Infantile Spasms: Little Seizures, BIG Consequences

Shields, W. Donald

doi:10.1111/j.1535-7511.2006.00100.x

Cited by 116 publications

(125 citation statements)

References 57 publications

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“…Riikonen [4,5] reported that patients of WS with a favorable outcome had normal neurodevelopment before the onset of the spasms. Nearly all previous studies on this variable reported similar results [3,7,10,[21][22][23][24][25][26]. The presence of parental consanguinity and epilepsy in near relatives showed no significant effect on prognosis in this study.…”

Section: Neuro-developmental and Social Outcomessupporting

confidence: 84%

Long-term outcomes in patients with West syndrome: An outpatient clinical study

Güveli

Çokar

Dörtcan

et al. 2015

Seizure

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Section: Neuro-developmental and Social Outcomessupporting

confidence: 84%

Long-term outcomes in patients with West syndrome: An outpatient clinical study

Güveli

Çokar

Dörtcan

et al. 2015

Seizure

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“…134 For treatment of IS, however, the goal is complete sei- zure amelioration, to improve developmental outcome, even when absolute developmental normalization is precluded by comorbid diagnoses. 134 Vigabatrin has substantially reduced or completely ameliorated seizures in many patients and may improve the developmental outcome of some IS patients, particularly those with a cryptogenic etiology, tuberous sclerosis, or localized cortical dysplasia. 32,134 Given the catastrophic nature of IS, the risk of peripheral VFDs may be acceptable if appropriate seizure control can be achieved, thus providing an improved opportunity for normal development.…”

Section: Benefit-risk Assessmentmentioning

confidence: 99%

“…134 Vigabatrin has substantially reduced or completely ameliorated seizures in many patients and may improve the developmental outcome of some IS patients, particularly those with a cryptogenic etiology, tuberous sclerosis, or localized cortical dysplasia. 32,134 Given the catastrophic nature of IS, the risk of peripheral VFDs may be acceptable if appropriate seizure control can be achieved, thus providing an improved opportunity for normal development. 134 For this reason, many pediatric epileptologists continue to regard vigabatrin as the drug of choice in the treatment of IS, despite the risk of visual field abnormalities.…”

Section: Benefit-risk Assessmentmentioning

confidence: 99%

“…32,134 Given the catastrophic nature of IS, the risk of peripheral VFDs may be acceptable if appropriate seizure control can be achieved, thus providing an improved opportunity for normal development. 134 For this reason, many pediatric epileptologists continue to regard vigabatrin as the drug of choice in the treatment of IS, despite the risk of visual field abnormalities.…”

Section: Benefit-risk Assessmentmentioning

confidence: 99%

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Vigabatrin

2007

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Summary: Refractory epilepsies such as infantile spasms (IS) and complex partial seizures (CPS) can have a severe negative impact on the neurological integrity and quality of life of affected patients, in addition to drastically increasing their risk of premature mortality. Early identification of potentially effective pharmacotherapy agents is important. Vigabatrin has been shown to be a generally well tolerated and effective antiepileptic drug (AED) in a wide variety of seizure types affecting both children and adults, particularly those with IS and CPS. A bilateral, concentric constriction of the peripheral visual field characterizes the visual field defect (VFD) associated with vigabatrin, well characterized by numerous studies. This peripheral VFD presents in 30 -50% of patients with exposure of several years; however, most of these patients are asymptomatic. In well-controlled studies, the earliest onset in patients with CPS is 11 months and at 5 months in infants, with average onsets being more than 5 years and 1 year, respectively. Patients with a peripheral VFD retain an average 65°of lateral vision (normal, 90°). The fact that many patients never develop the vigabatrin-related peripheral VFD, despite long-term exposure at high doses, may support the hypothesis that the injury is an idiosyncratic adverse drug reaction (as opposed to a strict dose-or duration-dependent toxicity). Effective testing methods are available to aid in the early detection and management of the peripheral VFD. This article discusses issues of importance to clinical decision-making in the use of vigabatrin to assist the physician and patient in assessing the benefits of vigabatrin therapy and understanding the potential risks of the VFD and uncontrolled seizures.

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“…Despite the numerous studies in literature reporting effectiveness of ACTH and oral steroids in treatment of infantile spasm, the exact mechanism is unknown. 56 Clinical benefits of ACTH in infantile spasms thought to be due to release of adrenocorticosteroids and neurosteroids. Recent studies show that several newer anticonvulsant agents (such as vigabatrin and benzodiazepines), which are positive allosteric modulators of GABA A receptors, are as effective as ACTH in acutely controlling infantile spasms.…”

Section: Infantile Spasmmentioning

confidence: 99%