1. Ketogenic Diet & Epilepsy
Dr. Tamer Rizk
MD FRCPCH MBPNA(UK) MINA(USA) MICNA(SWE)
Consultant Paediatric Neurologist
Al-Takhassusi Hospital
Dr Sulaiman Al-Habib Medical Group

2. Overview Epilepsy Prevalence Main Goal
Biochemical changes with fasting
Ketoacidosis
Energy requirements & Calculations
Dietitian Aims
Specific Considerations
Key points
Steps to start KD in Epileptic Patient

3. Prevalence

4. Alter the Brain’s Fuel Source from
The Main Goal
Alter the Brain’s Fuel Source from
Glucose   FAT

5. Biochemical changes with fasting
It was not until the early twentieth century that medical use of the K.D. emerged as a strategy to mimic the biochemical effects of fasting (or starvation)
Two French physicians, Guelpa & Marie authored the first scientific report on the value of fasting in epilepsy; reported that seizures were less severe during treatment, but no details were given
Murray RK, Granner DK, Mayes PA, Rodwell VW (eds.). Harper’s Biochemistry, 24th ed., Vol. 29. Appleton & Lange, Norwalk, CT, 1996, p. 288.
Guelpa G, Marie A. La lutte contre l’épilepsie par la désintoxication et par la rééducation alimentaire. Rev Ther Medico–Chirurgicale 1911;78:8–13.

6. It is all about Ketoacidosis
Acetone, Acetic acid, and Beta-hydroxybutyric acid appear in a normal subject by:
Starvation
Diet containing too low carbohydrate and high proportion of fat.
Ketoacidosis appears to be the immediate result of the oxidation of certain fatty acids in the absence of a sufficient proportion of oxidizing glucose
Ketone bodies are formed from fat and protein whenever a disproportion exists between the amount of fatty acid and the amount of sugar actually burning in the tissues.
It is possible to provoke ketogenesis by feeding diets which are very rich in fat and low in carbohydrate.
Woodyatt RT. Objects and method of diet adjustment in diabetics. Arch Intern Med 1921;28:125–141.
Wilder RM. The effect on ketonemia on the course of epilepsy. Mayo Clin Bull 1921;2:307.

8. Efficacy of Fasting !

9. Peterman’s K.D.
Peterman first reported the calculation and effectiveness of K.D. from the Mayo Clinic in 1924.
Peterman K.D. composed of : (identical to what is currently used)
One gram of protein per kilogram of body weight
10–15 g of carbohydrate per day
The remainder of the calories in fat
Peterman also observed that excess ketosis could lead to nausea and vomiting, symptoms that were quickly relieved by orange juice.
Peterman MG. The ketogenic diet in epilepsy. JAMA 1925;84(26):1979–1983.
Peterman MG. The ketogenic diet in the treatment of epilepsy: a preliminary report. Am J Dis Child 1924;28:28–33.
Wheless JW. The ketogenic diet: an effective medical therapy with side effects. J Child Neurol 2001;16(9):633–635.

10. Behavior and Cognitive Effects
Peterman noted behaviour & cognitive effects of K.D:
“The mental development has been normal in all patients, and exceptionally good in seven of the twenty who are now free from attacks.
In all the children treated with the ketogenic diet there was a marked change in character, concomitant with the ketosis;
a decrease in irritability,
increased interest and alertness;
the children slept better
were more easily disciplined “This action of the diet warrants further study”
M. B. Pulsifer and others, 75 yr later, performed the first prospective study of the effects of the ketogenic diet on development and behavior.
Pulsifier concluded, “At follow-up, mean developmental quotient showed
statistically significant improvement (p < 0.05),
significant behavioural improvements in attention and social functioning,”
 verifying Peterman’s earlier observations.
Peterman MG. The ketogenic diet in epilepsy. JAMA 1925;84(26):1979–1983.
Pulsifer MB, Gordon JM, Brandt J, Vinig EPG, Freeman JM. Effects of the ketogenic diet on development and behavior: preliminary report of a prospective study. Dev Med Child Neurol 2001;43:301–306.

