Occurrence, Outcome, and Prognostic Factors of Infantile Spasms and Lennox‐Gastaut Syndrome

Abstract

Summary: Purpose: To analyze the occurrence, outcome, and prognostic factors of infantile spasms (IS) and the LennoxGastaut syndrome (LGS) in a defined population.Methods: All children treated because of IS and LGS in the Department of Pediatrics, University of Oulu, from January 1, 1976, until December 31, 1993, who came from the primary catchment area of the hospital were included. Detailed information concerning their individual pre-, peri-, and postnatal medical histories and medical and laboratory examinations were compiled.Results: Thirty-seven children (1 8 boys) had IS, and 25 (14 boys) had LGS. The occurrence of IS of 0.41/1,000 live births [95% confidence interval (CI), 0.29-0.57/1,000] did not differ significantly from that of LGS, which was 0.28/1,000 live births (95% CI, 0.18-0.41/1,000). Ten (27%) of the 37 patients with IS evolved to LGS, which was 40% of the LGS cases. All the 10 children with both IS and LGS had symptomatic epilepsy, were mentally retarded, and had active epilepsy at the end of -10 years' follow-up. Twenty-six (87%) of the 30 symptomatic IS cases and all the 17 symptomatic LGS cases were due to either congenital or genetic etiologies. The outcome in cryptogenic IS cases was favorable; the risk for a poor neurologic and mental outcome was extremely low; odds ratio, 0.015 (95% CI, 0.001-0.196). as it was for therapy-resistant epilepsy; odds ratio, 0.013 (95% CI, 0.001-0.166). In LGS patients, cryptogenic etiology did not decrease the risk for a poor outcome.Conclusions: Cryptogenic etiology is associated with a very low risk for a poor outcome in IS patients, but not in LGS patients. The outcome of IS children and the relation of IS to LGS are determined by the underlying brain disease, not by the epilepsy itself.

The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

“…[17] Studies show that 27% of patients with WS evolve to Lennox-Gastaut syndrome (LGS), and that 40% of patients with LGS had WS. [18] This evolution of WS to LGS was not evident in our study, since the follow-up period of patients was rather short, and we had few patients.…”

Síndrome de West, experiencia con una serie de casos con acceso al tratamiento de primera línea, en Lima

“…dentro de los rangos reportados y la etiología fue variada, entre las que destacaron las causas prenatales, tales como las malformaciones cerebrales, Esclerosis Tuberosa, Síndrome de Down y las causas perinatales tal como se ha reportado previamente (10,11,29,30). Se encontró una mayor frecuencia de causalidad secundaria en comparación posiblemente por el mayor acceso de esta población a las pruebas diagnósticas.…”

Predicting antiepileptic drug response in children with epilepsy

“…At follow-up of at least 10 years, 5-13% of individuals were in remission and 8% were not mentally retarded. Patients with cryptogenic LGS had a better cognitive outcome compared with patients who had symptomatic LGS [38], but another study reported no difference between the groups [39]. …”

“…LGS is often (in 13-65% of cases), and in cases of symptomatic etiology in particular, a continuation of West syndrome [36,38,39]. However, as shown in a communitybased study, prenatal or perinatal abnormalities are not necessarily in correlation with the severity of epilepsy [40].…”