Background: The clinical implications of myelin oligodendrocyte glycoprotein autoantibodies (MOG-Abs) are increasing. Establishing MOG-Ab assays is essential for effectively treating patients with MOG-Abs. We established an in-house cell-based assay (CBA) to detect MOG-Abs to identify correlations with patients’ clinical characteristics. Methods: We established the CBA using HEK 293 cells transiently overexpressing fulllength human MOG, tested it against 166 samples from a multicenter registry of central nervous system (CNS) inflammatory disorders, and compared the results with those of the Oxford MOG-Ab-based CBA and a commercial MOG-Ab CBA kit. We recruited additional patients with MOG-Abs and compared the clinical characteristics of MOG-Ab-associated disease (MOGAD) with those of neuromyelitis optica spectrum disorder (NMOSD). Results: Of 166 samples tested, 10 tested positive for MOG-Abs, with optic neuritis (ON) being the most common manifestation (4/15, 26.7%). The in-house and Oxford MOG-Ab CBAs agreed for 164/166 (98.8%) samples (κ = 0.883, P < 0.001); two patients (2/166, 1.2%) were only positive in our in-house CBA, and the CBA scores of the two laboratories correlated well (r = 0.663, P < 0.001). The commercial MOG-Ab CBA kit showed one falsenegative and three false-positive results. The clinical presentation at disease onset differed between MOGAD and NMOSD; ON was the most frequent manifestation in MOGAD, and transverse myelitis was most frequent in NMOSD. Conclusions The in-house CBA for MOG-Abs demonstrated reliable results and can potentially be used to evaluate CNS inflammatory disorders. A comprehensive, long-term study with a large patient population would clarify the clinical significance of MOG-Abs.

Background: To report the clinical manifestations of non-arteritic anterior ischemic optic neuropathy (NAION) cases after coronavirus disease 2019 (COVID-19) vaccination in Korea. Methods: This multicenter retrospective study included patients diagnosed with NAION within 42 days of COVID-19 vaccination. We collected data on vaccinations, demographic features, presence of vascular risk factors, ocular findings, and visual outcomes of patients with NAION. Results: The study included 16 eyes of 14 patients (6 men, 8 women) with a mean age of 63.5 ± 9.1 (range, 43–77) years. The most common underlying disease was hypertension, accounting for 28.6% of patients with NAION. Seven patients (50.0%) had no vascular risk factors for NAION. The mean time from vaccination to onset was 13.8 ± 14.2 (range, 1–41) days. All 16 eyes had disc swelling at initial presentation, and 3 of them (18.8%) had peripapillary intraretinal and/or subretinal fluid with severe disc swelling. Peripapillary hemorrhage was found in 50% of the patients, and one (6.3%) patient had peripapillary cotton-wool spots. In eight fellow eyes for which we were able to review the fundus photographs, the horizontal cup/ disc ratio was less than 0.25 in four eyes (50.0%). The mean visual acuity was logMAR 0.6 ± 0.7 at the initial presentation and logMAR 0.7 ± 0.8 at the final visit. Conclusion Only 64% of patients with NAION after COVID-19 vaccination have known vascular and ocular risk factors relevant to ischemic optic neuropathy. This suggests that COVID-19 vaccination may increase the risk of NAION. However, overall clinical features and visual outcomes of the NAION patients after COVID-19 vaccination were similar to those of typical NAION.

PURPOSE: To describe the clinical characteristics and course of optic neuritis (ON) and its association with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Korea. METHODS: In this retrospective case series, 125 eyes of 91 Korean patients with ON were included. The medical documents of adult patients with ON were retrospectively reviewed. Patients were assigned into idiopathic ON, NMOSD, and MS groups according to the presence of an association with NMOSD or MS for subgroup analysis. Clinical characteristics, disease course, and visual and systemic prognosis were analyzed. RESULTS: During the mean follow-up of 3.7 years, 73 patients were diagnosed as idiopathic ON, 14 patients were diagnosed as NMOSD, and four patients developed definite MS. At the final visit, there were 13 (13%) eyes out of 100 eyes with idiopathic ON, nine (43%) eyes out of 21 eyes with NMOSD, and one (25%) eye out of four eyes with MS had a severe visual loss of 20 / 200 or less. The mean Expanded Disability Status Scale was 3.1 ± 1.5 in NMOSD and 1.8 ± 1.5 in the MS group at the final visit. In the NMOSD group, 50% of patients showed severe visual loss in at least one eye or were unable to ambulate without assistance at the final visit (5.3 ± 4.4 years after the initial episode of ON). CONCLUSIONS: Fourteen percent of patients showed positive results for NMO-immunoglobulin G test and 50% of patients with NMOSD showed a severe visual loss in at least one eye or were unable to ambulate without assistance. The proportion of MS was relatively low in Korean ON patients.
Adult ; Follow-Up Studies ; Humans ; Korea ; Multiple Sclerosis ; Neuromyelitis Optica ; Optic Neuritis ; Prognosis ; Retrospective Studies