11. The Magic Treatment =
A New York lawyer & his wife were troubled because of the daily seizures of their son.
The boy’s uncle (Dr. John Howland) was a professor of paediatrics, but best medical treatment failed to help.
In despair, the parents turned to Dr. Hugh Conklin, a disciple of Bernar Macfadden, a physical cultist.
The treatment was called, euphemistically, a water diet. This meant starvation for three or four weeks. Dramatically, the boy’s seizures left him.
Lennox WG, Lennox MA. Epilepsy and Related Disorders, Vol. 2. Little, Brown, Boston, 1960, pp. 735–739.

12. The Magic Treatment =
A New York lawyer & his wife were troubled because of the daily seizures of their son.
The boy’s uncle (Dr. John Howland) was a professor of paediatrics, but best medical treatment failed to help.
In despair, the parents turned to Dr. Hugh Conklin, a disciple of Bernar Macfadden, a physical cultist.
The treatment was called, euphemistically, a water diet. This meant starvation for three or four weeks. Dramatically, the boy’s seizures left him.
Lennox WG, Lennox MA. Epilepsy and Related Disorders, Vol. 2. Little, Brown, Boston, 1960, pp. 735–739.

13. Is Ketosis maintained over the day?
Mayo Clinic investigators were the first to note daily variations in the intensity of ketosis, with a max in late afternoon & nadir in the early morning hours.
McQuarrie and Keith were aware of the recently re-recognized tendency of children to have seizures early in the morning, when ketosis is minimal, and they suggested the addition of a midnight snack to maintain early ketosis.
Degree of ketosis to prevent seizures vary across individuals, so adjusting diet for individual patient was necessary to ensure optimal ketosis. Such adjustments are routinely made in multidisciplinary epilepsy clinics.
Talbot FB. The ketogenic diet in epilepsy. Bull N Y Acad Med 1928;4:401–408.

14. Is Ketosis maintained over the day?
Ketone body concentrations elevated during the day, reached a maximum in the afternoon, and often were lower in the morning.
Measured urinary ketones reflected changes noted in the serum.
This led to the adoption of urine ketone body measurement as a standard method for determining the degree of ketosis on the KD.

16. KD can make a real change !
Thousands of seizures
Countless medications,
5 pediatric neurologists,
2 homeopathic physicians,
1 faith healer,
1 surgery,
Charlie’s seizures remained.
Prognosis: “Continued seizures & progressive retardation”
Charlie was brought to Johns Hopkins for initiation of KD, under the discretion of an experienced dietician.
Charlie initiated the diet, became seizure-free, and soon posted developmental progress.

17. Guide to energy requirements
<1 year kcal/kg
1-3 years kcal/kg
4-6 years kcal/kg
7-10 years kcal/kg
11 years kcal/kg
Always calculate according to ideal weight!

18. Calculations: Fat (1/10) 0.1000 x 1,350 = 135x9 = 1215 Protein (1/80)
The ketogenic ratio – 4:1
Calculate the calories needed/day (based on RDAs)
The following calculation is for a 2 yr. old child weighing 18kg.
Grams Calories
Fat (1/10)
x 1,350 =
135x9 = 1215
Protein (1/80)
x 1,350
16.87x4 =67.48
CHO (1/80)

19. Calculations:
Divide the total # of grams for fat, protein and carbohydrate by the # of meals in a day.
Example:
135.0/3 = 45 g fat
16.87/3 = g protein
16.87/3 = g carbohydrate
Not only does the caloric intake for the day have to be in a 4:1 ratio, but each meal must be 4:1

20. The dietician aims Ensure nutritional well being is not compromised
Ensure growth is adequate
Implement a dietary regimen that is:
Palatable
Possible to comply with

21. Considerations Age of child? What do they eat?
Are there any food fads or allergies?
Who prepares the food?
Is the food home cooked or purchased?
Are packed lunches required?
Any financial considerations?
Does the family understand the diet rigidity?

22. Key points Decide optimal calories from diet history
Decide ratio. it is best to start 2:1 ratio and increase over the week or slower to induce ketosis.
Some younger children become ketotic on lower ratios and if this is giving results then they can stay at the lower ratio.
Under 2 years and adolescents’ there is a tendency to use more 3:1 ratio.
The ratio may be altered by using orange juice as the CHO and then it can be gradually reduced until you have the desired ratio.

