West syndrome and Lennox-Gastaut syndrome

Renzo Guerrini, Carla Marini

Research output: Chapter in Book/Report/Conference proceedingChapter


Infantile spasms and West syndrome Infantile spasms (IS) are a distinctive form of seizure disorder, mainly observed in infants, in the first year of life, and refractory to conventional antiepileptic drugs. Developmental delay or deterioration and a characteristic electroencephalographic (EEG) pattern (hypsarrhythmia) are often associated with IS. All these elements occur together in West syndrome. The seizure type in itself, irrespective of the age and clinical context, is defined as epileptic spasm (Dulac et al. 1994) and may rarely occur in childhood or even in adulthood (Egli 1985; Ikeno et al. 1985; Bednarek et al. 1998; Cerullo et al. 1999; de Menezes and Rho 2002). A cumulative incidence of 2.9 per 10000 live births and an age-specific prevalence of 2.0 per 10,000 in 10-year-old children were observed in the USA (Trevathan et al. 1999). Infantile spasms are manifested as clusters of increasing-plateau–decreasing-intensity brisk (0.5–2.0 s) flexions or extensions of the neck, with abduction or adduction of the upper limbs. Clusters include a few units to several dozens of spasms and are repeated many times per day. After a series, the child is usually exhausted. Other seizure types can coexist. The muscle contraction of IS has distinctive features. It reaches its maximum more slowly than a myoclonic jerk and decreases in an equally slower way (Fusco and Vigevano 1993), though it is faster and less sustained than observed in tonic seizures.

Original languageEnglish
Title of host publicationThe Causes of Epilepsy: Common and Uncommon Causes in Adults and Children
PublisherCambridge University Press
Number of pages16
ISBN (Print)9780511921001, 9780521114479
Publication statusPublished - Jan 1 2011

ASJC Scopus subject areas

  • Medicine(all)


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