Developmental and epileptic encephalopathy
Conditions
Interventions
Group of ion channel genes:Ketogenic diet
Group of other genes:Ketogenic diet
Sponsors
Beijing Children's Hospital, Capital Medical University, National Center for Children's Health
Eligibility
Sex/Gender
All
Age
0.2 Years to 18 Years
Inclusion criteria
Inclusion criteria: 1. Genetic testing confirmed DEE caused by single gene variants, except fatty acid metabolism disorders and other contraindications of KD, Dravet syndrome, tuberous sclerosis complex and other disease entities with other clinical features and combined causes, as well as type I glucose transporter deficiency syndrome, pyruvate dehydrogenase deficiency KD preferred indications are not within the scope of this study; 2. Aged 2 months to 18 years, onset age = 2 times a month before KD; 3. The type and dose of the original antiseizure medications and vagus nerve stimulation parameters in the first month of KD are not adjusted temporarily; 4. Able to receive regular follow-up, monitoring of blood ketone, blood glucose, recording seizures, cognitive and growth development, regular review, organ ultrasound, developmental assessment; 5. Intolerable adverse reactions or due to various reasons can not continue KD, can withdraw from the study at any time.
Exclusion criteria
Exclusion criteria: 1. Parents/guardians did not agree to participate in this study and did not sign the informed consent form; 2. The ketogenic ratio did not reach the lowest effective ratio of 2:1.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Seizure frequency; | — |
Secondary
| Measure | Time frame |
|---|---|
| Cognitive development;Adverse reaction;Reason for withdrawal; | — |
Countries
China
Contacts
Public ContactDeng Jie
Beijing Children's Hospital, Capital Medical University, National Center for Children's Health
Outcome results
None listed