Screening visit 4 weeks Controls 12 weeks Diet 12months Neal, et al. Lancet Neurol 2008

Slide courtesy of Dr. Helen Cross

6-Month Seizure Reduction from Ketogenic Diet Seizure-free 90-99% <50% 50-90% 11

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Kossoff et al., Epilepsia 2005 (updated 2013) 15

2013: A Gentler and Smarter Ketogenic Diet We know who does best Fasting and admission not required 16

Kossoff Epilepsia 2009 17

Surgery vs. Diet 45 children with lesional epilepsy 24 underwent surgery Surgery: 63% seizure-free KD: 0% seizure-free In general, the KD is used primarily in patients with generalized, non-lesional epilepsy Stainman R, et.al. Seizure 2007 18

Do we need to Fast? Good evidence of identical 3-month equivalency Wirrell 2002 Kim 2004 Vaisleib 2004 Bergqvist 2005 Bergqvist AG, et.al. Epilepsia 2005 19

Kossoff Epilepsia 2008 20

2013: A Gentler and Smarter We know who does best Ketogenic Diet Fasting and admission not required Adjust calories, ratio, fluids for growth and satiety NOT for seizure control www.ketocalculator.com Adverse effects prevented not just treated 21

Potential Side Effects Constipation Weight loss Vomiting Acidosis Kidney stones Growth slowing Hyperlipidemia Vitamin D deficiency Prolonged QT (selenium deficiency related) 22

Kossoff Epilepsia 2009 23

Kidney Stones and Oral Citrates Kidney stones occur in 6% historically NOT higher in those on topiramate or zonisamide Cytra-K (30 meq twice daily) Reduces risk of kidney stones on the KD from 6-fold when used empirically McNally et al. Pediatrics 2009 24

2013: A Gentler and Smarter We know who does best Ketogenic Diet Fasting and admission not required Adjust calories, ratio, fluids for growth and satiety NOT for seizure control www.ketocalculator.com Adverse effects prevented not just treated Minimum and maximum durations have changed 3 other diets available: MCT diet Modified Atkins Diet Low glycemic index treatment 25

How Long is Enough? Most studies show benefit within 2-4 weeks We suggest 3 months minimum Traditional duration is 2 years For infantile spasms, shorter durations have been reported Children/adults can remain on dietary therapy 20-30 years if necessary 26

The Modified Atkins Diet No calorie or fluid restriction No hospital admission (clinic education) No weighing of foods on gram scales No fasting required 27

MAD : Other Potential Advantages Less time needed for parent education Can be started urgently in the clinic Parents can do it along with their child Products available in stores 28

MAD at a Decade 32 publications from 2003-2013 17 prospective studies in children and adults 208 (48%) of 434 patients with >50% seizure reduction to date 55 (13%) became seizure-free 29

Low Glycemic Index Treatment Target carbohydrates with glycemic indicies <50 Similar composition to the MAD Does not induce urinary ketosis Reported recently as beneficial for tuberous sclerosis and Angelman syndrome Muzykewicz et al. Epilepsia 2009 30

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Diets for Adults Centers in Baltimore, Chicago, London, India, Washington (so far) Modified Atkins Diet probably the most straightforward to use Consider ketogenic diet for adults with gastrostomy tubes or in status epilepticus 33

Impact on Clinical Care and Practice Using diets can be very helpful, especially for patients with non-lesional epilepsy Consider earlier in the management of refractory epilepsy Safer and easier to use than decades ago Also of potential value for adults, especially with non-lesional epilepsy American Epilepsy Society 2013 Annual Meeting 34