[Introduction].
The ketogenic diet (ketogenic diet) is a high-fat, low-carbohydrate and appropriate protein diet. Although its antiepileptic mechanism is not fully understood, its effectiveness and safety are well established and it can effectively treat many types of epilepsy in children and adults, such as: spastic seizures, akathisia, myoclonic seizures, generalized tonic seizures, generalized clonic seizures, generalized tonic-clonic seizures, focal seizures, and secondary generalized seizures. It may be most effective for spastic seizures, myoclonic seizures, atonic seizures or sudden collapse seizures, and atypical aphasic seizures. Jianmin Zhong, Department of Neurology, Jiangxi Children’s Hospital Currently, ketogenic therapy is more widely available worldwide, with approximately 80 centers in 45 countries offering ketogenic diet therapy. Over the past decade, many studies of the ketogenic diet have demonstrated its efficacy and safety. Approximately 50% of children remain on the diet for at least one year, and about half of these children experience a 50% or greater reduction in seizures, 30% experience a 90% or greater reduction in seizures, and 20% of these children experience complete seizure control that is well tolerated. Many children have improved and enhanced cognitive function after seizure reduction.
Parents on the ketogenic diet need a ketogenic diet team including a dietitian. (This booklet should only be used as a guide to using the ketogenic diet. Parents who wish to learn more or understand more can read further references and websites).
Background of Basic Knowledge
The ketogenic diet originated in 1920 as an effective treatment for refractory epilepsy in children. Between 1940 and 1950, the use of the ketogenic diet slowly decreased with the advent of anticonvulsant drugs. As many children with refractory epilepsy failed on multiple anticonvulsant drugs, the ketogenic diet began to gain renewed attention again.
[Brain energy and ketogenic mechanisms].
In the presence of food, glucose is transported into the brain via glucose carrier protein-1 (Glut-1) as a source of energy. During fasting, unsaturated fatty acids provide energy to muscles and other tissues, but are not able to provide energy to the brain. Ketone bodies are synthesized in the liver from unsaturated fatty acids and ketogenic amino acids and are then transported into the brain via monocarboxylate transporter protein-1 (MCT1) to provide energy to the brain during illness or longer fasts. There are three main types of ketone bodies: acetoacetic acid, beta-hydroxybutyric acid, and acetone. During the neonatal period, ketone bodies are also precursors for lipid and amino acid synthesis. Younger children are three to four times more efficient at absorbing and utilizing ketone bodies than older ones.
[Mechanism of action of ketogenic diet].
A ketogenic diet is a high-fat, low-carbohydrate, and moderate-protein diet that mimics the metabolic situation during fasting. The degradation of fat produces ketone bodies, which serve as the body’s reserve energy in the brain and produce an anticonvulsant effect. However, the mechanism of this anticonvulsant remains unclear and the following anticonvulsant mechanisms are commonly hypothesized.
(i) altered energy metabolism in the brain.
(ii) reduction of cellular excitability and inhibition of epileptic discharges.
(iii) alterations in neurotransmitter function, synaptic transmission.
④Alteration of the external environment of the brain, thus inhibiting the excitability of cells.
Indications and contraindications]
Indications contraindications various refractory epilepsy and epileptic encephalopathy fatty acid oxidation deficiency glucose carrier protein 1 (GLUT-1) deficiency ketone body synthesis or catabolism disorder pyruvate dehydrogenase deficiency (PDHD) pyruvate carboxylase deficiency tuberous sclerosis, central nervous inflammatory or degenerative diseases
【Preparation
1. Training in knowledge related to ketogenic diet.
① Parents or guardians to start training (diet overview)
② Parental training (diet calculation, monitoring ketosis, urine ketone test strips, seizure diary, disease management, when to contact the doctor)
③ Regular follow up and follow-up.
2. Main equipment and tools needed for the ketogenic diet
①Ketogenic diet products
②Electronic food scale (accurate to the gram)
③Urinary ketone test paper (to monitor urinary ketones)
④Urinary occult blood test paper (to monitor kidney stones)
⑤Measuring cups with a scale of milliliters Large children such as those who prepare their own meals will additionally need a home crusher, storage box and meal preparation software, etc.