25-year-old male with history of ornithine transcarbamoylase deficiency as well as seizures, increased spasticity, and shuffling gate. There is no evidence of intracranial hemorrhage or mass. Diffusion weighted imaging of the anterior frontal lobes is somewhat limited due to extensive pneumatization of the frontal sinuses; however, no definite restricted diffusion is evident. There is slightly increased symmetric focal encephalomalacia affecting the bilateral frontal and parietal lobes in the juxtacortical zones, with associated gyriform cortical and subcortical increased T2 signal in these regions. These findings are superimposed on a background of diffuse leukoencephalomalacia, which is not substantially changed from the prior exam. The signal and morphology of the bilaterally hippocampi is unremarkable. There is no abnormal intracranial enhancement. There is marked ex vacuo dilatation of the lateral ventricles and moderate ex vacuo dilatation of the third ventricle, appearing similar to the prior examination. There is no midline shift or herniation. There are bilateral maxillary mucous retention cysts, increased from the prior exam. The major cerebral flow voids are intact. The orbits, skull, and scalp soft tissues are grossly unremarkable.
1.Slightly increased focal symmetric encephalomalacia affecting the bilateral frontal and parietal lobe cortical zones, suggestive of prior infarction, perhaps watershed in nature, superimposed on a background of diffuse leukoencephalomalacia. These findings may be related to the patient's ornithine transcarbamoylase deficiency.2.Unchanged ex vacuo dilatation of the lateral and third ventricles.3.No intracranial mass, hemorrhage or acute infarction.