Síndrome de West: evolução clínica e eletrencefalográfica de 70 pacientes e resposta ao tratamento com hormônio adrenocorticotrófico, prednisona, vigabatrina, nitrazepam e ácido valpróico

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.

West syndrome: early remission is not assurance of normal final outcome

“…The general features of the infants here reported are similar to those described by others: male predominance (64.8%), beginning of seizures on average at the age of 5 months (4.56 Ϯ 2.39) and the majority of symptomatic cases (78.3%). Regarding etiology, neonatal hypoxia was more common in our series than previously described in our country (Antoniuk et al 2000), but among prenatal (Down syndrome, tuberous sclerosis, cerebral malformation and metabolic disorders) and postnatal causes, we had similar findings. We think our sample is reasonably representative of Brazilian children with WS, because of the open referral system of our hospital and because of the large population assisted.…”

“…The American Academy of Neurology and the Child Neurology Society favour ACTH and suggest that this should be the first line drug for the control of the infantile spasms. The reason for this is the lack of robust randomized clinical trials, and most observations come from open studies (Antoniuk et al . 2000; Ito et al .…”

“…The American Academy of Neurology and the Child Neurology Society favour ACTH and suggest that this should be the first line drug for the control of the infantile spasms. The reason for this is the lack of robust randomized clinical trials, and most observations come from open studies (Antoniuk et al 2000;Ito et al 2002;Lux et al 2004Lux et al , 2005Azam et al 2005;Cohen-Sadan et al 2009). Regarding steroids, in the UK, a study with 107 infants showed that 40 mg/day of prednisolone or 2 mg/kg/day of prednisone could reach the same results as 40 IU of ACTH in alternate days (Lux et al 2004(Lux et al , 2005.…”

Os 50 anos de uso do hormônio adrenocorticotrófico (ACTH) no Tratamento da Síndrome de West: revisão de literatura e protocolo da UNIFESP

“…12 No Brasil, um estudo restrospectivo avaliando 70 pacientes demostrou que o ACTH e a VGB foram as drogas mais eficazes para o controle da SW, com taxas de 68,7% e 60% respectivamente, de pacientes que atingiram controle clínico dos espasmos. 2…”

Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants

“…46 Treatment included steroids, VBG, levetiracetam, sodium valproate, or topiramate. 46 A retrospective study 47 (n ¼ 70) evaluated the efficacy of ACTH (n ¼ 16; 1 dose 20 to 40 IU), VGB (n ¼ 5; 35 to 75 mg/kg/ day), prednisone (n ¼ 14; 2 to 5 mg/kg/day), valproic acid (n ¼ 17; 15 to 60 mg/kg/day), and nitrazepam (n ¼ 15; 0.5 to 1 mg/kg/day). Patients started with monotherapy for 4 weeks, but in case of failure, clinicians replaced or added another drug.…”