Cargando…

West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report

West syndrome (WS), also known as infantile spasms, is a severe form of epileptic disorder of infancy and early childhood. It was first described by William West in 1841. Children with WS exhibit a triad of myoclonic-tonic seizures (spasms), a distinct electroencephalogram (EEG) pattern known as hyp...

Descripción completa

Detalles Bibliográficos
Autores principales: Dias Freitas, Filipa, Cavaco Raposo, Sofia, Luis Nogueira, Augusto
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9792333/
https://www.ncbi.nlm.nih.gov/pubmed/36579257
http://dx.doi.org/10.7759/cureus.31848
_version_ 1784859612331114496
author Dias Freitas, Filipa
Cavaco Raposo, Sofia
Luis Nogueira, Augusto
author_facet Dias Freitas, Filipa
Cavaco Raposo, Sofia
Luis Nogueira, Augusto
author_sort Dias Freitas, Filipa
collection PubMed
description West syndrome (WS), also known as infantile spasms, is a severe form of epileptic disorder of infancy and early childhood. It was first described by William West in 1841. Children with WS exhibit a triad of myoclonic-tonic seizures (spasms), a distinct electroencephalogram (EEG) pattern known as hypsarrhythmia and psychomotor development arrest. WS is classified into three main categories as symptomatic, idiopathic and cryptogenic based on etiological factors. The long-term prognosis depends on the etiological cause, but generally has a poor prognosis, and is associated with impaired development, neurologic structural anomalies, autism spectrum disorder and death. Treatment guidelines from the American Academy of Neurology and Child Neurology Society recommend that adrenocorticotropic hormone (ACTH) and vigabatrin are possibly effective in the cessation of spasms and hypsarrhythmia. We report an incidental diagnosis of WS in a six-month-old male baby that went to the Pediatric Emergency Department due to upper respiratory tract symptoms. The diagnosis was made after the development of spasms during a medical examination. This case highlights the importance of early diagnosis, parental education and prompt effective treatment as it may improve prognosis.
format Online
Article
Text
id pubmed-9792333
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Cureus
record_format MEDLINE/PubMed
spelling pubmed-97923332022-12-27 West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report Dias Freitas, Filipa Cavaco Raposo, Sofia Luis Nogueira, Augusto Cureus Family/General Practice West syndrome (WS), also known as infantile spasms, is a severe form of epileptic disorder of infancy and early childhood. It was first described by William West in 1841. Children with WS exhibit a triad of myoclonic-tonic seizures (spasms), a distinct electroencephalogram (EEG) pattern known as hypsarrhythmia and psychomotor development arrest. WS is classified into three main categories as symptomatic, idiopathic and cryptogenic based on etiological factors. The long-term prognosis depends on the etiological cause, but generally has a poor prognosis, and is associated with impaired development, neurologic structural anomalies, autism spectrum disorder and death. Treatment guidelines from the American Academy of Neurology and Child Neurology Society recommend that adrenocorticotropic hormone (ACTH) and vigabatrin are possibly effective in the cessation of spasms and hypsarrhythmia. We report an incidental diagnosis of WS in a six-month-old male baby that went to the Pediatric Emergency Department due to upper respiratory tract symptoms. The diagnosis was made after the development of spasms during a medical examination. This case highlights the importance of early diagnosis, parental education and prompt effective treatment as it may improve prognosis. Cureus 2022-11-24 /pmc/articles/PMC9792333/ /pubmed/36579257 http://dx.doi.org/10.7759/cureus.31848 Text en Copyright © 2022, Dias Freitas et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Family/General Practice
Dias Freitas, Filipa
Cavaco Raposo, Sofia
Luis Nogueira, Augusto
West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title_full West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title_fullStr West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title_full_unstemmed West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title_short West Syndrome and the Importance of Routine Physical Examinations and Parental Education: A Case Report
title_sort west syndrome and the importance of routine physical examinations and parental education: a case report
topic Family/General Practice
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9792333/
https://www.ncbi.nlm.nih.gov/pubmed/36579257
http://dx.doi.org/10.7759/cureus.31848
work_keys_str_mv AT diasfreitasfilipa westsyndromeandtheimportanceofroutinephysicalexaminationsandparentaleducationacasereport
AT cavacorapososofia westsyndromeandtheimportanceofroutinephysicalexaminationsandparentaleducationacasereport
AT luisnogueiraaugusto westsyndromeandtheimportanceofroutinephysicalexaminationsandparentaleducationacasereport