Posterior Ischemic Optic Neuropathy Because of Hematologic Malignancy

Abstract

O ptic neuropathy because of infiltration by leukemia or lymphoma is rare, and confirmed through optic nerve MRI, demonstrating enlargement, increased T2 signal, and enhancement of the optic nerve and leukemia or lymphoma cells in the cerebrospinal fluid (CSF) or on biopsy (1). We present 2 cases of posterior ischemic optic neuropathy (PION) because of meningeal leukemia or lymphoma infiltrating the optic nerve sheath, describe characteristic MRI findings, and propose a pathophysiologic cause (see Supplemental Digital Content, Text, http://links.lww.com/ WNO/A653, for more background information).The first patient was a 29-year-old woman with relapsed acute B-cell lymphoblastic leukemia. She experienced complete vision loss in the right eye with right-sided headache, and difficulty focusing out of her left eye, without other neurologic or visual symptoms. Vision was no light perception (NLP) in the right eye and 20/400 in the left eye, with a right-sided afferent pupillary defect (APD). The right optic disc was pale. There were no other findings on ophthalmic examination. Orbital MRI revealed an enlarged right optic nerve with increased T2 signal. After contrast administration (Fig. 1A), there was enhancement in the perioptic subarachnoid space, but no enhancement of the optic nerve itself. Diffusion-weighted imaging (DWI) (Fig. 1B) revealed restricted diffusion in the optic nerve, confirmed on apparent diffusion coefficient (ADC) map (Fig. 1C). Lumbar puncture revealed B lymphoblasts in the CSF. The patient refused further treatment, was transferred to hospice care, and died.The second patient was a 66-year-old woman with relapsed large B-cell lymphoma. She developed complete loss of vision in the right eye over 3 days, with right eye pain, and no other neurologic or visual symptoms. Vision