DR. SAMEER DASH

Dr. Rutayani Dash, Dr. DASH SUCHITRA

Abstract

Stargardt disease is considered most common form of juvenile macular dystrophy accounting for vision loss in young indivisuals under the age of 50 years .It has a characteristic foveal atropy surrounded by discrete yellow flecs in the RPE level. The disease entity is primarily autosomal recessive. We document occurence of this typical disease in 3 generations of a family where affected member being materanal grandmother,mother &mothers sister[male counter part being normal] and all 3 siblings of the present generation.Pedigree analysis led us to discover the variation of inheritance from the prototype autosomal dominant character.Further evaluation and workup done & Patients were counselled. & low vision aid was advised.

This case report gains importance because of its autosomal dominant character.