**4.5 Treatment and prognosis**

The aim of treatment in patients with DS is reducing seizure frequency, minimizing comorbidities, limiting antiepileptic drug toxicity, and avoiding seizurerelated injury and SUDEP [54]. A greater degree of cognitive and behavioral impairment has been associated to higher frequencies of seizures [55, 56].

It is prominent that seizures are triggered by hyperthermia and less frequently by photosensitivity or pattern sensitivity; thus antipyretics for fever, minimizing

warm baths or exercising on warm days, and avoiding photosensitivity triggers are recommended [54].

Sodium channel-blocking drugs such as carbamazepine, oxcarbazepine, lamotrigine, and phenytoin should also be avoided because they can aggravate seizures.

Valproic acid, clobazam, topiramate, levetiracetam, and stiripentol are the drugs of choice. Stiripentol combined with valproic acid and clobazam, as well as topiramate, give promising results [54, 57, 58]. The ketogenic diet is an alternative with good results for several patients, achieving a reduction of the seizures by 50% or more [59, 60].

The prognosis for children with DS is poor; the complete cessation of epileptic seizures is not achievable in these patients. Since the onset of disease, the neurological status worsens, and about 10–20% of afflicted children will die prematurely [61, 62]. Early mortality, sometimes due SUDEP, occurs in about 10% of patients. However, the outcome, in at least some children, improves with early diagnosis and appropriate therapeutic intervention.
