A variant form of septo-optic-pituitary dysplasia (SOPD) complicated with acute optic neuritis

We herein report a case of a variant form of septo-optic-pituitary dysplasia (SOPD). A 40-year-old man was admitted due to sudden occurrence of left blurred vision and lasting polyuria. He showed short statue of height of 144 cm and the neurological examination revealed hypesthesia of the left trigeminal nerve and temporal pallor in the left fundus oculi. Brain MR imaging demonstrated agenesis of the septum pellucidum and hypoplasia of the corpus callosum with subcortical spotty lesions, but optic nerve hypoplasia was not detected. The left eye showed a prolonged P100 latency of pattern reversal VEPs. He was diagnosed as having hypopituitarism since growth hormone-releasing factor did not stimulate growth hormone secretion and restriction of water-intake did not induce secretion of antidiuretic hormone. Thus we regarded this case as a variant form of SOPD. The mutation of HESX 1 gene, however, was not detected in the case. P100 of the left eye showed a reduction in latency four months after discharge. This case was considered to be a variant form of SOPD complicated by acute optic neuritis.