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

Domenica Immacolata Battaglia, D Chieffo, D Ricci, G Baranello, D Martinelli, C Veredice, D Lettori, C Dravet, E Mercuri, F. 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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