Multicenter long‐term follow‐up of children with idiopathic West syndrome: ACTH versus vigabatrin

Cohen-Sadan, S.; Kramer, Uri; Ben‐Zeev, Bruria; Lahat, Eli; Şahar, Esra Atalay; Nevo, Yoram; Eidlitz, T.; Zeharia, Avraham; Kivity, Sara; Goldberg-Stern, Hadassah

doi:10.1111/j.1468-1331.2008.02498.x

Cited by 60 publications

(46 citation statements)

References 19 publications

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“…In a Class III study, early treatment initiation (\1 month following onset of symptoms) in 102 children with cryptogenic spasms, led to improved neurodevelopmental outcome [48]. Favourable cognitive outcome with shorter interval to treatment (\3 weeks [108], \4 weeks [109] and \4 weeks [42]) has also been seen in recent studies including patients with both cryptogenic and symptomatic spasms.…”

Section: Tuberous Sclerosis (Ts)mentioning

confidence: 90%

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Recent Advances in the Pharmacotherapy of Infantile Spasms

Riikonen

2014

CNS Drugs

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Adrenocorticotrophic hormone (ACTH), oral corticosteroids and vigabatrin are now first-line treatments for infantile spasms in the US and Europe. There is now increased knowledge regarding the role of ACTH, corticosteroids and vigabatrin (e.g. efficacy, doses, side effects, treatment in specific aetiological subtypes of infantile spasms), and other antiepileptic drugs (i.e. topiramate, valproate, zonisamide, sulthiame, levetiracetam, lamotrigine, pyridoxine, ganaxolone), as well as adjunctive flunarizine and novel drugs not yet in clinical use for infantile spasms (i.e. pulse rapamycin and melanocortin receptor agonists). The existence of a latent period, weeks to months following a precipitating brain insult, raises the possibility of preventive interventions. Recent experimental data emerging from animal models of infantile spasms have provided optimism that new and innovative treatments can be developed, and knowledge that drug treatment can affect long-term cognitive outcome is increasing. The aim of this article is to review recent developments in the pharmacotherapy of infantile spasms and to highlight the practical implications of the latest research.

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Section: Tuberous Sclerosis (Ts)mentioning

confidence: 90%

“…In a multicentre retrospective study in Israel in children with idiopathic West syndrome [42] (Class IV), 14 children were treated with vigabatrin (100-180 mg/kg/day) and 14 with ACTH (100 IU on alternate days). Spasm cessation with vigabatrin at 2 weeks was 80 % and with ACTH was 88 %.…”

Section: Is Acth More Effective Than Vigabatrin For Shortterm Treatmementioning

confidence: 99%

Recent Advances in the Pharmacotherapy of Infantile Spasms

Riikonen

2014

CNS Drugs

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“…On sekiz aydan sonra nadir görülmekle birlikte, başlangıcı 4 yaş kadar geç olan olgular bildirilmiş-tir [5]. Önceki çalışmalarla benzer şekilde [10][11][12][13], çalışmamızda başlangıç yaşı ortalama 6.23 aydı.…”

Section: Discussionunclassified

“…Belirtilerin başlangıcından tanıya kadar geçen süre gelişmiş ülkelerde 1-1.5 ay arasında bildirilirken [10,12,13], gelişmekte olan ülkelerde 7.9 ay kadar uzun olabilmektedir [11]. Çalışmamızda ise bu süre 1.36 aydı.…”

Section: Discussionunclassified

West syndrome: Clinical characteristics and short-term outcome

Yılmaz

2014

J Clin Exp Invest

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“…These treatments may stop IS in 60% -90% of cases, depending on etiology, but they do not necessarily prevent recurrences or persistence of other epileptic seizures or neurodevelopmental deficits. In WS of unknown etiology (old cryptogenic category), early cessation of spasms with hormonal therapy has been shown to partially improve neurodevelopmental outcomes (Riikonen 1982;Lombroso 1983;Kivity et al 2004;Lux et al 2005;Lux and Osborne 2006;Cohen-Sadan et al 2009;Darke et al 2010;O'Callaghan et al 2011), although no difference in outcome was reported in a different study (Mohamed et al 2011). The need for more effective and better-tolerated therapies with rapid onset of action is evident, including infants with IS caused by structural/metabolic etiologies who have poorer prognosis and treatment response.…”

Section: Animal Models Of Epileptic/is and West Syndromementioning

confidence: 99%

Neonatal and Infantile Epilepsy: Acquired and Genetic Models

Galanopoulou

Moshé

2015

Cold Spring Harb Perspect Med

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The incidence of seizures and epilepsies is particularly high during the neonatal and infantile periods. We will review selected animal models of early-life epileptic encephalopathies that have addressed the dyscognitive features of frequent interictal spikes, the pathogenesis and treatments of infantile spasms (IS) or Dravet syndrome, disorders with mammalian target of rapamycin (mTOR) dysregulation, and selected early-life epilepsies with genetic defects. Potentially pathogenic mechanisms in these conditions include interneuronopathies in IS or Dravet syndrome and mTOR dysregulation in brain malformations, tuberous sclerosis, and related genetic disorders, or IS of acquired etiology. These models start to generate the first therapeutic drugs, which have been specifically developed in immature animals. However, there are challenges in translating preclinical discoveries into clinically relevant findings. The advances made so far hold promise that the new insights may potentially have curative or disease-modifying potential for many of these devastating conditions. T he neonatal and infantile periods show relatively high age-adjusted incidence of epilepsy compared with other ages of life (Hauser et al. 1993). The etiology of newly diagnosed epilepsies at these ages also is distinct, showing greater representation of epilepsies of unknown, genetic, or congenital etiologies (Hauser et al. 1993). The semiology and syndromic phenotypes of epilepsies that first appear in this early life period are also distinct and often more fulminant, appearing with evident neurodevelopmental problems or regression (e.g., epileptic encephalopathies) or characteristic seizure types (e.g., infantile spasms [IS], multifocal clonic seizures) ( Table 1). In addition, the treatments for epilepsies of these age groups can be specialized, as is the case with the use of hormonal therapies (e.g., adrenocorticotropic hormone [ACTH] in certain epileptic encephalopathies). Given the serious repercussions for the development of these neonates and infants and the importance of finding better therapies with disease-modifying potential, several efforts have been made to create animal models of these conditions to increase our understanding and ability to treat them. Here, we will review selected animal models, genetic or induced, that address epilepsies and epileptic encephalopathies characteristic of neonates and infants.

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Multicenter long‐term follow‐up of children with idiopathic West syndrome: ACTH versus vigabatrin

Abstract: Idiopathic WS with normal MRI is associated with a good cognitive outcome. Early ACTH treatment, administered within 1 month, yields a better cognitive and seizure outcome than vigabatrin or late ACTH.

Cited by 60 publications

References 19 publications

Recent Advances in the Pharmacotherapy of Infantile Spasms

Recent Advances in the Pharmacotherapy of Infantile Spasms

West syndrome: Clinical characteristics and short-term outcome

Neonatal and Infantile Epilepsy: Acquired and Genetic Models

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