23. Fluids to maintenance level
1-10 kg 100ml/kg
11-20 kg 1000ml + 50ml for each kg >10kg
> 20 kg 1500ml 20ml/kg thereafter
Usually need % maintenance; high fluid intake can dilute ketone detection

24. Proteins & Vitamins
Protein requirements are usually 1g/kg, 0.8 (minimum req.)-1.5g/kg depending on age and requirements
Remember full vitamin and mineral supplementation is necessary.
Tend to use sugar free formulas.
Be guided by amount of Calcium needed first and set dose around that.
Intake can always be checked on Diet plan.

25. Typical foods / recipes
Butter
Mayonnaise
Heavy whipping cream
Olive oil
Canola oil
Eggs
Chicken
Ground beef
Hot dogs
Sausages
Tuna fish
Macadamia nuts
Almonds
Strawberries
Blueberries
Avocado
Cheese
Green vegetables
Lettuce
Mushrooms
Artificial sweeteners

26. Sample Meals: Meal 1: Meal 2: melted butter heavy whipping cream
chicken
apple
sugar free Jell-O
Meal 2:
Hot dog slices w/ sugar-free ketchup
Asparagus w/ butter
Chopped lettuce w/ mayo
Sugar-free vanilla cream popsicle

27. Maintenance of the Diet:
Medications are continued initially
Constant calculations!
BE VERY PRECISE!
Monitor ketone levels twice a day
Watch for cheating!
Special toothpaste
No added seasonings

29. Keto Pancakes Ingredients: 30gm Macadamia nuts - roasted
28gm Egg, raw - mixed well
6gm Oil - Grapseed, walnut or any vegetable oil
Optional: 3 drops Vanilla Extract
Directions:
1. Grind macadamia nuts in a blender until finely chopped.
2. Mix egg and oil into chopped macadamia nuts.
3. Optional: Add 3 drops Vanilla Extract
4. Spray a non-stick skillet with oil-spray.
5. Drop batter into desired size circles into heated pan. Turn over with a spatula when browned.

30. RaspberryCake Ingredients Grams carbohydrates
200 g grainy cream cheese, 20% fat ,6 g
40 g crème fraîche, 40% fat ,0 g
A pinch of sweetener ,0 g
20 g raspberries ,0 g
3 or 4 tablespoons of water ,0 g
3 sheets of white or red gelatine ,0 g
8,6 g
Sponge base ,3 g
Total ,9 g
Directions
Produce a round sponge base
Soak the gelatin
Beat the remaining ingredients until they are frothy
Press out the gelatine and dissolve it
Mix 2 or 3 tablespoons of the raspberry cream into the gelatine, then mix with the rest of the cream
Spread it on the sponge base (use a spring-form)
After solidified, take the raspberry cake out of the spring-form

31. Pumpkin Fries Preparation - Cut the pumpkin into French fries
- Warm the oil in a frying pan
- Bake the pumpkin fries in the heated corn oil

32. Omelets All Bran Preparation
• Mix all ingredients, except the peanut oil, together
• Warm the peanut oil in a frying pan
• Pour the mixture into the frying pan and bake it at both sides

33. Standardized protocols and management recommendations for Ketogenic Diet
In December 2006, The Charlie Foundation commissioned a panel comprised of 26 pediatric epileptologists and dieticians from nine countries with particular expertise using the KD.
This group was created in order to create a consensus statement regarding the clinical management of the KD.
Subsequently endorsed by the Practice Committee of the Child Neurology Society, issues such as:
Patient selection
Pre-KD counselling and Evaluation
Specific dietary therapy selection
Implementation, Supplementation & Follow-up
Adverse event monitoring & Management
Eventual KD discontinuation

34. How to Start ??
The KD was traditionally started in the hospital after a 48-h fast followed by a gradual introduction of calories in the form of a KD over a 3-day period.
Children to be seen periodically in clinic for medical and nutritional follow-up.