PURPOSE: To investigate the clinical features of acute ophthalmoplegia associated with anti-GQ1b antibody in the Republic of Korea. METHODS: From January 2011 to July 2018, we retrospectively reviewed the medical records of all patients who tested positive for anti-GQ1b antibody, and who had been concurrently diagnosed with acute ophthalmoplegia at a tertiary hospital in the Republic of Korea. Ophthalmic and neurological features were carefully reviewed. Laboratory results including ganglioside antibody panels and treatment outcomes were analyzed. RESULTS: Thirty-three patients were enrolled in the study and 8 (24%) of them showed pure Miller-Fisher syndrome with all three cardinal symptoms of ophthalmoplegia, ataxia, and areflexia. One patient (3%) showed ophthalmoplegia, areflexia, and pharyngeal- cervical-brachial weakness without ataxia and 2 patients (6%) showed only ophthalmoplegia and pharyngeal-cervical- brachial weakness without ataxia and areflexia. Twenty-two patients (67%) showed only ophthalmoplegia without any other neurological symptom. Early immunosuppressant treatment was used to treat 17 patients, and observation only was conducted as the initial treatment for 16 patients (48.5%). Twenty-four patients (72.7%) showed improvement and 9 patients showed persistent strabismus and diplopia. The average duration until recovery was 2.52 ± 2.11 months. CONCLUSIONS The symptoms of acute ophthalmoplegia associated with anti-GQ1b antibody can manifest in various forms that overlap with clinical features of demyelinating neuropathy, so it is important to recognize such characteristics when treating acute ophthamoplegia.

PURPOSE: To report the long-term surgical outcomes of a muscle union procedure in patients with paralytic strabismus. METHODS: We retrospectively reviewed the medical records of 20 patients who underwent muscle union procedure for paralytic strabismus from September 2010 to March 2018. We analyzed the clinical results before and at the final visit after surgery. We also compared the outcomes of the first year after surgery between patients with sixth cranial nerve palsy, with third cranial nerve palsy and with medial rectus muscle rupture after endoscopic sinus surgery. RESULTS: The mean follow-up duration was 42 ± 20 months (12–79 months). The mean age at surgery was 40 ± 19 years (7–65 years). Eleven patients underwent surgery for sixth cranial nerve palsy, six patients underwent surgery for third cranial nerve palsy, and three patients underwent surgery for medial rectus rupture after endoscopic sinus surgery. The mean horizontal deviation at the primary eye position was 58 ± 19 prism diopters before surgery and decreased to 14 ± 17 prism diopters at the final visit. The success rate at the last visit was 60%. The mean horizontal deviation at postoperative 1 year was 4 ± 9 prism diopters in the sixth nerve palsy group and 26 ± 16 prism diopters in the third nerve palsy group (p = 0.002). The success rate was 91% in the sixth nerve palsy group and 33% in the third nerve palsy group at postoperative 1 year (p = 0.017). There were no complications during surgery or anterior segment ischemia for any of the patients. CONCLUSIONS A muscle union procedure had good long-term surgical outcomes in patients with paralytic strabismus, especially in patients with sixth cranial nerve palsy. However, in the case of third cranial nerve palsy or rupture of the medial rectus muscle, the effects were limited.

PURPOSE: The purpose of this study was to demonstrate whether the pattern of optic nerve enhancement in magnetic resonance imaging (MRI) can help to differentiate between idiopathic optic neuritis (ON), neuromyelitis optica (NMO), and multiple sclerosis (MS) in unilateral ON. METHODS: An MRI of the brain and orbits was obtained in patients with acute unilateral ON. Patients with ON were divided into three groups: NMO, MS, and idiopathic ON. The length and location of the abnormal optic nerve enhancement were compared for ON eyes with and without NMO or MS. The correlation between the pattern of optic nerve enhancement and the outcome of visual function was analyzed. RESULTS: Of the 36 patients with ON who underwent an MRI within 2 weeks of the onset, 19 were diagnosed with idiopathic ON, 9 with NMO, and 8 with MS. Enhancement of the optic nerve occurred in 21 patients (58.3%) and was limited to the orbital segment in 12 patients. Neither the length nor the location of the optic nerve enhancement was significantly correlated with visual functions other than contrast sensitivity or the diagnosis of idiopathic ON, MS, or NMO. Patients with greater extent of optic nerve sheath enhancement and more posterior segment involvement showed higher contrast sensitivity. CONCLUSIONS: Our data revealed that the pattern of optic nerve enhancement was not associated with diagnosis of idiopathic ON, NMO, or MS in Korean patients with unilateral ON. We believe further studies that include different ethnic groups will lead to a more definitive answer on this subject.
Brain ; Contrast Sensitivity ; Diagnosis ; Ethnic Groups ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Neuromyelitis Optica ; Optic Nerve* ; Optic Neuritis* ; Orbit

