PT  - JOURNAL ARTICLE
AU  - Daniela A. Pimentel Maldonado
AU  - Robert Lisak
AU  - Steven Galetta
AU  - Laura Balcer
AU  - Thomas Varkey
AU  - Andrew Goodman
AU  - Jennifer Graves
AU  - Michael Racke
AU  - Scott S. Zamvil
AU  - Scott Newsome
AU  - Elliot M. Frohman
AU  - Teresa C. Frohman
TI  - Recurrent Optic Neuritis and Perineuritis Followed by an Unexpected Discovery
AID  - 10.1212/NXI.0000000000200051
DP  - 2023 Jan 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e200051
VI  - 10
IP  - 1
4099  - http://nn.neurology.org/content/10/1/e200051.short
4100  - http://nn.neurology.org/content/10/1/e200051.full
SO  - Neurol Neuroimmunol Neuroinflamm2023 Jan 01; 10
AB  - 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.ACE=angiotensin-converting enzyme; anti-AQP4=aquaporin-4 antibody; BNS=Bing-Neel syndrome; BTK=Bruton tyrosine kinase; CBA=cell-based assay; CLL=chronic lymphocytic lymphoma; ED=emergency department; MMF=mycophenolate mofetil; MOG=myelin oligodendrocyte glycoprotein; MOGAD=MOG antibody disease; NMOSD=neuromyelitis optica spectrum disorder; ON=optic neuritis; RBC=red blood cell; VA=visual acuity; WBC=white blood cell; WM=Waldenstrom macroglobulinemia