Early development in Dravet syndrome; visual function impairment precedes cognitive decline

Daniela Chieffo, Daniela Ricci, Giovanni Baranello, Diego Martinelli, Chiara Veredice, Donatella Lettori, Domenica Battaglia, Charlotte Dravet, Eugenio Mercuri, Francesco Guzzetta

Research output: Contribution to journalArticlepeer-review


Aim of the study was to describe prospectively the early neuropsychological evolution including the first pre-cognitive stages of the Severe Myoclonic Epilepsy in Infancy (SMEI) or Dravet syndrome. Five cases, four of whom since before a diagnostic evidence of the Dravet syndrome, were followed up. Full clinical assessment including developmental, visual function and behaviour assessments were serially performed. In four cases, a variable onset age of cognitive decline assessed with developmental scales was preceded some months before by an impairment of visual function; the remaining patient during all the course of follow-up till 51 months of age showed a normal development without visual impairment. A cognitive decline with variable onset was generally confirmed in Dravet syndrome. The previous early impairment of visual function seems to herald the cognitive decline and provides useful prognostic information; furthermore, it possibly suggests some clues for a better understanding of the mechanisms of cognitive deterioration in this syndrome.

Original languageEnglish
Pages (from-to)73-79
Number of pages7
JournalEpilepsy Research
Issue number1
Publication statusPublished - Jan 2011


  • Dravet syndrome
  • Early development
  • Visual function

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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