Chronic relapsing inflammatory optic neuropathy (CRION) is a rare cause of recurrent optic neuritis characterized by steroid responsiveness and relapse upon treatment withdrawal. Because no definitive diagnostic test exists, the diagnosis is clinical and requires exclusion of more common demyelinating and antibody-mediated disorders. We describe a 45 year-old woman with a history of psoriasis, photodermatitis, and Hashimoto thyroiditis who presented with four episodes of severe left retro-orbital pain and progressive visual blurring over a three-year period, with the right eye remaining unaffected throughout. Each episode demonstrated left optic nerve enhancement on magnetic resonance imaging and responded promptly to intravenous corticosteroids. Extensive evaluation for multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody disease was consistently negative. A diagnosis of seronegative CRION was established based on the recurrent steroid-responsive clinical pattern. Long-term immunosuppressive therapy with mycophenolate mofetil, later transitioned to mycophenolic acid due to gastrointestinal intolerance, resulted in clinical stability during follow-up. One additional relapse occurred following a brief three-day interruption of mycophenolate for treatment of cellulitis. CRION should be considered in patients with recurrent optic neuritis when workup for demyelinating and antibody-mediated conditions is negative. Early recognition and initiation of long-term immunosuppressive therapy are important to reduce relapse frequency and prevent cumulative optic nerve damage.
Abstract
Case Reports
Journal Article
eng
42211618
Thakkar, Keval, and Chaitanya V. Amrutkar. "Seronegative Chronic Relapsing Inflammatory Optic Neuropathy." Cureus, vol. 18, no. 4, 2026, pp. e107823.
Thakkar K, Amrutkar CV. Seronegative Chronic Relapsing Inflammatory Optic Neuropathy. Cureus. 2026;18(4):e107823.
Thakkar, K., & Amrutkar, C. V. (2026). Seronegative Chronic Relapsing Inflammatory Optic Neuropathy. Cureus, 18(4), e107823. https://doi.org/10.7759/cureus.107823
Thakkar K, Amrutkar CV. Seronegative Chronic Relapsing Inflammatory Optic Neuropathy. Cureus. 2026;18(4):e107823. PubMed PMID: 42211618.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR
T1 - Seronegative Chronic Relapsing Inflammatory Optic Neuropathy.
AU - Thakkar,Keval,
AU - Amrutkar,Chaitanya V,
Y1 - 2026/04/27/
PY - 2026/04/27/accepted
PY - 2026/5/29/medline
PY - 2026/5/29/pubmed
PY - 2026/5/29/entrez
KW - chronic relapsing inflammatory optic neuropathy(crion)
KW - immunosuppression
KW - mycophenolate mofetile
KW - optic neuritis
KW - seronegative
SP - e107823
EP - e107823
JF - Cureus
JO - Cureus
VL - 18
IS - 4
N2 - Chronic relapsing inflammatory optic neuropathy (CRION) is a rare cause of recurrent optic neuritis characterized by steroid responsiveness and relapse upon treatment withdrawal. Because no definitive diagnostic test exists, the diagnosis is clinical and requires exclusion of more common demyelinating and antibody-mediated disorders. We describe a 45 year-old woman with a history of psoriasis, photodermatitis, and Hashimoto thyroiditis who presented with four episodes of severe left retro-orbital pain and progressive visual blurring over a three-year period, with the right eye remaining unaffected throughout. Each episode demonstrated left optic nerve enhancement on magnetic resonance imaging and responded promptly to intravenous corticosteroids. Extensive evaluation for multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody disease was consistently negative. A diagnosis of seronegative CRION was established based on the recurrent steroid-responsive clinical pattern. Long-term immunosuppressive therapy with mycophenolate mofetil, later transitioned to mycophenolic acid due to gastrointestinal intolerance, resulted in clinical stability during follow-up. One additional relapse occurred following a brief three-day interruption of mycophenolate for treatment of cellulitis. CRION should be considered in patients with recurrent optic neuritis when workup for demyelinating and antibody-mediated conditions is negative. Early recognition and initiation of long-term immunosuppressive therapy are important to reduce relapse frequency and prevent cumulative optic nerve damage.
SN - 2168-8184
UR - https://www.unboundmedicine.com/prime/citation/42211618/Seronegative_Chronic_Relapsing_Inflammatory_Optic_Neuropathy.
DB - PRIME
DP - Unbound Medicine
ER -
