Ketogenic Diet as Treatment Option for Infants with Intractable Epileptic Syndromes

Abstract

A special diet for those suffering from epilepsy such as the ketogenic diet (KD) is a successful alternative treatment option in young children when treatment with different combinations of anti epileptic drugs (AEDs) fails.

Appendices

Appendix 1: Diet Schedules for Infants

Example I: 9 Months Old (9 kg)

Bottle feeding/day

Ingredients/24 h

• 70 g Ketocal 3:1^®

• 12 mL Calogen^® neutral

• Water until 800 mL of feeding

Supplement

• 5 μg vitamin D solute into oil

Divided into

• 3  ×  200 mL

• 2  ×  100 mL

Breakfast	200 mL bottle feeding
Morning snack	Fruit at 2.5 g carbohydratea
	25 g crème fraiche (35 g/100 gram fat)
	100 mL bottle feeding
Afternoon	200 mL bottle feeding
Afternoon snack	Vegetables at 1 g carbohydratea
	5 g vegetable oil
	5 g fatty cheese (48 g fat/100 g)
	100 mL bottle feeding
Evening meal	200 mL bottle feeding

^aAccording to variation lists

Analysis
Calories per day	705	78 kcal/kg
Grams of protein	13	7 energy %
Grams of LCT fat	69	88 energy %
Grams of carbohydrates	9	5 energy %
Ratio	3:1

Example II: 9 Months Old (8.5 kg) with Decreased Resting Energy Expenditure

Bottle feeding/day

Ingredients/24 h

• 85 g Ketocal^® 3:1

• 710 mL water

Supplement

• 5 mg vitamin D solute in oil

Divided into

• 5  ×  150 mL

Extra

• 1  ×  100 mL water/sugar-free lemonade

Breakfast	150 mL bottle feeding
Morning	150 mL bottle feeding
	100 mL sugar-free lemonade
Afternoon	150 mL bottle feeding
Afternoon snack	Vegetables/cream/oil/ cheese calculated based on analysis of 150 mL bottle feedinga
	100 mL sugar-free lemonade
	NB: when the child eats only 50% of his or her meal, supplement 75 mL
	Bottle feeding
Evening	150 mL bottle feeding

^aAnalysis of 150 mL bottle feeding made of 17 g Ketocal^® 3:1:

119 kcal, 2.6 g protein, 12 g LCT fat, 1 g carbohydrate, ratio 3:1

Analysis
Calories per day	594	69 kcal/kg
Grams of protein	13	8 energy %
Grams of LCT fat	58	88 energy %
Grams of carbohydrates	6	4 energy %
Ratio	3:1

Appendix 2: Emergency Regimes in Case of Illness [8]

Use of ORS Junior

100 mL ORS Junior, prepared according to the information on the package, contains 22 g carbohydrate (2 sachets for 1 L)

• 1. 1 sachet ORS Junior can be diluted into 1,000 mL water and contains 11 g carbohydrate.

• 2. The amount of ORS Junior depends on the carbohydrate content of the KD and must be calculated individually.

• 3. In daily practice, in the case of the classic KD, a limited amount of ORS Junior can be used.

Illness, fever with vomiting or diarrhea
Phase I	Give 24 h ORS Junior according to the preceding guidelines and calculated based on weight and age of individual child
	Compensate each time vomiting and or diarrhea occurs with 10 mL ORS Junior/kg body weight
	Use diet composition indicated in individual emergency plan
Phase II	In case of bottle feeding
	Dilute bottle feeding 50–50 with water
	Give numerous, smaller portions throughout day
	In case of solid food
	Calculate meals at a lower ratio than the usual diet (2.5:1 when normal 3:1, or 3:1 when normal 4:1)
	Distribute the food over smaller portions
	Compensate each vomiting/diarrhea episode with 10 mL ORS Junior/kg body weight
	Use composition indicated in individual emergency plan
	Intake of solid foods may be limited and can be accepted if child continues to feed from bottle
Phase III	If complaints subside, a switch can be made to the regular diet
Illness, fever without vomiting and/or diarrhea
Phase I	In case of bottle feeding
	Dilute bottle feeding 50–50 with water
	Give numerous, smaller portions throughout day
	In case of solid food
	Calculate meals at a lower ratio than the usual diet (2.5:1 when normal 3:1, or 3:1 when normal 4:1)
	Distribute food over smaller portions
	Compensate each vomiting/diarrhea episode with 10 mL ORS Junior/kg body weight
	Use composition indicated in individual emergency plan
	Intake of solid foods may be limited and can be accepted if child continues to feed from bottle
Phase II	If complaints subside, a switch can be made to the regular diet
	If complaints do not subside, see advice under Phase I
	First 24 h can be continued for a maximum of 2 days

Glossary

Infant

Child <12 months of age.

Infantile spasms (IS): or West syndrome

A form of severe epilepsy presenting between 3 and 10 months of age characterized by a brisk flexion or extension of the extremities, sometimes followed by a brief tonic posture, appearing in clusters and often occurring after sleep transition and accompanied by developmental regression. Characteristically interictal EEGs reveal hypsarrhytmia, high-amplitude, chaotic background pattern, and asynchronous slow waves intermixed with multifocal spikes.

Intractable epileptic syndrome

General term covering epileptic syndromes (like Ohtarara syndrome and West syndrome) that do not respond to initial treatment with (multiple) anti epileptic drug regimes.

Ohtahara syndrome

Identical to West syndrome but presenting before 3 months of age.

Ketogenic diet (KD)

High-fat, low-carbohydrate diet with adequate amount of protein that mimics the metabolic state of fasting during an anabolic situation.

Due to the lack of carbohydrates energy metabolism shifts from carbohydrate to fat burning for energy, which induces production of ketone bodies (aceto acetate, β (beta)-hydroxybutyrate and acetone) that can be measured in urine and blood.

An adequate level of ketosis is defined as 3–4  +  (8–16 mmol/L) ketones in urine or 2–5 mmol/L ­ketones in blood.

Ketogenic ratio

The ratio of ketone-producing foods in the diet (i.e. dietary fat). The most used ratio’s are 3:1 or 4:1. This means either 3 or 4 g of ketone-producing fat vs. 1 g non-ketone-producing protein and carbohydrates.