35. Patient selection
KD can effectively treat epilepsy in individuals from infancy through adulthood.
Regardless of age, seizure type, or aetiology, the KD appears to provide a third of the patients with >90% reduction in their seizure frequency.
KD should be offered to a child after two anticonvulsants are used unsuccessfully.
Henderson et al., 2006

37. Epilepsy syndromes and conditions in which the KD has been reported as particularly beneficial
Probable benefit:
Glucose transporter protein 1 (GLUT-1) deficiency
Pyruvate dehydrogenase deficiency (PDHD)
Myoclonic-astatic epilepsy (Doose syndrome)
Tuberous sclerosis complex
Rett syndrome
Severe myoclonic epilepsy of infancy (Dravet syndrome)
Infantile spasms
Children receiving only formula (infants or enterally fed patients)
Suggestion of benefit:
Selected mitochondrial disorders
Glycogenosis type V
Landau-Kleffner syndrome
Lafora body disease
Subacute sclerosing panencephalitis (SSPE)
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

38. Treatment of Choice
KD is the treatment of choice for two distinct disorders of brain energy metabolism:
GLUT1 deficiency syndrome
Pyruvate dehydrogenase deficiency (PDHD)
In GLUT1 deficiency syndrome, glucose transport across BBB is impaired; resulting in: seizures, GDD & complex movement disorder
KD should be considered as a first-line therapy for GLUT1 deficiency syndrome.
In PDHD, a severe mitochondrial disease with lactic acidosis and severe impairment, pyruvate cannot be metabolized into acetyl-CoA).
In both disorders, the KD provides ketones that bypass the metabolic defect and serve as an alternative fuel to the brain.
Klepper & Leiendecker, 2007
Wexler et al., 1997

39. Contraindications to the use of the KD
Absolute:
Carnitine deficiency (primary)
Carnitine palmitoyltransferase (CPT) I or II deficiency
Carnitine translocase deficiency
b-oxidation defects
Medium-chain acyl dehydrogenase deficiency (MCAD)
Long-chain acyl dehydrogenase deficiency (LCAD)
Short-chain acyl dehydrogenase deficiency (SCAD)
Long-chain 3-hydroxyacyl-CoA deficiency
Medium-chain 3-hydroxyacyl-CoA deficiency.
Pyruvate carboxylase deficiency
Porphyria
Relative:
Inability to maintain adequate nutrition
Surgical focus identified by neuro-imaging & VEEG monitoring
Parent or caregiver noncompliance
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

40. Sankar & Sotero de Menezes, 1999
When to suspect IEM?
Clinical suspicion about IEM includes:
Developmental delay
Cardiomyopathy
Hypotonia
Exercise intolerance
Myoglobinuria
Easy fatigability
The presence of one of those clinical features suggests that the child should be tested to rule out an IEM prior to KD initiation.
Sankar & Sotero de Menezes, 1999

41. Pre-KD Evaluation Counseling
Discuss seizure reduction, medication, and cognitive expectations
Identify potential psychosocial barriers to the use of KD
Review anticonvulsants and other medications for carbohydrate content
Recommend family read parent-oriented KD information
Nutritional evaluation
Baseline weight, height, and ideal weight for stature
Body mass index (BMI) when appropriate
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

42. Pre-KD Evaluation Nutrition intake history:
3-day food record
Food preferences
Allergies
Aversions
Intolerances
Establish diet formulation: infant, oral, enteral, or a combination
Decision on which diet to begin (MCT, classic, modified Atkins, or low glycemic index)
Calculation of calories, fluid, and ketogenic ratio (or percentage of MCT oil)
Establish nutritional supplementation products based on dietary reference intake
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

43. Pre-KD Evaluation (Labs)
Laboratory evaluation:
Complete blood count with platelets
Electrolytes to include serum bicarbonate, total protein, calcium, zinc, selenium, magnesium, and phosphate
Serum liver and kidney tests (including albumin, AST, ALT, blood urea nitrogen, creatinine)
Fasting lipid profile
Serum acylcarnitine profile
Urinalysis
Urine calcium and creatinine
Anticonvulsant drug levels (if applicable)
Urine organic acids
Serum amino acids
Ancillary testing (optional):
Renal ultrasound and nephrology consultation (if history of kidney stones)
EEG
MRI
Cerebrospinal fluid (if no clear aetiology has been identified)
ECG (if history of heart disease)
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

44. LCT or MCT
MCT oils yield more ketones per kilocalorie of energy than their long chain counterparts; they are absorbed more efficiently and carried directly to the liver.
This increased ketogenic potential means less total fat is needed in the MCT diet, thus allowing inclusion of more carbohydrate and protein.
Data from studies 20 years apart now suggest no difference in efficacy between the two diets if applied appropriately in a calculated fashion.
There may be some differences in tolerability but this did not reach statistical significance in a recent randomized controlled trial with direct comparison between the two.
Schwartz et al., 1989
Neal et al., in press

