DR. SYED FARAAZ HUSSAIN

Abstract

Juvenile macular dystrophy is the term for several inherited and rare diseases that affect children and young adults, comprising of Pattern Dystrophy: Stargardt’s disease, Best disease and Juvenile Retinoschisis. Unfortunately, all three of the above are associated with severe visual loss with no proven treatment available till date. Here, we report a case of a fifteen year old who presented with gradual, progressive, painless loss of vision who was eventually diagnosed with bilateral macular dystrophy of Pattern type: Fundus pulverulentus associated with optic atrophy and rotational nystagmus which is very rare. Few cases with similar findings have been reported to be an initial presentation of Subacute Sclerosing Pan Encephalitis. Hence, it is prudent to thoroughly investigate such patients to rule out any underlying neurological cause