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.
Original language | English |
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Pages (from-to) | 73-79 |
Number of pages | 7 |
Journal | Epilepsy Research |
Volume | 93 |
Issue number | 1 |
DOIs | |
Publication status | Published - Jan 2011 |
Keywords
- Dravet syndrome
- Early development
- Visual function
ASJC Scopus subject areas
- Clinical Neurology
- Neurology