Optical coherence tomography in central nervous system demyelinating diseases related optic neuritis

AIM: To compare the thickness of the peripapillary retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) among patients with various forms of optic neuritis (ON) and to identify whether any particular parameters or their thinning pattern can be used to distinguish the type of ON. METHODS: This prospective study was conducted at the Department of Ophthalmology, Faculty of Medicine, Siriraj Hospital, Thailand, between January, 2015 and December, 2016. We enlisted patients over 18 years of age with history of ON and categorized patients into 4 groups: 1) aquaporin 4 antibodies (AQP4-IgG) positive; 2) multiple sclerosis (MS); 3) myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) positive; 4) idiopathic-ON patients. Healthy controls were also included during the same study period. All patients underwent complete ophthalmological examination and spectral domain optical coherence tomography (OCT) imaging to analyze RNFL and GCIPL thickness after at least 3mo since the last episode of acute ON. The generalized estimating equation (GEE) models were used to compare the data amongst ON groups. RESULTS: Among 87 previous ON eyes from 57 patients (43 AQP4-IgG+ON, 17 MS-ON, 8 MOG-IgG+ON, and 19 idiopathic-ON), mean logMAR visual acuity of AQP4-IgG+ON, MS-ON, MOG-IgG+ON, and idiopathic-ON groups was 0.76±0.88, 0.12±0.25, 0.39±0.31, and 0.75±1.08, respectively. Average, superior, and inferior RNFL were significantly reduced in AQP4-IgG+ON, MOG-IgG+ON and idiopathic-ON eyes, relative to those of MS-ON. Differences were not statistically significant for RNFL or GCIPL between the AQP4-IgG+ON and MOG-IgG+ON groups, whereas visual acuity in MOG-IgG+ON was slightly, but not significantly, better (0.39 vs 0.76). Although RNFL thickness in MOG-IgG+ON was significantly reduced as compared to MS-ON, mean visual acuity and GCIPL were not different. CONCLUSION: Thinning of superior and inferior quadrants of RNFL are more commonly seen in MOG-IgG+ON and AQP4-IgG+ON. Long term visual acuity in MOG-IgG+ON is often better than AQP4-IgG+ON, whereas the structural change from OCT is comparable

The Acute Optic Neuritis Network (ACON): Study protocol of a non-interventional prospective multicenter study on diagnosis and treatment of acute optic neuritis

Optic neuritis (ON) often occurs at the presentation of multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). The recommended treatment of high-dose corticosteroids for ON is based on a North American study population, which did not address treatment timing or antibody serostatus. The Acute Optic Neuritis Network (ACON) presents a global, prospective, observational study protocol primarily designed to investigate the effect of time to high-dose corticosteroid treatment on 6-month visual outcomes in ON. Patients presenting within 30 days of the inaugural ON will be enrolled. For the primary analysis, patients will subsequently be assigned into the MS-ON group, the aquapotin-4-IgG positive ON (AQP4-IgG+ON) group or the MOG-IgG positive ON (MOG-IgG+ON) group and then further sub-stratified according to the number of days from the onset of visual loss to high-dose corticosteroids (days-to-Rx). The primary outcome measure will be high-contrast best-corrected visual acuity (HC-BCVA) at 6 months. In addition, multimodal data will be collected in subjects with any ON (CIS-ON, MS-ON, AQP4-IgG+ON or MOG-IgG+ON, and seronegative non-MS-ON), excluding infectious and granulomatous ON. Secondary outcomes include low-contrast best-corrected visual acuity (LC-BCVA), optical coherence tomography (OCT), magnetic resonance imaging (MRI) measurements, serum and cerebrospinal fluid (CSF) biomarkers (AQP4-IgG and MOG-IgG levels, neurofilament, and glial fibrillary protein), and patient reported outcome measures (headache, visual function in daily routine, depression, and quality of life questionnaires) at presentation at 6-month and 12-month follow-up visits. Data will be collected from 28 academic hospitals from Africa, Asia, the Middle East, Europe, North America, South America, and Australia. Planned recruitment consists of 100 MS-ON, 50 AQP4-IgG+ON, and 50 MOG-IgG+ON. This prospective, multimodal data collection will assess the potential value of early high-dose corticosteroid treatment, investigate the interrelations between functional impairments and structural changes, and evaluate the diagnostic yield of laboratory biomarkers. This analysis has the ability to substantially improve treatment strategies and the accuracy of diagnostic stratification in acute demyelinating ON

The Acute Optic Neuritis Network (ACON): Study protocol of a non-interventional prospective multicenter study on diagnosis and treatment of acute optic neuritis

