Wolfram syndrome - clinical and diagnostic details.

Wolfram syndrome, a rare genetic disorder is characterized by juvenile onset diabetes mellitus and optic atrophy. We describe two cases of wolfram syndrome belonging to same family; 25 year old female and her only 15 year old brother. In female, diabetes mellitus and optic atrophy were manifested in 1(st) decade, diabetes insipidus in 2(nd) decade and hypoacusis at the age of 25 years. Her ophthalmic evaluation revealed bilateral optic atrophy, decreased vision and peripheral constriction of visual field. However she didn't have any renal dysfunction which is also considered to be one of the features of the syndrome. Though associated psychiatric features are later manifestations of the syndrome she was admitted with alleged suicidal consumption at the age of 25 years. The brother was asymptomatic except for the diabetes mellitus and insipidus.