Neurodevelopmental and Epilepsy Outcomes in a North American Cohort of Patients With Infantile Spasms

Partikian, Arthur; Mitchell, Wendy G.

doi:10.1177/0883073809341664

Cited by 43 publications

(41 citation statements)

References 25 publications

(39 reference statements)

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“…It is not known why vigabatrin treatment should be much longer than ACTH treatment, which can have a permanent effect after 2-4 weeks of therapy [24,34]. Effective short-duration treatment may avoid major side effects associated with the treatment of infantile spasms [35].…”

Section: Vigabatrinmentioning

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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Section: Vigabatrinmentioning

confidence: 99%

Recent Advances in the Pharmacotherapy of Infantile Spasms

Riikonen

2014

CNS Drugs

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“…Many clinical studies of ISS have focused on the subsequent intractable epilepsy that develops in 40–60% of affected children [28, 29]. Although important, this approach has underemphasized the broader phenotypes to which ISS may belong.…”

Section: The Biologic Link Between Infantile Spasms and Autismmentioning

confidence: 99%

Genetic and Biologic Classification of Infantile Spasms

Paciorkowski

Thio

Dobyns

2011

Pediatric Neurology

146

125

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Infantile spasms are an age-dependent epilepsy that are highly associated with cognitive impairment, autism, and movement disorders. Previous classification systems have focused on a distinction between symptomatic and cryptogenic etiologies, and have not kept pace with the recent discoveries of mutations in genes in key pathways of central nervous system development in patients with infantile spasms. Children with certain genetic syndromes are much more likely to have infantile spasms, and we review the literature to propose a genetic classification of these disorders. Children with these genetic associations with infantile spasms also have phenotypes beyond epilepsy that may be explained by recent advances in the understanding of underlying biological mechanisms. We therefore also propose a biologic classification of the genes highly associated with infantile spasms, and articulate models for infantile spasms pathogenesis based on that data. The two best described pathways of pathogenesis are abnormalities in the gene regulatory network of GABAergic forebrain development, and abnormalities in molecules expressed at the synapse. We intend for these genetic and biologic classifications to be flexible, and hope that they will encourage much needed progress in syndrome recognition, clinical genetic testing, and ultimately the development of new therapies that target specific pathways of pathogenesis.

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“…Normal or subnormal cognitive development has been reported in approximately 25% of adult patients with previous WS [5]. The prognostic consequences of WS have been causally related to various additional parameters, such as premorbid developmental status, age at onset of WS, time and type of management [10][11][12][13]. The present study was performed to examine the long-term consequences of epilepsy and the psychosocial impacts of WS in the patient population under long-term follow-up in our center.…”

Section: Introductionmentioning

confidence: 96%