Zur Geschichte des West-Syndroms (Blitz-Nick-Salaam-Epilepsie, „infantile epileptic spasms syndrome“ [IESS])
摘要
“Seizures” have always been observed in many infants, e.g., harmless symptoms such as startle reactions and benign sleep myoclonus, large seizures with fever but also frequent lightning-like small seizures and tonic stretches, so-called lightning, nodding, Salaam (LNS) seizures in severe developmental disorders, e.g., after oxygen deficiency or meningitis. The aim of the article is to present the eventful history of this age-typical form of epilepsy, which can be seen as the pathophysiological end point of very different causes. After detailed descriptions of early childhood seizures up to the beginning of the twentieth century, there was little interest in a differentiated diagnosis of early childhood epilepsy up until the time of the Second World War. The registration of the characteristic electroencephalograph (EEG) changes from the 1950s onwards allowed a more reliable diagnosis. The treatment options improved with the use of corticotropin or corticoids, which was first described in 1958, although the exact pathomechanism is still not understood. Reliable epidemiological studies are not known but the number of children with LNS epilepsy has clearly decreased with the improvement of perinatal care. Nowadays, most diagnosed children have genetic causes. According to the internationally accepted guidelines that are still in force, the treatment is mostly symptomatic and the prognosis is often unfavorable.