3 Spasm onset is associated with developmental plateauing or regression. 2 Given inconsistent terminology as well as variability in clinical and encephalographic presentations, the International League Against Epilepsy proposed a new consensus term for the syndrome: infantile epileptic spasms syndrome (IESS), an epileptic encephalopathy defined by the onset of infantile spasms in infants aged between 1 and 24 months, with peak onset between 3 and 12 months. The term “infantile spasms” has been used inconsistently in the literature, to encompass the syndrome or to refer to the seizures alone. West syndrome is historically defined as an epilepsy of infancy and early childhood characterized by a triad of (1) pathognomonic seizures (infantile spasms), (2) hypsarrhythmia on an electroencephalogram (EEG), and (3) associated neurodevelopmental arrest or regression.

This guide is designed to equip pediatricians with focus on the clinical features, workup, and management of this challenging disorder.ĭiagnostic criteria and clinical importance The primary care pediatrician is the front line for early recognition and treatment initiation in these children. The clinical features described-repeated attacks of “bowings and relaxings” increasing in severity and accompanied by developmental regression-are still deeply familiar to any parent or physician caring for a child with infantile spasms. 1 This letter was the first published description of what became known as West syndrome, or infantile spasms. It was at this time that I first observed slight bobbings of the head forward…these bobbings increased in frequency, and at length became so frequent and powerful, as to cause a complete heaving of the head forward to the knees, and then immediately relaxing into the upright position.” 1īritish physician William James West wrote the above in an 1841 letter to the editor of The Lancet, begging for the medical profession’s attention to a condition afflicting his infant son James. “ was a remarkably fine, healthy child when he was born, and continued to thrive till he was 4 months old.

Waist deep parents guide