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Case Report

West Syndrome

Liza Bulsara , Liza Bulsara , Sunil Mhaske , Sham Tarde

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Indian Journal of Trauma and Emergency Pediatrics 6(3-4):p 45-49, July - December 2014. | DOI:

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Abstract

West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if 1 of the 3 elements is missing (according to the international classification).West syndrome is an age-dependent expression of a damaged brain, and most patients with infantile spasms have some degree of developmental delay. The term infantile spasm has been used to describe the seizure type, the epilepsy syndrome, or both. In this article, the term infantile spasm is synonymous with West syndrome. The syndrome’s namesake, Dr W J West, gave the first detailed description of infantile spasms, which occurred in his own child. In a letter to the editor of The Lancet in 1841, West described the events as “bobbings” that “cause a complete heaving of the head forward towards his knees, and then immediately relaxing into the upright position … [T]hese bowings and relaxings would be repeated alternately at intervals of a few seconds, and repeated from 10 to 20 or more times at each attack, which would not continue more than 2 or 3 minutes; he sometimes has 2, 3 or more attacks in the day.” This detailed clinical description was followed approximately 100 years later by the report of the typical interictal EEG pattern termed hypsarrhythmia.

Keywords: Seizure Types; Learning Disabilities; EEG. 

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