45. How to Start ??
The traditional method of initiating the KD involves a period of fasting, with no carbohydrate-containing fluids provided, and serum glucose monitored periodically.
The duration of fasting varies from 12 h to ‘‘when urine ketones are large,’’ which can be longer than 48 h. Children should not be fasted longer than 72 h.
The meals are then typically advanced daily in one-third caloric intervals until full calorie meals are tolerated, while keeping the KD ratio constant.
Another approach begins with full calories, but the KD ratio increases daily from 1:1, 2:1, 3:1, to 4:1 to allow the patient to acclimate to the increasing concentration of fat.
Freeman et al., 2006
Bergqvist et al., 2005

46. Red Flags !!!
Valproic acid should not be used with KD; for idiosyncratic side effects of valproic acid (i.e., hepatotoxicity) . It is a short-chain fatty acid, enhancing fatty acid oxidation & increase the risk of hepatotoxicity.
Despite such fears, recent clinical evidence supports the safe use of valproic acid and the KD. It does appear that secondary carnitine deficiency, which can occur with either the KD or valproic acid alone, can be worsened.
Bergqvist et al., 2005
Lyczkowski et al., 2005
Coppola et al., 2006
Takeoka et al., 2002

47. Red Flags !!!
The KD is also known to cause a transient but often clinically asymptomatic Metabolic Acidosis. Carbonic anhydrase inhibitors (Topiramate & Zonisamide) may in fact worsen preexisting metabolic acidosis, but the greatest decreases in serum bicarbonate levels occur early after initiation of the diet.
It is recommended that bicarbonate levels should be monitored carefully, especially when receiving these anticonvulsants, and that bicarbonate supplements be given when patients are clinically symptomatic (vomiting, lethargy).
Bergqvist et al., 2005
Lyczkowski et al., 2005
Coppola et al., 2006
Takeoka et al., 2002

48. Supplementations Universal recommendations:
Multivitamin with minerals (and trace elements)
Calcium with vitamin D
Optional extra supplementations:
Oral citrates (Polycitra K)
Laxatives: Movicol, mineral oil, glycerine suppository
Additional selenium, magnesium, zinc, phosphorus, vitamin D
Carnitine
MCT oil or coconut oil (source of MCT)
Salt (sodium to add to modular formulas if used for > age 1 year)
All supplements listed should be provided as carbohydrate free preparations whenever possible.
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

49. Follow-up KD Clinic Visit
Nutritional assessment (Dietitian)
Obtain height weight, ideal weight for stature, growth velocity, BMI when appropriate
Review appropriateness of diet prescription (calories, protein, and fluid)
Review vitamin and mineral supplementation based on dietary reference intake guidelines
Assess compliance to therapy
Adjust therapy if necessary to improve compliance and optimize seizure control
Medical evaluation (Neurologist)
Efficacy of the diet (is the KD meeting parental expectations?)
Anticonvulsant reduction (if applicable)
Should the KD be continued?
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

50. Follow-up KD Clinic Visit
Laboratory assessment:
Complete blood count with platelets
Electrolytes to include serum bicarbonate, total protein, calcium, mg & phosphate
LFT & RFT (including albumin, AST, ALT, blood urea nitrogen, creatinine)
Fasting lipid profile
Serum acylcarnitine profile
Urinalysis
Urine calcium and creatinine
Anticonvulsant drug levels (if applicable)
Optional:
Serum b-hydroxybutyrate (BOH) level
Zinc and selenium levels
Renal ultrasound
Bone mineral density (DEXA scan)
EEG
Visits should be at least every 3 months for the first year of the KD
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

51. Adverse Effects of the KD
Metabolic abnormalities are relatively minor side effects of the KD include:
Hyperuricemia (2%–26%),
Hypocalcaemia (2%),
Hypomagnesaemia (5%),
Decreased amino acid levels
Acidosis (2%–5%)
Gastrointestinal symptoms occur in 12%–50% of children including :
Vomiting
Constipation
Diarrhoea
Abdominal pain
Schwartz et al., 1989, Chesney et al., 1999 , Kang et al., 2004, Kang et al., 2004, Berry-Kravis et al., 2001
Chesney et al., 1999; Kwiterovich et al., 2003; Kang et al., 2004
Furth et al., 2000; Kossoff et al., 2002a; Sampath et al.,2007

