BETH CECCARELLISODEXO DIETETIC INTERN

2015

Epilepsy and the Ketogenic Diet

Overview

Anatomy & Physiology Epilepsy

Diagnosis and

Treatment

Ketogenic Diet

Presentation of the Patient

Anatomy & Physiology

Anatomy & Physiology

Epilepsy

A neurological condition that affects the nervous system

Electrical activity in the brain becomes disturbed - these disturbances result in seizures

Seizures are events marked by symptoms like loss of consciousness, muscle jerks and spasms, change in mood, demeanor or emotion and/or staring spells.

Epilepsy

How is it diagnosed? - When an individual has 2 or more seizures more than 24 hours apart that are not provoked by other factors or - The diagnosis of an epilepsy syndrome

Everyone who has epilepsy has seizures but not everyone who has seizures has epilepsy

Types of Seizures

• Absence• Myoclonic• Clonic• Tonic• Tonic-Clonic• Atonic

Generalized

• Simple• ComplexPartial

Epilepsy

Brain tumors

Head injuries

Exposure to toxins

Brain infection

Causes of Non-epileptic Seizures:

Etiology

Genetic abnormaliti

esStructural Head

injuries

Tumors of the Central

Nervous System

Unknown

Diagnosis

Brain Imaging

Medical History

Seizure History

Neurological Exam

Bloodwork

Medical Treatment

Medication: Surgery Dietary

Medication

Medication helps 70% of people with epilepsy become seizure-free.

Common Anti-Epileptic Drugs: Dilantin, Keppra, DepakoteTrial and errorSide effects: Weight gain, fatigue, dizziness, depression, memory and thinking problems

Surgical

Surgery may be the best option for the other 30% of individuals with intractable epilepsy

Indicated when there is a seizure origination site or lesion

Types: Resection, Hemispherectomy Disconnection Vagus Nerve Stimulation

Surgical

ResectionRemoval of the lobe wherethe seizure originates

Hemispherectomy Total or partial removal of the affected brain hemisphere

Surgical

DisconnectionCorpus callostomy: A separation of the two hemispheres of the brain which severs the neuronal connections between the right and left halves of the brain

Surgical

StimulationVagus Nerve stimulation (VNS): Prevents seizures by sending pulses of electrical energy to the brain via the vagus nerve

Dietary Treatment

Ketogenic Diet

For patients with intractable epilepsy who do not respond to medications or when surgery is not an option.

Evidence based success: 50% seizure reduction in 1/2 of children 90% seizure reduction in 1/3 10-15% became seizure free

Ketogenic Diet

High-fat, low-carbohydrateFour variations: classic ketogenic diet (KD),

medium chain triglyceride diet (MCT), modified Atkins diet (MAD), low-glycemic index therapy (LGIT)

Ketogenic Diet

Fat provides 80%-90% of total calories 10%-20% from protein and carbohydrates (Fat mainly comes from heavy cream, butter and oils)

Uses a 3:1 or 4:1 ratio of fat to protein + carbs

Ketogenic Diet

Mechanism: - remains unknown - research is looking into the role of glucose during seizures, the role of GABA (gamma-Aminobutyric acid) and the neuroprotective effects of the diet.

What we do know: - Fat is converted into ketones which become the body’s main fuel source

Ketogenic Diet

Usually started on a three month trial and continued for up to three years if seizures have slowed down or stopped

Most children see improvement within the first 10 weeks of starting the diet

What is required: - A 3-4 day hospital stay for initiation - All nutrient requirements must be calculated and weighed on a gram scale - No generic brand products can be used - Extensive education - vitamin and mineral supplementation - frequent urine ketone checks

Ketogenic Diet

Meal 1: Meal 3:Cream, 56 g Cream, 46 gCanola oil, 6 Canola oil, 4 gFresh egg, 16 g Butter, 6 gCrisp bacon, 5 g Spaghetti squash, 20 gStrawberries, 14 g Cheddar cheese, 15 g

