Sindrome di West criptogenetica: Evoluzione cognitiva e studio neurofisiologico in 28 pazienti seguiti dall'esordio

Translated title of the contribution: Cryptogenetic West syndrome: Cognitive outcome and neurophysiologic study in 28 patients followed from the onset

F. Ragona, M. Morbi, S. Binelli, I. De Giorgi, L. Obino, E. Freri, F. Panzica, S. Franceschetti, T. Granata

Research output: Contribution to journalArticle

Abstract

In West Syndrome, as for other epilepsies, under the heading "cryptogenic" are probably included both patients with a true age-dependent epilepsy with favorable outcome, and patients with hidden etiology.We report a prospective longitudinal study of 28 patients diagnosed at the onset of spasms as having "Cryptogenic West Syndrome" (CWS), followed up with these aims: 1) to describe clinical and cognitive outcome 2) to identify prognostic factors 3) to identify electroclinical features that can suggest the symptomatic origin of spasms. The diagnosis of CWS was done in patients that fulfilled all these criteria: presence of spasms in series, hypsarrythmia,no previous signs of brain damage, normal development until the onset of spasms, normal MRI. All the patients have been serially evaluated, for a mean period of 9,5 ± 4,7 years, by neurological examination, standardized cognitive assessment and EEG recordings; selected EEG recordings underwent signal analysis including autospectra, coherence and phase analyses in order to assess the changes during follow-up. The outcome was favourable in 14 patients: no seizure recurred and mental development was completely normal.On the contrary, 7 patients continued to have seizures and 7 children developed mental retardation. The evaluation of the electroclinical features at onset demonstrated that a favorable outcome is predictable in patients with: preserved visual function, symmetric spasms, diffuse ictal eeg and classic hypsarhythmia. Signal analysis demonstrated that in patients with persisting spasms or evolution in focal epilepsy, late seizure onset had the same lateralization as that assessed on ictal events associated with early spasms. The consistent lateralization suggests that the early ictal pattern may help to assume "non-visible" cortical lesion that will continue to have a leading effect on late ictogenesis.

Translated title of the contributionCryptogenetic West syndrome: Cognitive outcome and neurophysiologic study in 28 patients followed from the onset
Original languageItalian
Pages (from-to)97-99
Number of pages3
JournalBollettino - Lega Italiana contro l'Epilessia
Issue number138
Publication statusPublished - 2008

ASJC Scopus subject areas

  • Clinical Neurology

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