Cerebral Edema Leading to Subfalcine and Uncal Herniation in a Patient With Retinal Vasculopathy With Cerebral Leukoencephalopathy and Systemic Manifestations

Deterioration of a patient's state of consciousness is among the most concerning signs encountered in clinical practice. The evaluation of this finding carries a broad initial differential diagnosis and must account for any relevant medical history. We describe the case of a 41-year-old male with known retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) who presented with progressive mental status decline and acute onset intractable headache. Head computed tomography (CT) revealed extensive vasogenic edema, resulting in right to left shift of 11 millimeters at the level of the lateral ventricles, with associated uncal and subfalcine herniation. He was treated with a 5-day course of methylprednisolone, leading to resolution of his lethargy and headache. Follow up neuroimaging with magnetic resonance (MRI) brain demonstrated interval improvement with the midline shift reduced to 3 millimeters after completion of high dose corticosteroids. Neurosurgical intervention was considered, but ultimately not required given his improvement. This case describes the management of life-threatening cerebral edema as a complication of RVCL-S disease progression. Due to the rarity of this disease, there are no standardized guidelines for treatment and the care for such patients relies on expert opinion, case studies, and extrapolation of principles learned from related conditions. Our intention is that the reporting of this case will contribute to the limited body of literature and aid those affected by this condition.