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A combined endpoint measure to define no evidence of disease activity (NEDA) is becoming increasingly appealing in the treatment of multiple sclerosis (MS). Initial efforts using a 3 parameter NEDA monitored disease activity using clinical and MRI lesion data. Later refinements, introduced more recently, include brain atrophy measurement and cognitive function analysis in defining NEDA-4. Using these stringent criteria clearly differentiated the usefulness of different disease modifying agents (DMDs) in achieving and sustaining NEDA over time. This in turn has changed attitudes and strategies in management of MS.

There is a dearth of epidemiological data about multiple sclerosis (MS) and related demyelinating disorders in India. In this study, a registry method was used for collecting data from secure sources and the index cases were verified Seventy nine patients were identified . A crude prevalence of 8.3/100,000 was obtained for MS and 6.2/100,000 for clinically-isolated syndrome (CIS). Age-standardized prevalence of MS relative to the world population was 7.8/100,000. Neuromyelitis optica (NMO) and spectrum disorders (NMOS) constituted 13.9% of all demyelinating disorders, with a prevalence of 2.6/100,000. Larger studies with more refined survey methodologies are required to understand the true prevalence of demyelinating disorders in India.

Serum antibodies directed against myelin oligodendrocyte glycoprotein (MOG) are found in patients with acquired CNS demyelinating syndromes that are distinct from multiple sclerosis and aquaporin-4-seropositive neuromyelitis optica spectrum disorder. Based on an extensive literature review and a structured consensus process, we propose diagnostic criteria for MOG antibody-associated disease (MOGAD) in which the presence of MOG-IgG is a core criterion. According to our proposed criteria, MOGAD is typically associated with acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis, and is less commonly associated with cerebral cortical encephalitis, brainstem presentations, or cerebellar presentations. MOGAD can present as either a monophasic or relapsing disease course, and MOG-IgG cell-based assays are important for diagnostic accuracy. Diagnoses such as multiple sclerosis need to be excluded, but not all patients with multiple sclerosis should undergo screening for MOG-IgG. These proposed diagnostic criteria require validation but have the potential to improve identification of individuals with MOGAD, which is essential to define long-term clinical outcomes, refine inclusion criteria for clinical trials, and identify predictors of a relapsing versus a monophasic disease course.

Recent advances in the understanding of neuromyelitis optica spectrum of disorders (NMOSD) have expanded. Diagnostic criteria have changed over the years. The clinical spectrum of disease manifestations are now understood to include sites outside the spinal cord and optic nerve. A variety of autoimmune diseases may coexist with this disorder. Non neurological manifestations have been recently reported. Novel biomarkers other than aquoporin 4 Immunoglobulin G (anti AQP4-IgG) have been discovered which may have clinical relevance. In particul myelin associated oligoglycoprotein antibody (MOG-Ab) associated NMOSD may be relatively benign. This update describes some of these new findings highlighting the clinical manifestations, biomarkers associated with the disease and magnetic resonance imaging characteristics of brain and spinal cord.

BackgroundThough considered optimal, live cell-based assay (LCBA) is often unavailable for the diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) in resource-poor regions. This study was undertaken to determine the agreement between LCBA and the widely available fixed cell-based assay (FCBA), for recommending testing guidelines within our region.MethodAll consecutive patients in our registry with a MOGAD phenotype were tested. The results from a commercially available FCBA (Euroimmun, Germany) were compared with a validated “in-house” LCBA. Clinical and MRI data were available for correlation.ResultsAmong the 257 patient samples tested, 118 (45.9%) were positive by FCBA titre ≥1: 10 and or LCBA titres ≥1: 160 titre and 139 samples were negative. There was robust agreement between the two assays (agreement 98.8%, Cohen’s kappa 0.98 [95% CI- 0.95-1.00], Spearman correlation 0.97 (p < 0.0001). Among five discordant samples, four had clinical and or MRI data which supported an alternate diagnosis. There was a modest correlation between assay titres, particularly for samples with titres ≥ 1:100 in FCBA (Spearman’s Rho 0.26, p 0.005). Thirty samples were positive by FCBA at < 1:100 titre and included 1:80 (20),1:40(7) and 1:10 (3) titres. Among them, 80% had clear positive titres when tested by LCBA.ConclusionThe FCBA tested with serum dilutions of 1:10 was highly predictive of MOGAD in our study and compared well with our “in-house” LCBA. The current recommendations for testing at higher dilutions need to be re-examined in light of our findings. The results of our study should ideally be replicated in a larger dataset but at the same time provide some guidance for the accurate diagnosis of MOGAD in resource-poor settings.

