Enfermedad de Leber

Es una distrofia retiniana grave que se caracteriza por:

  • Muy mala visión desde el nacimiento, fotofobia, nistagmus.
  • En la exploración se encuentra nistagmus de búsqueda o pendular, signo oculo-digital de Franceschetti (continuamente se aprietan los ojos con los puños), pupilas poco reactivas, fondo de ojo normal o anormal (papila pálida, fondo en sal y pimienta, retinopatía pigmentaria, colobomas maculares…) puede asociarse a alteraciones neurológicas y retraso mental.
  • Se hereda autosómica recesiva y raramente autosómica dominante. Los síntomas se aprecian desde el primer año de vida.

Hay una pérdida de fotorreceptores con degeneración de las células ganglionares de la retina y atrofia óptica.

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