Meal 2: Meal 4:Cream, 45 g Sugar-free mayo, 32 gCanola oil, 4 g Shredded chicken breast, 16 gRanch dressing, 20 g Dill pickles, 11 gIceberg lettuce, 16 g Red grapes, 11 gFresh spinach, 16 gGrilled chicken breast, 9 g

120 g total fat30 g protein + carbs

Ketogenic Diet

Side effects:

Constipation

Reflux

Kidney Stones

Dehydration

High triglycerides

Nausea

Lethargy

Vomiting

Presentation of the Patient

Greg Smith* (GS) Admitted to Nemours Alfred I. duPont Hospital for Children April 5, 2015 to begin Ketogenic Diet therapy

Social History

6-year- old

male

Functions at a

two-year age level

Goes to a

special school

Lives with his

parents and

8- year-old

sister

No family history

of seizure

s

Background and Medical History

Developmental delays at 8-9 months of age

Referred to CHOP’s neurology and early intervention prgram

Diagnosed with hypotonia

Seizures begin- get worse from 8 months to 1 year of age

Background and Medical History

Subclinical seizures

Full body seizures during sleep

Staring spells 1 ½ - 2 minutes

Quick, sudden jerks and head droopiness for 15-20 minutes at a time

Background and Medical History

Diagnised with a

myoclonic seizure disorder

Started on Keppra- seizures improved

Regression duPont

Background and Medical History

EEG with abnormal results

Started on a series of AED’s

Ketogenic Diet?

Active Problem List

Delay in development

Encephalopathy

Other convulsions

Lack of coordination

Verbal dyspraxia

Delayed Milestones

Seizure disorder, nonconvulsive, with status epilepticus

Labs

January 2015 February 2015Blood HematologyMCV 94.1é 92.8é MCH 31.3é 31.6é Absolute 1297éMonocytes 1540é Neutrophils 10,615é 4000 Blood ChemistyBUN x 14Cholesterol, total x 174éNon-HDL chol. x 129éLDL x 109Prealbumin x 21Chemistry EnzymesAST 15ê 28ALT 8 13

Metabolic Acylcarnities *results are within normal limits and do not show a disorder of fatty acid oxidation which would be inhibit the use of ketogenic diet therapy

Medications

Valproic Acid (Depakote)Diastat Acudial (Diazepam)Topiramate (Topomax)Clobazam (Onfi)Divalproex Sodium (Depakote Sprinkles)

Assessment

Outpatient visit with dietitian

Dec. 2015• 3-day diet record• 1,500 kcals per day

Height/Weight

• 24.3 kg• BMI 16.6• 82nd %tile BMI-for-age • IBW 22.5 kg – 108%

Assessment

Energy Needs: WHO equation

Activity factor 1.4

Energy requirements: REE for males ages 3-10 years: REE = 22.7 x wt + 495 = 22.7 x (22.5 kg) + 495 = 1,046 kcals x activity factor 1.4 = 1,465 kcals

Assessment

Protein and Fluid Needs:

Protein requirements: Recommended protein needs for a six-year old are 0.95 g/kg of IBW Protein = 0.95 g/kg x 22.5 kg = 21 g protein per day

Fluid requirements: Over 20 kg = 1500 mL plus 20 mL for each kg over 20 kg ( = 1500 + 80 mL (using actual body weight) = 1580 mL fluid for normal maintenance

Inpatient Diet Initiation

Day 1

• 1/3 total calories given

• KetoCal formula

Day 2

• 2/3 total calorie needs

Days 3 and 4

• Full strength diet

Patient admitted to duPont April 5, 2015

Inpatient Diet Initiation

Meal 1Cream, 45 gApplesauce. 6 gSausage, 55 gVeg oil, 14 g

Meal 2Cream, 45 gCooked Carrots, 22 gChicken breast, 23 gMayo, 37 g

Meal 3Heavy Cream, 28 gStrawberries, 10 g

Meal 4Cream, 45 gCantaloupe, 12 gAmerican cheese, 10 gHam, 30 gButter, 32 g

Inpatient Diet Initiation

Three days of education to parents - Weighing food, proper products to use, food preparation, sick days

