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

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

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

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.
Lingua originaleEnglish
pagine (da-a)73-79
Numero di pagine7
RivistaEpilepsy Research
Volume93
DOI
Stato di pubblicazionePubblicato - 2011

Keywords

  • Child, Preschool
  • Cognition Disorders
  • Disease Progression
  • Epilepsies, Myoclonic
  • Female
  • Humans
  • Longitudinal Studies
  • Male
  • Vision Disorders

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