Sturge-Weber Syndrome Presenting as an Acute Life-Threatening Event

Abstract

Young infants may present to an emergency department after an acute life-threatening event. The differential diagnosis for an acute life-threatening event is quite extensive. Fortunately, most will have benign or treatable causes. A case of Sturge-Weber syndrome is described in a 2-day old who presented to the emergency department after an acute life-threatening event. A thorough history and detailed physical examination are generally helpful in discerning the etiology for most acute life-threatening events and may at times reveal unusual diseases such as Sturge-Weber syndrome.

Sturge–Weber syndrome without facial nevus: An unusual cause of neonatal seizures

“…6 However, seizures of neonatal onset is rare. 7,8 In two previous reports of leptomeningeal angioma sparing occipital lobe and without facial nevus, one patient had seizure onset at the age of 4 months 3 and two other children had headache and seizures at the age of 13 and 11 years. 4 Our patient represents the earliest reported age of seizure onset for this variant.…”

“…6 However, seizures of neonatal onset is rare. 7,8 In two previous reports of leptomeningeal angioma sparing occipital lobe and without facial nevus, one patient had seizure onset at the age of 4 months 3 and…”

Sturge Weber Syndrome: review of literature with case illustration

“…Muniz reported a two days old baby with acute life threatening event ,who presented to an emergency department to seek management [7,8]. Focal motor seizures are the most common neurological manifestation in SWS patients, usually present by the age of three years.…”

“…This syndrome comprises of constellation of symptoms and signs i.e. including facial nevus, seizures, intracranial hemiparesis, calcification and association of mental retardation [2][3][4][5][6][7][8].…”