Supplements: - Powdered multivitamin supplement 2 times per day; fluoride drops

Fluid - 50 oz. of fluid per day to prevent dehyration

Follow-Up

Eating well weight

stable

2 seizures

since dischar

ge

More alert

Better balanc

e

Improved speech speaking

more clearly and more

approproately for his age

THANK YOU!

References

Epilepsy Foundation. About Epilepsy: The Basics. 2014. Available at: http://www.epilepsy.com/learn/about-epilepsy-basics. Accessed March 15, 2015.

  Epilepsy Foundation. Epilepsy Statistics. 2014. Available at:

http://www.epilepsy.com/learn/epilepsy-statistics. Accessed March 15, 2015.

Seattle Children’s Hospital Research Foundation: Epilepsy. 2014. Available at: http://www.seattlechildrens.org/medical-conditions/brain-nervous-system-mental-conditions/epilepsy/. Accessed March 23, 2015.

Fisher, Robert S., et al. "ILAE official report: a practical clinical definition of epilepsy." Epilepsia 55.4 (2014): 475-482.

References

Modi, Avani C., et al. "Treatment factors affecting longitudinal quality of life in new onset pediatric epilepsy." Journal of pediatric psychology 36.4 (2011): 466-475.

Nelms, M. et al. Nutrition Therapy and Pathophysiology, 2nd ed.

John’s Hopkins Medicine. Neurology and Neurosurgery. Available at: http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/epilepsy/seizures/types/complex-partial-seizures.html. Accessed March 3, 2015.

Abad, Rocío Sánchez-Carpintero, Francesc X. Sanmartí Vilaplana, and José María Serratosa Fernandez. "Genetic causes of epilepsy." The neurologist 13.6 (2007): S47-S51.

 

References

Brodie, Martin J. "Diagnosing and predicting refractory epilepsy." Acta Neurologica Scandinavica 112.s181 (2005): 36-39.

John’s Hopkins Medicine. Neurology and Neurosurgery. Available at: http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/epilepsy/treatment/surgery/corpus_callosotomy_surgery.html. Accessed March 3, 2015.

Neal, Elizabeth G., et al. "The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial." The Lancet Neurology 7.6 (2008): 500-506.

Yudkoff, Marc, et al. "Ketogenic diet, brain glutamate metabolism and seizure control." Prostaglandins, leukotrienes and essential fatty acids 70.3 (2004): 277-285.

References

Masino, Susan A., and Jong M. Rho. "Mechanisms of ketogenic diet action."Jasper's Basic Mechanisms of the Epilepsies 4 (2012).

Auvin, Stéphane. "Should we routinely use modified Atkins diet instead of regular ketogenic diet to treat children with epilepsy?." Seizure 21.4 (2012): 237-240.

Dressler, Anastasia, et al. "Long-term outcome and tolerability of the ketogenic diet in drug-resistant childhood epilepsy—The Austrian experience." Seizure19.7 (2010): 404-408.

Miranda, Maria J., et al. "Danish study of a modified Atkins diet for medically intractable epilepsy in children: can we achieve the same results as with the classical ketogenic diet?." Seizure 20.2 (2011): 151-155.

References

The Charlie Foundation for Ketogenic Therapies. Classic Ketogenic and Modified Ketogenic. 2014. Available at: http://www.charliefoundation.org/explore-ketogenic-diet/explore-2/classic-ketogenic. Accessed March 28, 2015.

 Richardson, Candy and Beth Zupec-Kania. Introduction

to Ketogenic Diet Therapy: A Primer for Hospital Employees (powerpoint slides), 2014.