By Jacques Emmery, General Practitioner, “Order of Tarn’s doctors”’ vice-president
FIRES’ specificity is its resistance to antiepileptic treatments, which makes the syndrome so dangerous. Nonetheless, the association of several antiepileptic therapies (3 on average, sometimes 4 or even 5) in the chronic phase helps to reduce the frequency of the seizures and their overall intensity. These treatments do have side effects however.
The ketogenic diet: this is a high lipids/low glucose and protein diet. This diet is recommended in the treatment of some drug-resistant epilepsies and some metabolic diseases. This diet limits, or even excludes, some foods such as bread, cereals, pasta, milk, sweets… This diet mimics the sensation of hunger (ketosis). The body then uses the energy from the fat (lipids) and changes it into ketonic elements.
The full effect of this diet on EME in FIRES is not yet known but it appears to transport energy to neurons whose glucose regulation mechanism has been disturbed by EME. A study done in the neuropaediatric department in Necker Hospital in Paris (Pr Dulac, Pr Nabbout) on 9 children has shown this diet to have a significant effect in the reduction of the seizure’s frequency but, overall, the results were very inconsistent. The implementation, monitoring and administration of this diet follows very strict rules and constraints, which limits its use somewhat.