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Since its first description (1841) the identity of West syndrome
was deeply investigated and is now recognized as an epileptic
syndrome in infancy (ILAE Task Force, 1989). West syndrome has
become a paradigmatic model of an epileptic syndrome causing
neurological deterioration (epileptic encephalopathy) and the
object of a number of studies aimed at understanding the complex
relationships between an epileptic disorder and neurodevelopment.
Although the symptomatic triad (peculiar electrographic findings
named hypsarrhythmia, brief tonic spasms, and arrest of psychomotor
development) that characterizes the syndrome suggests a unique
pathogenetic mechanism, causal heterogeneity heavily influences
syndrome variability in terms of neurodevelopment, treatment
choices, management and, possibly, electroclinical semiology.
Important insights may arise for that might help developing models
of epileptic encephalopathies in the basic sciences. However, a
more immediate benefit may arise for clinicians in everyday
practice. A group of clinical researchers recently met in Rome to
discuss hot points concerning infantile spasms and West syndrome.
Their contributions were collected and are presented in this book
that we hope will contribute to the progress of knowledge of this
paradigmatic epileptic disorder.
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