( persists after colonizing the gut in childhood, and potentially regulates host immune system through this process. Earlier studies have shown that infection in childhood, may protect against MS in later life. Such an association was not seen with AQP4-IgG positive NMOSD, while the association with MOGAD is unclear. To evaluate frequency of among patients with MOGAD, MS, NMOSD and matched controls and its effect on disease course. To ascertain whether childhood socio economic factors were linked to prevalence of infection. In all, 99 patients diagnosed to have MOGAD, 99 AQP4 IgG+ NMOSD, 254MS and 243 matched controls were included. Patient demographics, diagnosis, age at disease onset, duration and the last recorded expanded disability status scale (EDSS) were obtained from our records. Socioeconomic and educational status was queried using a previously validated questionnaire. Serum IgG was detected using ELISA kits (Vircell, Spain). Frequency of IgG was significantly lower among MOGAD (28.3% vs 44%, p-0.007) and MS (21.2% vs 44%, p-0.0001) but not AQP4-IgG+ NMOSD patients (42.4% vs 44%, p-0.78) when compared to controls. Frequency of IgG in MOGAD & MS patients combined (MOGAD-MS) was significantly lower than those with NMOSD (23.2% vs 42.4%, p- 0.0001). Seropositive patients with MOGAD- MS were older (p-0.001. OR -1.04, 95% CI- 1.01- 1.06) and had longer disease duration (p- 0.04, OR- 1.04, 95% CI- 1.002- 1.08) at time of testing. Educational status was lower among parents/caregivers of this study cohort (p- 0.001, OR -2.34, 95% CI- 1.48-3.69) who were IgG In developing countries infection may be a significant environmental factor related to autoimmune demyelinating CNS disease. Our preliminary data suggests that may exert a differential influence - a largely protective role for MS-MOGAD but not NMOSD and may influence disease onset and course. This differential response maybe related to immuno-pathological similarities between MOGAD and MS in contrast to NMOSD. Our study further underscores the role of as a surrogate marker for poor gut hygiene in childhood and its association with later onset of autoimmune diseases.

Introduction of international consensus criteria (2015 IPND criteria) for neuromyelitis optica spectrum disorders (NMOSDs) has improved diagnostic accuracy for aquaporin 4 (AQP4)-IgG-associated and seronegative NMOSDs. This study aimed to review relevant publications related to the incidence and prevalence of NMOSDs and provide an updated review of the global epidemiology of NMOSDs in the light of new diagnostic criteria. A comprehensive literature search was performed from January 2015 to June 2021 by using appropriate keywords in PubMed, Scopus, and Web of Science. Relevant papers that fulfilled inclusion criteria were shortlisted and reviewed. Twenty-one papers were selected for this review. Incidence of NMOSDs was 0.04-0.25/100,000 in predominantly white and 0.34-1.31/100,000 in nonwhite populations. Prevalence was 0.70-1.91/100,000 in white and 0.86-4.52/100,000 in nonwhite populations. The 2015 IPND criteria significantly improved the incidence and prevalence rates for NMOSDs when compared to the Wingerchuk 2006 criteria. Incidence of MOG-IgG-associated NMOSDs was 0.12-0.13/100,000, with prevalence in children 0.03-1.4/100,000 and in adults 0.65-2/100,000. In this systematic review, studies that used uniform diagnostic criteria and confirmed cases after testing for AQP4-IgG were included. The prevalence of NMOSDs was estimated to be <5/100,000 globally. A clear bias was seen in favor of nonwhite and indigenous populations. This review highlights the need for prospective population-based epidemiological studies and the importance of surveys in nonwhite populations around the globe.

