Transcript Epilepsy and the Ketogenic Diet

Epilepsy and the Ketogenic Diet
BETH CECCARELLI
SODEXO DIETETIC INTERN
2015
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
Generalized
Partial
•
•
•
•
•
•
Absence
Myoclonic
Clonic
Tonic
Tonic-Clonic
Atonic
• Simple
• Complex
Epilepsy
Causes of Non-epileptic Seizures:
Brain tumors
Head injuries
Exposure to toxins
Brain infection
Etiology
Genetic
abnormalities
Structural
Head injuries
Tumors of the
Central
Nervous
System
Unknown
Diagnosis
Medical
History
Bloodwork
Brain
Imaging
Neurological
Exam
Seizure
History
Medical Treatment
Medication:
Surgery
Dietary
Medication
 Medication helps 70% of people with epilepsy
become seizure-free.
 Common Anti-Epileptic Drugs:
Dilantin, Keppra, Depakote
 Trial and error
 Side 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
Resection
Removal of the lobe where
the seizure originates

Hemispherectomy
Total or partial removal of the
affected brain hemisphere
Surgical
Disconnection
Corpus 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
Stimulation
Vagus 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-carbohydrate
 Four 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:
Cream, 56 g
Canola oil, 6
Fresh egg, 16 g
Crisp bacon, 5 g
Strawberries, 14 g
Meal 3:
Cream, 46 g
Canola oil, 4 g
Butter, 6 g
Spaghetti squash, 20 g
Cheddar cheese, 15 g
Meal 2:
Cream, 45 g
Canola oil, 4 g
Ranch dressing, 20 g
Iceberg lettuce, 16 g
Fresh spinach, 16 g
Grilled chicken breast, 9 g
Meal 4:
Sugar-free mayo, 32 g
Shredded chicken breast, 16 g
Dill pickles, 11 g
Red grapes, 11 g
120 g total fat
30 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-yearold
male
Functions
at a twoyear age
level
Goes to
a
special
school
Lives
with his
parents
and 8year-old
sister
No
family
history
of
seizures
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
Keppraseizures
improved
Regression
duPont
Background and Medical History
Ketogenic
Diet?
Started on a
series of
AED’s
EEG with
abnormal
results
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 2015
Blood Hematology
MCV
94.1 92.8
MCH
31.3
31.6
Absolute
1297
Monocytes
1540
Neutrophils
10,6154000
Blood Chemisty
BUN
x
14
Cholesterol, total
x
174
Non-HDL chol.
x
129
LDL
x
109
Prealbumin
x
21
Chemistry Enzymes
AST
15
28
ALT
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
Day 2
• 1/3 total
calories given
• KetoCal
formula
• 2/3 total
calorie needs
Days 3 and 4
• Full strength
diet
Patient admitted to duPont April 5, 2015
Inpatient Diet Initiation
Meal 1
Cream, 45 g
Applesauce. 6 g
Sausage, 55 g
Veg oil, 14 g
Meal 2
Cream, 45 g
Cooked Carrots, 22 g
Chicken breast, 23 g
Mayo, 37 g
Meal 3
Heavy Cream, 28 g
Strawberries, 10 g
Meal 4
Cream, 45 g
Cantaloupe, 12 g
American cheese, 10 g
Ham, 30 g
Butter, 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
discharge
Better
balance
More
alert
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/medicalconditions/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_cl
inics/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_cl
inics/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): 151155.
References
 The Charlie Foundation for Ketogenic Therapies. Classic
Ketogenic and Modified Ketogenic. 2014. Available at:
http://www.charliefoundation.org/explore-ketogenicdiet/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.