How it Affects:

Children: F.I.R.E.S can occur in children at any age from 1 to 17 years old but preferentially affects school-age children, aged 5 to 12. It is slightly more common in boys than girls.

Treatment: There is currently no specific therapy for F.I.R.E.S and studies are urgently needed to determine the best therapeutic options. The treatment of SE initially consists of benzodiazepines (lorazepam, diazepam, or clonazepam), followed by a standard anticonvulsant medication, as in most cases of SE. Preference is given to drugs that are available in IV form (valproic acid, phenytoin, levetiracetam, phenobarbital and lacosamide). Seizures in F.I.R.E.S typically do not respond to high doses of conventional anti-convulsant medications. The two options at that point are either to try additional anticonvulsant medications and/or to induce pharmacological coma with an anesthetic drug. In the former case, medications available in an IV formulation are often favored but others (e.g., topiramate, pregabalin, clobazam) are sometimes used later as add-on therapy. In the latter case, choices include infusion of midazolam and barbiturates (pentobarbital in the USA and thiopental in Europe). The prolonged use of propofol is discouraged in children given the risk of propofol infusion related syndrome (PRIS), a potentially lethal syndrome of acidosis, kidney and heart failure.

Given the putative causal role of inflammation in F.I.R.E.S, approaches that modulate the immune system have been tried. Although they have not been systematically studied, preliminary evidence suggests that treatments such as steroids, intravenous immunoglobulins and plasma exchanges might be helpful. There have been reports of a dramatic response to the ketogenic diet in some children with F.I.R.E.S, but this needs to be confirmed in controlled studies.


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