Diabetes insipidus- diabetes mellitus- optic atrophy
Overview
Wolfram syndrome is the inherited association of childhood-onset diabetes mellitus and progressive-onset optic atrophy. All people affected by Wolfram syndrome have juvenile-onset diabetes mellitus and degeneration of the optic nerve (optic atrophy).
Symptoms
* Anxiety * Emotional liability * Depression * Hypotonia * Impaired problem solving
Diagnosis
Urine examination showed 4+sugar and a specific gravity varying from 1008 to 1012. Fasting blood sugar level was 266 mg%. X-ray optic foraminae was normal. Lateral view of X-ray skull showed a somewhat smaller sella turcica which was 6 mm deep and 8 mm anteroposteriorly. Audiogram revealed a high tone sensory neural deafness in both ears. Examination of parents and the only other sibling was found to be normal.