Purpose: Video-EEG in a family of three patients with slow development and familial dysautonomia demonstrated absence seizures associated with 3-Hz generalized spike-and-wave discharges. The seizures were refractory to antiepileptic drugs (AEDs).
Methods: Treatment was given with rice-based cereal electrolyte oral solution.
Results: Treatment induced seizure freedom and normalization of EEG in all three patients. Repeated video-EEG monitoring with discontinuation of AEDs and maintenance of the oral hydration therapy was associated with recurrence of epileptic activity. All three patients have remained seizure free (approximately 1 year) with a combination of topiramate and electrolytic therapy.
Conclusions: Rice-based oral electrolyte hydration therapy may play a role in prevention and control of seizures in patients with familial dysautonomia.