[Proposals for the treatment of children with West's syndrome]

J L Herranz1, A Argumosa

  • 1Servicio de Neuropediatría, Hospital Universitario Marquís de Valdecilla, Santander, Spain. jherranz@nacom.es

Revista De Neurologia
|October 31, 2000
PubMed

Insights

Treatment guidelines for infantile spasms (West's syndrome) recommend starting with prednisone for idiopathic cases and vigabatrin for symptomatic cases. Adjustments to vigabatrin, prednisone, topiramate, or valproate are suggested based on efficacy and side effects.

Area of Science:

  • Pediatric Neurology
  • Clinical Pharmacology

Context:

  • Infantile spasms, also known as West's syndrome, represent a severe epilepsy syndrome in infants.
  • Establishing effective treatment protocols is crucial for improving outcomes and reducing long-term neurological deficits.

Purpose:

  • To review and justify treatment guidelines for infantile spasms (West's syndrome) in children.
  • To evaluate the efficacy and side-effects of various pharmacological interventions based on prospective studies.

Summary:

  • For idiopathic or cryptogenic West's syndrome, initial treatment with oral prednisone (up to 8 mg/kg/day) is recommended, transitioning to vigabatrin if ineffective or poorly tolerated.
  • For secondary or symptomatic West's syndrome, vigabatrin (up to 200 mg/kg/day) is the preferred first-line treatment, with prednisone as an alternative if vigabatrin fails or causes side effects.
  • If initial treatments are unsuccessful, topiramate (up to 24 mg/kg/day) or valproate (up to 200 mg/kg/day) are suggested as subsequent therapeutic options.

Impact:

  • Provides evidence-based recommendations for the management of West's syndrome, aiming to optimize treatment strategies.
  • Guides clinicians in selecting appropriate first-line and alternative therapies based on the etiology and response of infantile spasms.
Abstract

Related Concept Videos

Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Sex-linked Disorders02:28

Sex-linked Disorders

Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.Y chromosome mutations are called “Y-linked” and only affect males since they alone carry a copy of that chromosome. Mutations to the relatively small Y chromosome can impact male sexual function and secondary sex characteristics. Y-chromosome infertility is a disorder that affects sperm...
Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...