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Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982

Journal

JOURNAL OF CLINICAL NEUROPHYSIOLOGY
Volume 39, Issue 7, Pages 521-528

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WNP.0000000000000908

Keywords

Hypsarrhythmia; Infantile spasms; ACTH; West syndrome; History

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This article provides a historical review of knowledge developments about hypsarrhythmia and infantile spasms, with a focus on the period from 1952 to 1982. The goal is to highlight clinical elements that were discovered during that time and remain clinically relevant today.
The initial description of infantile spasms and its association to developmental abnormalities was attributed to Dr. Williams J. West in 1841 but the clinical scenario at the time had also been seen by other physicians. French physician Henry Gastaut proposed the eponym of West syndrome in the 9th Colloquium de Marseille in 1960. The description of hypsarrhythmia in 1952 by Gibbs and Gibbs added the EEG component to the triad of infantile spasms. The hypsarrhythmia discovery led to a sudden interest in understanding the etiology and developing treatments for this devastating disease affecting infants and young children. It was in the 1950s when cases of infantile spasms with absence of hypsarrhythmia were initially observed. Also, the treatment with adrenocorticotrophic hormone was initially reported as efficacious for treating infantile spasms and hypsarrhythmia in the late 1950s. Adrenocorticotrophic hormone remains the best treatment option for these epilepsy types. This article will provide a historical review of knowledge developments about hypsarrhythmia and infantile spasms, emphasizing the period 1952 to 1982. The goal of the article was to highlight clinical elements that were discovered then and remain clinically relevant today.

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