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Five-Year-Old Boy With Behavioral Changes and Papilledema

Ram, Radha, MD; Jones, Jeremy, Y., MD; Mohila, Carrie, A., MD, PhD; Shah, Veeral, S., MD, PhD

Section Editor(s): Miller, Neil R. MD; Rucker, Janet MD

Journal of Neuro-Ophthalmology: March 2018 - Volume 38 - Issue 1 - p 75–80
doi: 10.1097/WNO.0000000000000562
Clinical-Pathological Case Study

Abstract: A 5-year-old boy had initial symptoms of behavioral changes, nausea, vomiting, headache, weight loss, and progressive vision failure. Brain MRI revealed abnormal signal intensity in both optic nerves, the optic chiasm, the right medial temporal lobe, and tissues surrounding the right supraclinoid internal carotid artery with associated leptomeningeal and spinal cord enhancement. After nondiagnostic dural and spinal arachnoid biopsies, a temporal lobe biopsy was diagnostic for a rare malignant peripheral nerve sheath tumor.

Departments of Ophthalmology (RR, VSS), Radiology (JYJ), and Pathology (CAM), Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.

Address correspondence to Veeral S. Shah, MD, PhD, Department of Ophthalmology, Baylor College of Medicine, Texas Children's Hospital, 6701 Fannin, Suite 610.25 Houston, Texas 77030; E-mail:

Supported in part by the clinical programs at Texas Children's Hospital-Baylor College of Medicine, Houston, TX.

The authors report no conflicts of interest.

© 2018 by North American Neuro-Ophthalmology Society