What do we need to know about optic neuritis in children?丨WOC 2022

Editor's note: Optic neuritis (ON) refers to optic neuropathy that can hinder the conduction function of the optic nerve and cause a series of changes in visual function. Children's ON is one of the special types. Although the incidence is less common than that of adults, its clinical features are different from those of adults. ON. At present, there is still a lack of standardized treatment standards in the treatment of children with ON, and doctors treat patients according to their own clinical experience. At this World Ophthalmology Congress (WOC 2022), Dr. STACY PINELES from the United States shared her experience in treating children with ON based on her clinical experience and some research results. Treatment of ON cases in children Dr. STACY PINELES first introduced the topic with the treatment process of a case of ON in children. The patient, an 8-year-old girl, complained of decreased visual acuity accompanied by eye pain and eye movement pain for one week, and the decrease in binocular vision had worsened in the past 4 days. The patient had no history of surgery, glaucoma, trauma and autoimmune diseases. The child has not been exposed to alcohol or tobacco, has only one dog at home, and has not been bitten by lice or cats recently. The child had no history of lung disease, arthralgia, or rash, nor had there been previous episodes of local numbness, loss of bowel or bladder function, vertigo, or other focal neurological symptoms. The results of the eye examination are shown below: After magnetic resonance imaging (MRI), lumbar puncture, and laboratory tests (shown below), the child was diagnosed with ON in children. After glucocorticoid therapy, the child's symptoms were relieved and his vision improved. What new evidence is there in the field of pediatric ON diagnosis and treatment? Dr. STACY PINELES then introduced the findings of the ON study in children - PON1 in detail. PON1 is the first prospective study to look at visual outcomes in children with ON, and 44 children were included in the study for 22 months and followed up for 2 years. The patient's visual acuity was the primary outcome, and the results were also combined with MRI findings, laboratory findings, recurrence/re-deterioration, and neurological diagnosis. The PON1 study concluded that ON in children is often associated with neurological syndromes and that myelin oligodendrocyte glycoprotein (MOG) is common in ON subjects in approximately 54%. Distant vision improved significantly in most patients with treatment, with no significant change between 6 months and 2 years. However, the predictive value of MRI cannot be judged because the follow-up loss rate is too large. MOG is a myelin protein on the outer surface of the myelin sheath and is currently considered a suspected antigen for demyelinating diseases of the central nervous system. Patients participating in the PON1 study were asked to participate in a subgroup study to test for antibodies to MOG, which was done at the Mayo Clinic. Thirteen patients agreed to have their sera tested, and 54% (7/13) of them had positive results. MOG is commonly seen in patients with acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD), and optic neuritis. Results of a 2020 prospective study of 239 children with demyelinating syndrome showed that MOG was seen in about 50% of subjects, ON was a very common presentation, bilateral edema, optic nerve sheath, and peripheral fat were seen. MRI image enhancement is fairly common and specific. MOG-positive disease generally responds well to glucocorticoids, with significant visual recovery and a good prognosis. Recurrent cases may require immunotherapy (no randomized controlled trials are currently available), often with gamma globulin, but also with azathioprine, mycophenolate mofetil, or rituximab. Also of concern is NMOSD, an inflammatory central nervous system disease characterized by severe immune-mediated demyelination and axonal damage, primarily targeting the ON and spinal cord. The disease has specific antibodies to aquaporin-4 (AQP4), and its characteristic symptoms include acute bilateral ON, rhabdomyositis, and rarely, multiple sclerosis (MS)-like brain damage. Therefore, diagnosis is very important, and if NMOSD patients are treated according to MS, the patient's condition may be aggravated. The diagnosis and treatment of ON in children suggests that if a child is clinically suspected of having ON, an MRI of the brain and orbit should be done first. The longer the lesion time, the greater the possibility of NMO or MOG positive, and the more obvious some features on the MRI. Lumbar puncture can assess biomarkers of MS and can be used as evidence to assess infection. If NMO is suspected, the patient is treated with corticosteroids and plasma exchange; if NMO is definitely absent, intravenous corticosteroids can be administered. Dr. STACY PINELES finally shared her personal treatment experience, she recommended MOG and NMO testing for all ON patients. In most cases, she will have patients follow up every 3 months after treatment and repeat MRIs 2 years later.