Pediatric diseases are important because diagnosis and care for these can be complex. Among them, specific diseases have been associated with ocular involvement. This review presents the ocular manifestations of various pediatric diseases relevant to the clinician. An array of ocular manifestations of hyperthyroidism, hypoparathyroidism, diabetes mellitus, porphyria, cystinosis, mucopolysaccharidosis, Wilson disease, juvenile idiopathic arthritis, systemic lupus erythematosus, Marfan syndrome, Weill-Marchesani syndrome are described. In this review we will review ocular manifestations of systemic pediatric diseases for comprehensive understanding of eye involvement. With this review, authors can recognize the ocular manifestations for diagnosis and management of pediatric systemic diseases.
Arthritis, Juvenile ; Cystinosis ; Diabetes Mellitus ; Diagnosis ; Hepatolenticular Degeneration ; Hyperthyroidism ; Hypoparathyroidism ; Lupus Erythematosus, Systemic ; Marfan Syndrome ; Mucopolysaccharidoses ; Pediatrics ; Porphyrias ; Weill-Marchesani Syndrome

PURPOSE: To analyze innervated myotendinous cylinders (IMCs) in the extraocular muscles (EOMs) of normal subjects and strabismic patients. METHODS: The rectus muscles of 37 subjects were analyzed. Distal myotendinous specimens were obtained from 3 normal subjects, 20 patients with acquired strabismus, 11 with infantile strabismus, and from 3 with congenital nystagmus, and were studied by using light microscopy. Some specimens (6 rectus muscles) were also examined by transmission electron microscopy. RESULTS: IMCs were found in the distal myotendinous regions of EOMs. The IMCs of patients with acquired strabismus showed no significant morphological alterations. However, significant IMCs alterations were observed at the distal myotendinous junction of patients with congenital strabismus and congenital nystagmus. CONCLUSIONS: This study supports the notion that IMCs in human EOMs function mainly as proprioceptors, along with effector properties, and a disturbance of ocular proprioceptors plays an important role in the pathogenesis of oculomotor disorder. We suggest that a proprioceptive feedback system should be stimulated and calibrated early in life for the development of binocular vision.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Microscopy, Electron, Transmission ; Middle Aged ; Oculomotor Muscles/*innervation/physiopathology/ultrastructure ; Proprioception/physiology ; Strabismus/*pathology/physiopathology ; Young Adult

PURPOSE: To provide quantitative data on the distribution of MyHCeom and compare the proportion of myosin heavy chain (MyHC) isoforms between the central and peripheral regions of human extraocular muscles (EOMs). METHODS: Medial rectus, lateral rectus, superior rectus, inferior rectus, superior oblique, and inferior oblique muscle samples were taken from three men with brain death. To examine the longitudinal distribution of myosin isoforms, the muscles were divided into central and peripheral portions of equal length. Electrophoresis and densitometry were used to quantify the distribution of MyHC isoforms. RESULTS: Electrophoresis of whole-muscle extracts of sampled EOMs revealed four MyHC bands that were identified as MyHCI, MyHCeom, MyHCIIa, and MyHCIIx. The proportion of MyHCeom was higher in the central region, whereas the proportion of MyHCIIa was higher in the peripheral region. The relative proportions of MyHCI, MyHCeom, and MyHCIIa were not significantly different among the EOMs. There was a tendency for higher levels of MyHCIIx in the inferior rectus muscle. CONCLUSIONS: The proportion of MyHCeom was higher in the central region of human EOMs. Further studies are needed to investigate the consequences of this distributional difference on the function of EOMs.
Brain Death ; Densitometry ; Electrophoresis ; Humans ; Male ; Muscles ; Myosin Heavy Chains ; Myosins ; Protein Isoforms

PURPOSE: To evaluate the effect of age wearing prescription glasses on changes in refractive error in accommodative esotropia. METHODS: We retrospectively reviewed the charts of 63 patients with accommodative esotropia. The patients were divided into three groups according to their age when glasses were prescribed. Changes of the refractive error between the three groups were compared. RESULTS: The mean age at the first visit was 4.1+/-2.0 years and the mean follow-up period was 5.7+/-2.7 years. In children that began wearing glasses before two years of age, the spherical equivalent (SE) refractive error initially increased, peaked 2.5 years after starting to wear glasses, and slowly decreased thereafter. In children who started wearing glasses after two years but not before four years of age the SE refractive error increased and peaked 1.5 years after starting to wear glasses. For children who began wearing glasses after four years of age the SE refractive error increased and peaked after one year. Changes in the refractive error in the youngest age group were significantly different from the other two groups (p=0.064). CONCLUSIONS: The age when glasses are prescribed may influence normal emmetropization in accommodative esotropia. However, further studies with longer follow-ups will be needed to determine the effect of wearing glasses on the final SE refractive error.
Child ; Esotropia ; Eyeglasses ; Follow-Up Studies ; Glass ; Humans ; Prescriptions ; Refractive Errors ; Retrospective Studies