Presentation
Squinting for many years associated with right-sided weakness.
Patient Data
Diffusely thinned-out bilateral optic nerves (R>L) and optic chiasma is seen. The septum pellucidum is not visualized.
There is an area of gliosis with cystic encephalomalacia and possible foci of chronic hemorrhage within left corona radiata and centrum semiovale. There is resultant ex vacuo dilatation of the left lateral ventricle and suggestion of porencephaly. This is likely a sequel of prior insult such as ischemia/infarct.
Thinning of the body and splenium of the corpus callosum is seen. Subtle partial atrophy of the left cerebral peduncle is seen. These findings suggest Wallerian degeneration.
Atrophy of left hippocampus is seen with hyperintensity, causing ex vacuo dilatation of temporal horn of left lateral ventricle, possibly sequel of prior insult / mesial temporal sclerosis.
Case Discussion
Overall above findings are suggestive of septo-optic dysplasia with porencephaly. Septo-optic dysplasia often presents with squint. It can lead to progressive vision loss and eventually blindness at times.