Multiple sclerosis (MS) is less prevalent among Indians when compared to white populations. Genetic susceptibility remaining the same it is possible that environmental associations may have a role in determining disease prevalence. To determine whether childhood infections, vaccination status, past infection with Helicobacter pylori (H.pylori), diet, socioeconomic and educational status were associated with MS. 139 patients and 278 matched control subjects were selected. A validated environmental exposure questionnaire was administered. Estimation of serum H.pylori IgG antibody was done by ELISA. Patients and controls were genotyped for HLA-DRB1*15:01. In our cohort a significant association was seen with measles (p < 0.007), vegetarian diet (p < 0.001, higher educational status (p < 0.0001) and urban living (p < 0.0001). An inverse relationship was seen with H.Pylori infection and MS (p < 0.001). Measles infection (OR 6.479, CI 1.21-34.668, p < 0.029) and high educational status (OR 3.088, CI 1.212-7.872, p < 0.018) were significant risk factors associated with MS. H.pylori infection was inversely related to MS (OR 0. 319, CI 0.144- 0.706, p < 0.005). Environmental influences may be important in determining MS prevalence.

Objectives We investigated the clinical characteristics and treatment response in myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease and looked for evidence of subclinical disease. Methods We prospectively evaluated the frequency and pattern of relapse, tested afferent visual function and monitored treatment response in 42 south Asian patients from a single centre. Results Eighteen patients (42.9%) had monophasic and 24 (57.1%) a relapsing course. Disease duration was longer (P<0.02) in those with a relapsing course. Median time to the second attack was prolonged (P<0.04) in patients with recurrent transverse myelitis when compared with neuromyelitis optica spectrum disorder and recurrent optic neuritis. Thirteen out of 17 patients (76.5%) initially presenting with optic neuritis developed recurrent optic neuritis later. After the first attack of transverse myelitis, 17 out of 22 (77.3%) had disease confined to the spinal cord. Optical coherence tomography detected peripapillary retinal nerve fibre layer thickness (P<0.05) and macular ganglion cell complex volume (P<0.005) abnormalities in seven out of 10 (70.0%) patients without clinical optic neuritis. Immunosuppressants induced remission in 17 out of 22 (77.3%) patients during a median follow-up of 48 months and the median Expanded Disability Status Score was 1 (range 1–10). Conclusion Our study highlighted the tendency for stereotypical attacks in MOG-IgG-associated disease, heterogeneity in clinical course among subtypes, subclinical visual impairment and the need for early and sustained immunosuppressive therapy.

Background: The possible interaction between genetic and immunological factors in influencing clinical course of multiple sclerosis (MS) has not been studied previously in Indian population. Aim: In this study we evaluated the association of HLA alleles and OCB in affecting clinical course and disability of MS. Methods: Clinical and demographic features of 145 MS patients who had CSF oligoclonal bands (OCB) tested by isoelectric focussing technique were analyzed, disability status estimated, and HLA DRB1 alleles were genotyped. Results: OCBs were positive in 53.8% (78/145) of all MS cases. Patients with CSF OCB had more frequent relapses and an association with HLA DRB1*15. Early disease onset and a high annualized relapse rate was associated with HLA DRB1*03 allele. A relapsing remitting course for MS was seen with HLA DRB1*03 & 15 while a progressive disease was associated with DRB1*01. Presence of both OCB and HLA DRB1*13 was significantly associated with disability in this cohort. Conclusion: The results of our study suggest that an interaction between immunological and genetic factors may influence disease onset, course, and disability in MS.