Infantile spasms:A seizure disorder of infancy and earlychildhoodwith theonsetpredominantlyinthe first year of life of myoclonic seizures, hypsarrhythmia (abnormal, chaotic electroencephalogram), andmental retardation. The spasms are sudden, brief contractions of one or moremusclegroups, and may be followed by a longer (less than 10 seconds) tonic phase. Most often the spasms occur in clusters during which the intensity or the frequency of the spasms may increase progressively to a peak, decline, or cease. The clusters tend to occur soon after arousal from sleep. They are not a feature of falling asleep. The spasms usually involve the muscles of theneck, trunk, and extremities.
神经系统abnormalities other thanseizuresand retardation -- such ascerebralatrophy,congenitalabnormalities and hydrocephalus -- are commonly reported among children with infantile spasms.
Some patients may be treated successfully with either ACTH (adrenocorticotrophichormone) orprednisone. Newer antiepileptic medications, such as vigabatrin, and occasionally surgicalresectionof aseizure集中触发spasms, may be useful in selected patients.
Infantile spasms represent one of the most devastatingseizure disordersaffecting infants. Spontaneous cessation of spasms occurs in most patients with increasing age. Overall, however, the intellectualprognosisfor patients is generally poor because a large number of thesebabieshave neurological impairment prior to the onset of spasms. Many go on to have different types of seizures in later childhood.
Thediseaseis sometimes calledWest syndrome.
