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Título

WEST SYNDROME: A literary review

Resumo

Introduction: West Syndrome (WS) was first described by William James West (1793–1848) in 1841 and classically corresponds to an epileptic syndrome characterized by repetitive spasms and a pattern of hypsarrhythmia that result in a delay in neuropsychomotor development. This pathology has been progressively studied and can often be named as infantile spasm, based on the most relevant initial clinical event. Although its causes are unknown, in recent years new advances in this disorder have been made, and point to predisposing factors for the disease: hypoglycemia, lack of oxygen to the brain during childbirth, brain malformation, prematurity, sepsis, stroke, infections and genetic. Objective: To systematically demonstrate the West Syndrome questioning its complexities and peculiarities and what makes it a rare and complex syndrome. Methodology: This is a systematic literature review based on the extraction of data in indexes such as PUBMED and MEDLINE. Publications from 2018 to 2022, in English, were considered, with the following descriptors: West syndrome, Neurologia, Epilepsy, Infantile spasms. Results: Overreliance on the term hypsarrhythmia is particularly problematic, since many children who experience infantile spasms will not have this classic pattern. Presence or absence on the pre-treatment EEG has no impact on treatment response, risk of subsequent seizures, or later developmental outcome. The hypsarrhythmia requirement in the criteria for West syndrome is unsupported by documentary evidence and is overly restrictive. Thus, almost all babies with the infantile spasms type of seizure also have West syndrome. Conclusion: It can be concluded that many advances have been made, however there are still many issues to be resolved about SW, which suggests steps forward. As a result, the diagnosis of West syndrome should not be made in a restrictive manner and even though the defining triad of the disease can be considered, the absence of a criterion should not exclude the diagnosis, so it should not interfere in the planned treatment for the disease.