Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis

Chiron, Catherine; Dumas, Caroline; Jambaqué, Isabelle; Mumford, J. P.; Dulac, Olivier

doi:10.1016/s0920-1211(96)01006-6

Cited by 296 publications

(202 citation statements)

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“…The distribution of VGB responders among various etiology groups was the same as that of all responders. A positive VGB response does not appear to be associated with any specific etiology, with the possible exception of tuberous sclerosis, as suggested earlier (29,36). Infants from all etiology groups responded to VGB, as well as to other treatments, but those with cryptogenic and unidentified symptomatic etiology did so most often.…”

Section: Resultssupporting

confidence: 69%

“…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: Resultssupporting

confidence: 69%

mentioning

confidence: 99%

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

1999

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“…The first small prospective study in children with infantile spasms and an underlying diagnosis of TS found vigabatrin to be more efficient than hydrocortisone at stopping the spasms [45]. In the study by Elterman et al [40], which included subjects with TS, there were significantly more responders among children who had TS (13 of 15) compared with other aetiologies (19/117; p \ 0.001).…”

Section: Tuberous Sclerosis (Ts)mentioning

confidence: 97%

“…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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“…All the VGB treated children became seizure-free compared to only 5/11 of the steroid-treated children. In addition, side effects were greater in the steroid-treated than in the VGB-treated group (40). Additional controlled trials are needed to c o n f m these results.…”

Section: Pediatric Epilepsiesmentioning

confidence: 90%

Vigabatrin

French

1999

Epilepsia

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Summary: Vigabatrin (VGB) is a structural analogue of the inhibitory neurotransmitter y-amino butyric acid (GABA), which produces its antiepileptic effect by irreversibly inhibiting the degradative enzyme GABA-transaminase. This produces an increase in central nervous system (CNS) GABA levels. VGB is among the few antiepileptic drugs (AEDs) that was synthesized with a specific targeted mechanism in mind and was subsequently demonstrated to function by that mechanism. Ti- HISTORY AND MECHANISM OF ACTIONVigabatrin (VGB) was first synthesized in the 1970s and was used clinically for the first time in 1979. Therefore, the clinical experience with this drug spans a longer time period than most of the new AEDs. VGB is approved for use in over 50 countries worldwide, and it is estimated that 175,000 patients have been exposed to the drug. The efficacy of VGB is well known, both in partial epilepsy and in some pediatric epilepsy syndromes.VGB consists of a racemic mixture of R-and Senantiomers. The R-enantiomer is completely inactive (1). It is presumed that the pharmacologic and toxic effects of vigabatrin are produced by the S-enantiomer.The primary mechanism of action of VGB is believed to arise from irreversible inhibition of GABA-transaminase, producing an increase in brain GABA. Experimental evidence demonstrates a clear relationship between brain GABA levels and seizures. Patients with partial epilepsy have lower mean brain GABA levels than controls. In investigations performed with magnetic resonance spectroscopy (MRS), there was a significant correlation between low GABA levels and recent seizures. Well-controlled epilepsy patients had higher mean brain GABA levels than those who were poorly controlled (2). In addition, when GABA levels are measured with implanted microdialysis catheters in patients with temporal lobe epilepsy undergoing presurgical evalua-

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Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis

Cited by 296 publications

References 9 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

Vigabatrin

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