Vigabatrin as Initial Therapy for Infantile Spasms: A European Retrospective Survey

Aicardi, Jean; Mumford, J. P.; Dumas, Caroline; Wood, Susan M.

doi:10.1111/j.1528-1157.1996.tb00627.x

Cited by 175 publications

(121 citation statements)

References 12 publications

(3 reference statements)

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“…The initial response rate to VGB in our study, 26% (or 29% for IS infants from Uusimaa county), is <43-67% previously presented by other authors (32,34,35). If infants with late response also are defined as VGB responders, altogether 14 (33%) infants in our study ceased to have spasms with VGB treatment.…”

Section: Resultsmentioning

confidence: 62%

“…During the last 5 years, VGB also has been used in treatment of newly diagnosed IS, with response rates between 43 and 68% (30)(31)(32)(33)(34). In a placebo-controlled, double-blind study, spasms were not observed after 5 days in 43% of infants treated with VGB (50-150 mg/ kg) and in 15% treated with placebo (35).…”

mentioning

confidence: 99%

“…In a placebo-controlled, double-blind study, spasms were not observed after 5 days in 43% of infants treated with VGB (50-150 mg/ kg) and in 15% treated with placebo (35). In the European Retrospective Survey, 90% of the infants responded within 7 days to a mean dose of 105 mg/kg/day (range, 25400 mg/kg/day), but a relapse was seen in 21% of responders after a mean period of 4 months (77% of them by 3 months) (32). Although the response rate for VGB treatment has been higher for infants with cryptogenic etiology, the best response was observed in IS infants with tuberous sclerosis (29,36).…”

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confidence: 99%

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Treatment of Infantile Spasms: Results of a Population‐Based Study with Vigabatrin as the First Drug for Spasms

1999

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Summary:Purpose: The efficacy of a protocol consisting of vigabatrin (VGB) as the first and adrenocorticotropic hormone (ACTH) or valproate (VPA) as the second drug was studied in the treatment of newly diagnosed infantile spasms (IS) during 1994 to 1997 in a population-based design.Methods: Only total disappearance of the spasms with a minimal duration of 1 month was accepted as a response. The treatment response was confirmed by video-EEG study. All infants were studied by magnetic resonance imaging (MRI) or computed tomography (CT) for etiology.Results: Altogether 42 infants, 10 with cryptogenic and 32 with symptomatic etiology, were treated. Eleven (26%) responded to VGB, five (50%) with cryptogenic, and six (19%) with symptomatic etiology; 91% of infants responded to a dose of 50-100 mgikglday, and 82% of them within 1 week. ACTH was offered in combination with VGB to 22 and VPA to four infants for whom VGB failed. Eleven responded to ACTH and one to VPA. In total, 26 (62%) infants responded to the treatment protocol; all (100%) with cryptogenic etiology and 16 (50%) with symptomatic etiology. ACTH treatment was associated with more severe side effects than VGB or VPA. Only one infant relapsed after a spasm-free period with VGB of >4 months, but none after ACTH was combined with VGB.Conclusions: We suggest VGB as a first drug to all infants with IS. After a treatment trial of 10-14 days with increasing dose from 50 to 150 mgkg, ACTH should be considered. Key Words: Infantile spasms-Epilepsy-Infants-West syndrome-Vigabatrin.Infantile spasms (IS) is an age-dependent epileptic syndrome characterized by epileptic spasms and interictal epileptiform activity that may be focal or diffuse (1). Epileptic spasms start during the first year of life, usually between 4 and 7 months. They are brief axial muscular contractions and occur most often in clusters (2,3). Interictally most infants show hypsarrhythmia or multiple independent spikes on EEG (4). If hypsarrhythmia has been recorded, the term "West syndrome" (WS) also may be used (3).According to etiology, IS are classified into two main groups: a cryptogenic group characterized by normal neurologic and cognitive development before spasm onset and by absence of an identifiable cause, and a symptomatic group associated with underlying abnormalities. An idiopathic subgroup with favorable outcome, consisting of normal psychomotor development and disappearance of epilepsy, has been suggested by some authors (3,5,6 The immediate goal of treatment is complete suppression of spasms. The ultimate goals are lasting seizure control and improved intellectual outcome. Cognitive outcome is especially poor for infants with symptomatic etiology; normal or slightly impaired intelligence has been observed in only 9-18% in contrast to 53-60% of infants with cryptogenic etiology (7-10).Results achieved with adrenocorticotropic hormone (ACTH) have offered a gold standard for the treatment results of IS for 40 years. The initial response rates to steroids or ACTH have varied between 50 ...

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Section: Resultsmentioning

confidence: 62%

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confidence: 99%

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confidence: 99%

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Treatment of Infantile Spasms: Results of a Population‐Based Study with Vigabatrin as the First Drug for Spasms

1999

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“…[107]. The high figures in the study by Aicardi et al [47] could be explained by the lack of EEG evaluation. In the two studies where video telemetry was used [11,40], the response rates were only 16-10 %.…”

Section: Tuberous Sclerosis (Ts)mentioning

confidence: 88%

“…After pooling the data of eight separate prospective and retrospective studies, vigabatrin had a good response on spasms in 44 % of 478 patients [11,22,31,39,40,[45][46][47]. In the pooled data of nine separate prospective and retrospective studies, ACTH had a good response on spasms in 59 % of 476 patients [22, 24, 26-29, 34, 48, 49].…”

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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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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Open Comparative Long-term Study of Vigabatrin vs Carbamazepine in Newly Diagnosed Partial Seizures in Children

Zamponi¹,

Cardinali²

1999

Arch Neurol

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Vigabatrin as Initial Therapy for Infantile Spasms: A European Retrospective Survey

Abstract: This study supports the opinion that VGB may be considered an initial treatment for IS regardless of cause.

Cited by 175 publications

References 12 publications

Treatment of Infantile Spasms: Results of a Population‐Based Study with Vigabatrin as the First Drug for Spasms

Treatment of Infantile Spasms: Results of a Population‐Based Study with Vigabatrin as the First Drug for Spasms

Recent Advances in the Pharmacotherapy of Infantile Spasms

Open Comparative Long-term Study of Vigabatrin vs Carbamazepine in Newly Diagnosed Partial Seizures in Children

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