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West Syndrome: A Review and Guide for Paediatricians

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A Letter to the Editor to this article was published on 19 March 2018

Abstract

West syndrome (WS), also known as infantile spasms, occurs in infancy with a peak between 4 and 7 months. Spasms, neurodevelopmental regression and hypsarrhythmia on electroencephalogram (EEG) basically define WS. The International League Against Epilepsy commission classifies the aetiologies of WS into genetic, structural, metabolic and unknown. Early diagnosis and a shorter lag time to treatment are essential for the overall outcome of WS patients. These goals are feasible with the addition of brain magnetic resonance imaging (MRI) and genetic and metabolic testing. The present work analysed the medical literature on WS and reports the principal therapeutic protocols of its management. Adrenocorticotropic hormone (ACTH), vigabatrin (VGB) and corticosteroids are the first-line treatments for WS. There is no unique therapeutic protocol for ACTH, but most of the evidence suggests that low doses are as effective as high doses for short-term treatment, which is generally 2 weeks followed by dose tapering. VGB is generally administered at doses from 50 to 150 mg/kg/day, but its related retinal toxicity, which occurs in 21–34% of infants, is most frequently observed when treatment periods last longer than 6 months. Among corticosteroids, a treatment of 14 days of oral prednisolone (40–60 mg/day) has been considered effective and well tolerated. Considering that an early diagnosis and a shorter lag time to treatment are essential for successful outcomes in these patients, further studies on efficacy of the different therapeutic approaches with evaluation of final outcome after cessation of therapy are needed.

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Authors and Affiliations

  1. Pediatric Clinic, Department of Surgical and Biomedical Sciences, Università degli Studi di Perugia, Piazza Menghini 1, 06129, Perugia, Italy

    Renato D’Alonzo, Elisabetta Mencaroni & Susanna Esposito

  2. Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy

    Donato Rigante

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  1. Renato D’Alonzo
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Correspondence to Susanna Esposito.

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This review was supported by an unrestricted grant from the World Association of Infectious Diseases and Immunological Disorders (WAidid).

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D’Alonzo, R., Rigante, D., Mencaroni, E. et al. West Syndrome: A Review and Guide for Paediatricians. Clin Drug Investig 38, 113–124 (2018). https://doi.org/10.1007/s40261-017-0595-z

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