ABSTRACT

The clinical presentation of infantile spasms, so lucidly described by West, has been carefully studied and further characterized by the use of continuous electroencephalographic (EEG)-videotelemetry monitoring. Abdominal flexion may be massive, giving rise to the “jack-knife” or “salaam” seizures that are the hallmark of infantile spasms. Infantile spasms usually occur in clusters many times daily, but particularly upon awakening, and are often associated with a cry. The doubling over and crying seen with massive abdominal flexor spasms may lead to a misdiagnosis of colic. The diagnosis of infantile spasms is established by a clinical description of the seizure, a hard copy EEG, and EEG-videotelemetry monitoring for 6 to 12 hours. All children will develop Cushingoid features, and most will show extreme irritability early in the course. The etiology of this is obscure but because of this side effect, one should perform diagnostic computerized tomographic scans in children with infantile spasms prior to initiation of ACTH.