DRAVET SYNDROME: A PAEDIATRIC CASE SCENARIO IN A TERTIARY CARE HOSPITAL IN SOUTHERN INDIA

Eldhose Elias George, Anjali Shaju and Jobin Kunjumon Vilapurath*

ABSTRACT

Dravet syndrome (DS) is an epileptic encephalopathy that begins with prolonged seizures in infancy, often accompanied by a fever, and is initially classified as a febrile seizure. In 80 to 90 percent of cases, DS is linked to a mutation in the SCN1A gene. Early identification of Dravet syndrome in a child who presents with febrile seizures is critical, as it allows appropriate care to be instituted. This case study presents Dravet Syndrome of a 7 year old female child who is presented with the chief complaints of fever and cough for the past two days, abdominal distension, and multiple episodes of abnormal body movements for one week. Based on the subjective evidence and patient’s history,she had dravet syndrome with acute respiratory tract infection. The treatment given to the child include ampicillin, paracetamol, valproic acid, clobazam, phenobarbitone and salbutamol. The child was discharged after complete resolution of symptoms. The patient's condition had improved and there were no new concerns when the review was done two weeks later. This case report emphasizes early diagnosis and treatment of DS and the importance of giving proper treatment in improving the clinical outcome and in preventing serious complications.

Keywords: Dravet syndrome, Pediatrics, Epilepsy, SCN1A gene, Febrile convulsion.