West Syndrome: A Review and Guide for Paediatricians

Renato D’Alonzo, Donato Rigante, Elisabetta Mencaroni, Susanna Esposito

Research output: Contribution to journalReview article

5 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)113-124
Number of pages12
JournalClinical Drug Investigation
Volume38
Issue number2
DOIs
Publication statusPublished - Feb 1 2018

Fingerprint

Infantile Spasms
Vigabatrin
Therapeutics
Adrenocorticotropic Hormone
Early Diagnosis
Adrenal Cortex Hormones
Pediatricians
Spasm
Genetic Testing
Prednisolone
Electroencephalography
Epilepsy
Magnetic Resonance Imaging

ASJC Scopus subject areas

  • Pharmacology (medical)

Cite this

West Syndrome : A Review and Guide for Paediatricians. / D’Alonzo, Renato; Rigante, Donato; Mencaroni, Elisabetta; Esposito, Susanna.

In: Clinical Drug Investigation, Vol. 38, No. 2, 01.02.2018, p. 113-124.

Research output: Contribution to journalReview article

D’Alonzo, Renato ; Rigante, Donato ; Mencaroni, Elisabetta ; Esposito, Susanna. / West Syndrome : A Review and Guide for Paediatricians. In: Clinical Drug Investigation. 2018 ; Vol. 38, No. 2. pp. 113-124.
@article{6db5d669de68456fa4f439e3835b200b,
title = "West Syndrome: A Review and Guide for Paediatricians",
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.",
author = "Renato D’Alonzo and Donato Rigante and Elisabetta Mencaroni and Susanna Esposito",
year = "2018",
month = "2",
day = "1",
doi = "10.1007/s40261-017-0595-z",
language = "English",
volume = "38",
pages = "113--124",
journal = "Clinical Drug Investigation",
issn = "1173-2563",
publisher = "Adis International Ltd",
number = "2",

}

TY - JOUR

T1 - West Syndrome

T2 - A Review and Guide for Paediatricians

AU - D’Alonzo, Renato

AU - Rigante, Donato

AU - Mencaroni, Elisabetta

AU - Esposito, Susanna

PY - 2018/2/1

Y1 - 2018/2/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85032686727&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85032686727&partnerID=8YFLogxK

U2 - 10.1007/s40261-017-0595-z

DO - 10.1007/s40261-017-0595-z

M3 - Review article

C2 - 29086890

AN - SCOPUS:85032686727

VL - 38

SP - 113

EP - 124

JO - Clinical Drug Investigation

JF - Clinical Drug Investigation

SN - 1173-2563

IS - 2

ER -