First case of neurofibromatosis with posterior reversible encephalopathy syndrome showing spinal cord involvement

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a well-documented pathology of the brain in systemic upsets. Majority of PRES cases present with edema in the cerebrum, most commonly in the territory of posterior circulation. It has been reported to show spinal cord involvement in a rare subgroup known as PRES with spinal cord involvement (PRES-SCI), with very limited existing literature even in adult patients. Our institution recently encountered a pediatric case with neurofibromatosis type I (NF 1) showing PRES with extensive reversible spinal cord changes. This case illustrates the features of this rare entity in the pediatric group of patients, and is the first reported case in NF 1 patients.

Keywords

• hypertensive crisis
• neurofibromatosis
• pediatric posterior reversible encephalopathy syndrome
• posterior reversible encephalopathy syndrome
• spinal cord edema