Dravet Syndrome

Dravet syndrome (DS) is one of the refractory epileptic encephalopathies [3], one of the spectra of severe myoclonic Epilepsy of Infancy (SMEI) which occurs in otherwise healthy individuals [2]. Its incidence has been estimated to be 1 in 2000040000. It is more common in males than in females. Positive family history is encountered in one fourth of the cases. DS Usually started by clonic/tonic-clonic seizures, hemi-convulsions or generalized seizures. They used to have febrile seizures as an initial presentation which will be followed by afebrile seizures thereafter. Seizure frequency will vary on individual basis, though usually lies between once every onetwo months. Status epilepticus is common in the form of generalized tonic clonic or hemi-clonic seizures; especially through the few months after presentation. Other forms of seizures, e.g. atypical absences, myoclonic and complex partial seizures will be elicited by the second or third year of life. Seizures will start to show increased frequency and decreased duration over time. Triggering factors like eye closure, photic stimulation can be beneficial through regular EEG recording, which is usually normal in the beginning of the disease and started to show generalized spike and polyspikes epileptiform discharges by the second year.