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.

Optic perineuritis (OPN) is an uncommon form of the orbital inflammatory disease targeting the optic nerve sheath, which is distinct from optic neuritis. It causes reduced visual acuity and ocular pain and is linked to infection or autoimmunity, including myelin oligodendrocyte glycoprotein-associated disease, but 30-50% are idiopathic. We report three cases of OPN with ocular scleritis and hypertrophic pachymeningitis occurring together or sequentially; in one, pachymeningitis extended from the optic sheath, in two, it was separate. Case 1: 54-year-old man with a history of ocular scleritis developed left ocular pain, diplopia, and visual loss. MRI showed left optic sheath hyperintensity and dural enhancement. Serology and CSF were unremarkable. Intravenous methylprednisolone (IVMP) followed by prednisolone (PSL) improved visual accuracy to 20/13, with no relapse at 10 months on tapering PSL. Case 2: 76-year-old man developed ocular scleritis, OPN, and hypertrophic pachymeningitis presenting as orbital apex syndrome. Blood examination showed pancytopenia. After the treatment of concomitant pneumonia, IVMP partially improved symptoms. Later, he was also diagnosed as myelodysplastic syndrome. He did not relapse without further immunotherapy. Case 3: 63-year-old woman with previous history of ocular scleritis developed acute right visual loss. MRI showed optic sheath and connected dural enhancement. IVMP followed by PSL treatment improved vision from no light perception to 20/125, without relapse at 7 months. These cases suggested possible shared connective-tissue autoimmunity, and warrant attention to the overlap of the three diseases.

This study aimed to describe the clinical, radiographic and laboratory characteristics of a cohort of patients diagnosed with anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibodies at two collaborating centers in Madrid (Spain). Besides the study of anti-MOG antibodies in serum and cerebrospinal fluid sample (CSF), this study highlights the results of the cytochemical study of CSF, kappa free light chain (κ-FLC) index and oligoclonal bands (OCBs).

On a rare day off from my duties as an ophthalmology resident, I wandered into a neighborhood bookstore and was recognized by a woman I had treated for optic neuritis in the emergency department. I didn't remember her, but she remembered everything. In that gap-between my routine and her life-altering night-I heard what the emergency department often muffles: how ordinary work for me can be extraordinary to the person living it. The piece follows our brief conversation to ask a simple question: how can I carry less and notice more, honoring meaning without slowing the work that must go on?

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an acquired demyelinating syndrome of the central nervous system mediated by MOG antibodies. Clinical phenotypes include optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. MOG antibody-associated meningitis (MOGAM) is a form of aseptic meningitis characterized by the presence of serum MOG antibodies. Parenchymal lesions are typically absent in MOGAM; however, some cases may progress to MOGAD. Leptomeningeal lesions are rarely detected on contrast-enhanced T1-weighted magnetic resonance imaging (MRI) but may be conspicuous on contrast-enhanced fluid-attenuated inversion recovery (FLAIR) imaging. We report a case of a 5-year-old boy who initially presented with a prolonged headache and fever. Contrast-enhanced FLAIR imaging revealed localized leptomeningeal enhancement (LME), and the patient was treated for suspected aseptic meningitis. Subsequently, additional parenchymal lesions developed, and serum MOG antibodies were detected, leading to a diagnosis of MOGAD. He achieved remission with corticosteroid therapy but experienced a relapse a year later, with LME localized to the same site on MRI. At recurrence, the contrast-enhanced T1-weighted image showed faint leptomeningeal enhancement, whereas contrast-enhanced FLAIR imaging clearly depicted LME. Intravenous methylprednisolone pulse therapy was promptly initiated, which resulted in resolution without progression to MOGAD. This is the first pediatric report of recurrent MOGAM with LME recurring at the identical site. Contrast-enhanced FLAIR imaging enabled early detection of LME at recurrence and facilitated timely corticosteroid therapy, which may have prevented progression to MOGAD.