Angelman Syndrome
Conditions
Interventions
During study patients use adequately dosed AED in depended to kind of study
group.
First period:
After a run-in period of 1 month in which patients follow normal
diet and AED will be continued and
3 months
Randomisation into
1. Diet group: KD (5 energy% carbohydrates), containing 15 energy% MCT fat and
AED.
2. Control group: continue normal diet (patient related) .
Third period: 3 months
1.
- patients with > 50 % seizure reduction switch to a MAD (10 energy %
range 20-40 grams carbohydrates ), energy from fat and protein is
unrestricted,
15 energy% MCT fat supplemented.
- patients w
Sponsors
KInderneurologie
Eligibility
Age
2 Years to 17 Years
Inclusion criteria
Inclusion criteria: - Angelman syndrome (genetically proven) - > 2 year - 50% of energy need by oral food. - Written informed consent by parents - AED use
Exclusion criteria
Exclusion criteria: - 18 years. - on KD or LGID treatment during the past 6 months. - overweight: weight /height : > +2.5 SD or BMI >30 - underweight : weight/height; 50% of energy need) tube feeding dependent. - deviated lipid profiles
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - 50% seizure reduction or more after 3 months of dietary treatment. | — |
Secondary
| Measure | Time frame |
|---|---|
| -observation of level of ketosis during dietary treatment. ketosis will be measured on daily basis by parents -improved EEG outcomes. -maintaining or improved QoL. -maintaining patient related growth curves. -safety by closely monitoring of side effects of AED and dietary treatment. -feasibility by monitoring behavior, feeding difficulties and parental stress by questionnaires . -protocol compliance. | — |
Countries
Netherlands
Outcome results
None listed