Ketogenic dietary therapy for epilepsy and other disorders: current perspectives

Figures & data

Table 1 Characteristics of the different KD therapies

Diet type	Macronutrient prescription and food sources			Features	Suitability
	Fat	Protein	Carbohydrate
Classical KD	4:1 ratio; 90% of energy, long chain	4:1 ratio; 10% of energy from protein and carbohydrate combined. Protein provided from high biological value sources (eg, meat, fish, egg, cheese). Carbohydrate provided from controlled portions of vegetables or fruit		Strictly prescribed recipes Very restricted carbohydrates, limited protein intake Very high fat; portion sizes will appear smaller	Young children and poor eaters who need small meals and snacks Enterally fed patients as a complete formula feed is available Nutritionally at-risk as prescription very controlled
MCT KD	70%–75%	10%	15%–18%	MCT must be included at all meals and snacks and increased gradually during the diet initiation process to avoid gastrointestinal side effects	Children or adolescents who prefer a highcarbohydrate intake and are able to include the MCT supplement into their food and drink choices
	(30%–60% MCT)	Increased ketogenic potential of MCT allows less fat and a higher protein and carbohydrate intake. The diet can be more varied and include foods that provide both macronutrients, eg, meat and fish products, milk, yogurt
Modified Atkins diet	60%–65% High-fat food choices advised	30% No restriction on protein intake	5%–10% Very restricted carbohydrate intake	No food weighing Requires long-term control of dietary energy intake to prevent excess weight gain as no initial calorie prescription given	Families, adolescents, and adults who prefer a less-restrictive diet and who are able to design their own meals from given food choices
Low glycemic index treatment	60%	30% Generous protein allowance	10% Usually allows 40–60 g carbohydrate daily (including fiber) but must have glycemic index <50	Low glycemic index carbohydrates may have other health benefits Uses household measures rather than food weighing

Abbreviations: KD, ketogenic diet; MCT, medium-chain triglycerides.

Table 2 Medical contraindications to use of KD therapy

Definite contraindications

Primary carnitine deficiency

CPT I or CPT II deficiency

Carnitine translocase deficiency

Fatty acid β-oxidation defects

MCAD deficiency

LCAD deficiency

SCAD deficiency

Long-chain 3-hydroxyacyl-CoA deficiency

Medium-chain 3-hydroxyacyl-CoA deficiency

Pyruvate carboxylase deficiency

Disorders requiring a high-carbohydrate diet treatment, eg, acute intermittent porphyria

Hypoglycemia under investigation

Defects in ketoneogenesis or ketolysis

Severe liver disease

Possible contraindications (suitability to be assessed carefully by pediatric neurologist)

Familial hyperlipidemia

Dysphagia or significant gastroesophageal reflux (must be appropriately managed before starting KD therapy)

History of renal stones

Diabetes mellitus (may need medication adjustment)

On diuretics or medications that increase risk of acidosis

Concomitant steroid use (will limit ketone-generating ability)

Abbreviations: KD, ketogenic diet; CPT, carnitine palmitoyltransferase; MCAD, medium-chain acyl-dehydrogenase; LCAD, long-chain acyl-dehydrogenase; SCAD, short-chain acyl-dehydrogenase; CoA, coenzyme A.