Optic neuritis (ON) often occurs at the presentation of multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). The recommended treatment of high-dose corticosteroids for ON is based on a North American study population, which did not address treatment timing or antibody serostatus. The Acute Optic Neuritis Network (ACON) presents a global, prospective, observational study protocol primarily designed to investigate the effect of time to high-dose corticosteroid treatment on 6-month visual outcomes in ON. Patients presenting within 30 days of the inaugural ON will be enrolled. For the primary analysis, patients will subsequently be assigned into the MS-ON group, the aquapotin-4-IgG positive ON (AQP4-IgG+ON) group or the MOG-IgG positive ON (MOG-IgG+ON) group and then further sub-stratified according to the number of days from the onset of visual loss to high-dose corticosteroids (days-to-Rx). The primary outcome measure will be high-contrast best-corrected visual acuity (HC-BCVA) at 6 months. In addition, multimodal data will be collected in subjects with any ON (CIS-ON, MS-ON, AQP4-IgG+ON or MOG-IgG+ON, and seronegative non-MS-ON), excluding infectious and granulomatous ON. Secondary outcomes include low-contrast best-corrected visual acuity (LC-BCVA), optical coherence tomography (OCT), magnetic resonance imaging (MRI) measurements, serum and cerebrospinal fluid (CSF) biomarkers (AQP4-IgG and MOG-IgG levels, neurofilament, and glial fibrillary protein), and patient reported outcome measures (headache, visual function in daily routine, depression, and quality of life questionnaires) at presentation at 6-month and 12-month follow-up visits. Data will be collected from 28 academic hospitals from Africa, Asia, the Middle East, Europe, North America, South America, and Australia. Planned recruitment consists of 100 MS-ON, 50 AQP4-IgG+ON, and 50 MOG-IgG+ON. This prospective, multimodal data collection will assess the potential value of early high-dose corticosteroid treatment, investigate the interrelations between functional impairments and structural changes, and evaluate the diagnostic yield of laboratory biomarkers. This analysis has the ability to substantially improve treatment strategies and the accuracy of diagnostic stratification in acute demyelinating ON. Trial registration: ClinicalTrials.gov, identifier: NCT05605951

Rhinogenic Optic Neuritis Caused by Sphenoid Mucocele with Sinusitis

A 59-year-old male who presented with a nonspecific headache at the vertex, resembling retrobulbar optic neuritis, was treated as such but did not show any improvement. Although optic nerve compression from sphenoid mucocele was finally discovered, the delayed diagnosis and improper treatment led to a permanent visual loss. Optic neuritis could be caused by a common problem, “mucocele/sinusitis,” but might be easily overlooked in general practice. Rhinogenic optic neuropathy should, therefore, be considered in every case of optic neuritis whenever atypical presentation occurs or is unresponsive to high-dose steroid treatment

Efficacy and safety of rituximab in multiple sclerosis and neuromyelitis optica spectrum disorder

Abstract In Thailand, resource limitations lead many multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) patients to use off-label immunosuppressants. This study assesses the efficacy and safety of rituximab (RTX) with a CD19-based reinfusion regimen among Thai MS and NMOSD patients. A retrospective review of patients at the Faculty of Medicine Siriraj Hospital from January 1994 to April 2023 was conducted. The primary outcome assessed was the change in annualized relapse rate (ARR) for patients using RTX for over a year. Secondary outcomes included changes in the Expanded Disability Status Scale (EDSS) scores, time to the first relapse after RTX initiation for patients using RTX for over a year, and an evaluation of the safety of RTX. The study encompassed 36 MS and 39 NMOSD patients. A majority of patients (91.7% of MS and 79.5% of NMOSD) experienced no relapses during a median follow-up of 30 months (Interquartile range [IQR] 20–46) and 31 months (IQR 23–41), respectively. The median ARR significantly decreased in both MS (from 0.77 [IQR 0.42–1.83] to 0 [IQR 0–0], p < 0.001) and NMOSD (from 0.92 [IQR 0.68–1.78] to 0 [IQR 0–0.17], p < 0.001) patients after switching to RTX, with no difference between those following a fixed 6-month time point regimen and a CD19-based reinfusion regimen. Median EDSS scores improved significantly at the last follow-up visit in both groups. The mean time to the first subsequent relapse was 8.3 ± 3.0 months in MS and 6.8 ± 1.7 months in NMOSD. Mild adverse drug reactions occurred in 44% of patients. RTX effectively prevents relapses in Thai MS and NMOSD patients, with no observed serious adverse drug reactions