beth ceccarelli sodexo dietetic intern 2015 epilepsy and the ketogenic diet
TRANSCRIPT
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.