title: [West syndrome: clinical and electroencephalographic follow up of 70 patients and response to its treatment with adrenocorticotropic hormone, prednisone, vigabatrin, nitrazepam and valproate].
abstract: In a retrospective study we assessed the outcome of the criptogenic and symptomatic forms of West syndrome and evaluated the efficacy of adrenocorticotropic hormone, vigabatrin, prednisone, valproate and nitrazepam in the spasms control. Seventy patients were follwed up by 2 years. Twelve (17%) were criptogenics and 58 (83%) symptomatics. In criptogenic group significantly more patients were in regular school classes and with normal motor development, better control of seizure, less tendency to evoluate to Lennox Gastaut syndrome and 83. 3% had control of spasms (72.4% of patients from symptomatic group had control of spasms). Adrenocorticotropic hormone and vigabatrin were the most efective drugs, with 68.75% and 60% of spasms control, respectivelly, when used as first line of therapy and 75% and 50%, respectivelly, as second line of therapy.
authors.affiliation: Centro de Neurologia Pediátrica, Departamento de Pediatria, Hospital de Clínicas, Universidade Federal do Paraná.
authors.forename: S A
authors.initials: SA
authors.lastname: Antoniuk
authors.forename: I
authors.initials: I
authors.lastname: Bruck
authors.forename: A
authors.initials: A
authors.lastname: Spessatto
authors.forename: S M
authors.initials: SM
authors.lastname: Halick
authors.forename: L R
authors.initials: LR
authors.lastname: de Bruyn
authors.forename: E
authors.initials: E
authors.lastname: Meister
authors.forename: D
authors.initials: D
authors.lastname: de Paola
mesh_terms: D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009567:Nitrazepam / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000188:drug therapy*; D016896:Treatment Outcome; D014635:Valproic Acid / Q000627:therapeutic use; D020888:Vigabatrin / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D005938:Glucocorticoids; D014635:Valproic Acid; D000324:Adrenocorticotropic Hormone; D009567:Nitrazepam; D020888:Vigabatrin; D011241:Prednisone