Recurrent Optic Neuritis and Perineuritis Followed by an Unexpected Discovery: From the National Multiple Sclerosis Society Case Conference Proceedings

Daniela A Pimentel Maldonado; Robert Lisak; Steven Galetta; Laura Balcer; Thomas Varkey; Andrew Goodman; Jennifer Graves; Michael Racke; Scott S Zamvil; Scott Newsome; Elliot M Frohman; Teresa C Frohman

doi:10.1212/NXI.0000000000200051

Recurrent Optic Neuritis and Perineuritis Followed by an Unexpected Discovery: From the National Multiple Sclerosis Society Case Conference Proceedings

Neurol Neuroimmunol Neuroinflamm. 2022 Nov 10;10(1):e200051. doi: 10.1212/NXI.0000000000200051. Print 2023 Jan.

Affiliations

¹ From the Department of Neurology (D.A.P.M., S.N.), Johns Hopkins Hospital, Baltimore, MD; Department of Neurology (R.L.), Wayne State University, Detroit, MI; Departments of Neurology (S.G., L.B.), Population Health, and Ophthalmology, NYU Grossman School of Medicine; Department of Neurology (T.V.), Dell Medical School, The University of Texas at Austin; Department of Neurology (A.G.), University of Rochester, NY; Department of Neurosciences (J.G.), University of California at San Diego; Quest Diagnostics (M.R.), Secaucus, NJ; Department of Neurology and Program in Immunology (S.S.Z.), University of California San Francisco; and Distinguished Senior Fellows (Sabbatical) Neuroimmunology Laboratory of Professor Lawrence Steinman (E.M.F., T.C.F.), Stanford University School of Medicine, Palo Alto, CA.
² From the Department of Neurology (D.A.P.M., S.N.), Johns Hopkins Hospital, Baltimore, MD; Department of Neurology (R.L.), Wayne State University, Detroit, MI; Departments of Neurology (S.G., L.B.), Population Health, and Ophthalmology, NYU Grossman School of Medicine; Department of Neurology (T.V.), Dell Medical School, The University of Texas at Austin; Department of Neurology (A.G.), University of Rochester, NY; Department of Neurosciences (J.G.), University of California at San Diego; Quest Diagnostics (M.R.), Secaucus, NJ; Department of Neurology and Program in Immunology (S.S.Z.), University of California San Francisco; and Distinguished Senior Fellows (Sabbatical) Neuroimmunology Laboratory of Professor Lawrence Steinman (E.M.F., T.C.F.), Stanford University School of Medicine, Palo Alto, CA. elliot.frohman@austin.utexas.edu.

Abstract

We describe a woman with a history of relapsing acute optic neuritis and perineuritis. Testing failed to confirm a specific diagnosis; hence, she was diagnosed with seronegative neuromyelitis optica spectrum disorder and treated with the immunotherapy rituximab, later in conjunction with mycophenolate mofetil. She achieved a durable remission for 9 years until she presented with paresthesia affecting her left fifth digit, right proximal thigh, and left foot, while also reporting a 25-pound weight loss over the prior 3 months. New imaging demonstrated a longitudinally extensive and enhancing optic nerve, in conjunction with multifocal enhancing lesions within the spinal cord, in a skip-like distribution. The differential diagnosis is discussed.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Female
Humans
Magnetic Resonance Imaging
Multiple Sclerosis* / complications
Multiple Sclerosis* / diagnosis
Multiple Sclerosis* / drug therapy
Neuromyelitis Optica* / complications
Neuromyelitis Optica* / diagnosis
Neuromyelitis Optica* / drug therapy
Optic Nerve / pathology
Optic Neuritis* / diagnosis
Optic Neuritis* / drug therapy