52. Adverse Effects of the KD
Hypercholesterolemia has been reported in 14%–59% of children on KD .
Renal calculi occur in 3%–7% of children on the KD. Stone composition includes:
Uric acid 50%
Calcium oxalate
Calcium phosphate
Mixed calcium/uric acid stones.
They typically do not require diet discontinuation and lithotripsy is only rarely necessary.
Polycitra K appears to help prevent stone formation .
Eric H. Kossoff et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2):304–317, 2009

53. Adverse Effects of the KD
Carnitine deficiency has been demonstrated.
Cardiac abnormalities anecdotally reported, including :
Cardiomyopathy
prolonged QT interval
Pancreatitis has been reported.
The long-term complications in children maintained on KD for greater than 2 years have not been systematically reviewed; there is only one report in the literature looking at this small subgroup.
Best et al., 2000; Bergqvist et al., 2003; Kang et al., 2004
Stewart et al., 2001; Kang et al., 2004
Groesbeck et al., 2006

54. KD discontinuation
KD should be used for at least a mean of 3.5 months (SD 2.2 months) before considering discontinuation.
Recent data suggests that the KD works rapidly when effective, with 75% of children responding within 14 days, so shorter KD durations may be adequate to assess efficacy.
Should seizures worsen for more than a few days after starting the KD, similar to anticonvulsants, it could be discontinued immediately.
If a family chooses to remain on the KD for longer than 6 months despite no apparent seizure control, the decision is ultimately their own and should be supported.
In children with >50% seizure response, the KD is often discontinued after approximately 2 years; however, in children in whom seizure control is nearly complete (e.g., >90% seizure reduction) and side effects are low, the diet has been reported as helpful for as long as 6–12 years.
Kossoff et al., 2008
Groesbeck et al., 2006

55. How to discontinue?
Although the diet can be discontinued abruptly in an emergency, typically in ICU, it is more often tapered slowly over 2–3 months by gradually lowering the ketogenic ratio from 4:1 to 3:1 to 2:1, then ketogenic foods are continued, but calories and fluids are increased ad libitum.
Once urinary ketosis is lost, high carbohydrate foods can be reintroduced. (This recommendation is based on traditional practice patterns, and mimics the several week gradual wean of anticonvulsant drugs)
During this time period, continued nutritional supplementation is recommended.
If seizures worsen, the KD can be increased to the previously effective formulation. In the majority, seizure control can once again be attained with either KD or anticonvulsants.
Freeman et al., 2006
Martinez et al., 2007

56. The Dietician’s role:
Developing the calculated dietary requirements based on the ketogenic ratio
Communicate possible risks
Communicate the precision required for success
To aid in achieving the goal – freedom from epileptic seizures
Determine the appropriate supplements
Teach parents how to cook, calculate and watch for hidden carbohydrate in all foods and medications.

57. Different start with similar results!
Prospective data indicating that fasting is not necessary for achievement of ketosis, and that gradual initiation protocols offer the same seizure control at 3 months with significant lower frequency and severity of initiation related side effects.
In addition, weight loss, hypoglycemia, and acidosis were less common when children were not fasted in this study
Vomiting did not differ in the two protocols, but intravenous fluids for dehydration were more commonly needed in the fasting group.
Bergqvist et al., 2005

58. Take Home Messages
The KD should be strongly considered in a child who has failed two to three anticonvulsant therapies, regardless of age or gender, and particularly in those with symptomatic generalized epilepsies.
It can be considered the treatment of choice for two distinct disorders of brain metabolism, GLUT-1 deficiency syndrome and PDHD.
In the particular epilepsy syndromes of Dravet syndrome, infantile spasms, myoclonic-astatic epilepsy, TSC, KD could be offered earlier.
KD is probably only of limited benefit in children who are candidates for epilepsy surgery.
Before starting KD, IEM that could lead to a severe metabolic crisis should be ruled out (disorders of fatty acid mitochondrial transport, b-oxidation, and other mitochondrial cytopathies).