Prevalence Of Neuromyelitis Optica Spectrum Disorder Research Articles

Prevalence of neuromyelitis optica spectrum disorder in Colombia: Analysis of the official Ministry of Health administrative registry.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare entity with severe inflammatory demyelinating events of the central nervous system with debilitating sequelae. Its global prevalence ranges between 0.5 and 4/100,000 individuals, with variations by region and ethnicity. Latin America lacks epidemiological data on the disease, and Colombian prevalence is unknown. Prevalence of NMOSD in Colombia was estimated between 2017 and 2021 using the official Ministry of Health administrative database (SISPRO). This is an observational, cross-sectional retrospective study, using data between January 2017 and December 2021 in the SISPRO database using the International Classification of Disease code for NMOSD G36.0. Prevalence by gender, age and geographic distribution was estimated using official government statistics for 2019. World Health Organization (WHO) standard population was used to adjust using the direct method. 2,650 patients were diagnosed with NMOSD; the average age was 44.9 years with an overall unadjusted prevalence of 5.3/100,000 individuals, higher for females (7.8) than for males (2.8). No significant changes (from 5.3 to 5.4) were seen after adjusting to the WHO standard. According to this study Colombia has one of the highest prevalence rates of NMOSD in Latin America, further studies are needed to elucidate the contributing factors.

Effectiveness of rituximab versus oral immunosuppressive therapies in neuromyelitis optica spectrum disorder in a racially diverse cohort of subjects: A single-center retrospective study.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune, inflammatory disorder characterized by severe relapses and high level of disability. In clinical trials of NMOSD, Black patients are under-represented, < 12%, compared to a relatively high prevalence of NMSOD in this population, 10/100,000. Despite the higher prevalence of NMOSD in Black and Asian patients, there is limited knowledge of the effectiveness of disease modifying treatments across racially diverse groups. To assess the effectiveness of rituximab and oral immunosuppressive agents in a cohort of NMOSD patients, the majority of whom are Black, in a real-world, clinical setting. A single-center retrospective study was conducted at the University of Chicago Medical Center. (1) diagnosis according to the 2015 International Panel for NMO Diagnosis (IPND) Criteria, (2) positive anti-aquaporin-4 antibodies on ELISA or cell-based tests, (3) initiation of immunosuppressant therapy within 5 years of disease onset, (4) first-line treatment with rituximab, mycophenolate (MMF), or azathioprine (AZA). Patients with negative anti-AQP4 titers were excluded. Kaplan-Meier survival analysis was used to estimate proportion of relapse-free patients following initiation of first line immunosuppressive therapy. A Cox proportional hazards regression model assessed the association of risk of relapsing with first-line immunosuppressive treatments with and without adjustments of pre-specified factors (age at disease onset, duration of disease, sex, race, CNS location of relapse). 7 of 29 patients (24%) receiving rituximab experienced a relapse within the first 3 years of treatment vs. 13 of 23 patients (57%) receiving either AZA or MMF. Within the first 6 months of treatment, 2 (6.9%) patients treated with rituximab experienced a relapse vs. 7 (30.4%) patients treated with either MMF or AZA. In the 29 patients treated with rituximab, the 1-year and 3-year proportion of relapse-free patients was 88.8% and 70.9%. For the 23 patients treated with either AZA or MMF, the 1-year and 3-year proportion of relapse-free patients was 69.5% and 38.7%. In the univariate analysis, the risk of relapse was significantly higher in patients treated with AZA or MMF compared to those treated with rituximab (hazard ratio [HR] of 2.48 [0.99 - 6.21]; p=0.046). In this real-world study involving a majority of Black NMOSD patients, rituximab was relatively more effective in preventing relapses within 3 years of therapy initiation than AZA and MMF. Rituximab remains an effective option for treating NMOSD, especially when there are delays in treatment due to access and economic issues associated with newer treatments.

Correlation between Amerindian ancestry and neuromyelitis optica spectrum disorders (NMSOD) among patients in Midwestern Brazil.

Neuromyelitis optica spectrum disorder (NMOSD) is the second most frequently demyelinating, autoimmune, and inflammatory Central Nervous System (CNS) disease, and its prevalence varies greatly according to geography and ethnicity. To determine the prevalence and phenotype of NMOSD at a reference center for demyelinating diseases in Goiás State. This was a cross-sectional study, approved under CAAE number 8380.9317.9.0000.5078. All patients fulfilled the 2015 international consensus criteria. Our study showed NMOSD as 9.37% of all demyelinating diseases registered in. It occurred predominantly in women (81%) and non-white individuals (83.4% had self-declared mixed skin color), and the median age at onset was 48 years. Amerindian ancestry was significantly higher (68.75%) than others. Longitudinally extensive transverse myelitis (LETM) alone ≥3 vertebral segments (35%) and optic neuritis (ON) alone (35%) were the most common onset manifestations. The median length of time from disease beginning to study enrollment was 48 months. A relapsing course and moderate disability (Expanded Disability Status Scale (EDSS) 3.0-4.0) were most commonly observed. The worst neurological impairments, characterized by EDSS>4.5, occurred more frequently in males (44.5% among men versus 20.5% among women). The majority of the patients had been receiving immunosuppressive treatment with azathioprine since the diagnosis of NMSOD: 77% (37) had a good therapeutic response. The prevalent outcome (84%) was permanent disability: 52% became physically handicapped; 54% had permanent visual impairment (25% with bilateral and 75% with unilateral amaurosis) and 30% had sphincter disability (82% with neurogenic bladder and 18% with ostomy). The estimated prevalence of NMOSD in Goiás is 0.79/per 100,000 inhabitants. The predominant phenotype comprises women, non-whites, onset in the fourth decade of life, relapsing course, and permanent moderate disability. Our study was the first on the epidemiology of NMOSD in Goiás, where NMOSD predominantly correlates with Amerindian ancestry.

Prevalence of neuromyelitis optica spectrum disorder in antioquia between 2016 and 2018

BackgroundThere are few epidemiological studies published in the world evaluating the prevalence of Neuromyelitis Optica Spectrum Disorder (NMOSD). The true prevalence of the disease is not known and the studies carried out are based on the diagnostic criteria used prior to 2015. ObjectiveTo determine the prevalence of NMOSD in Antioquia, from January 2016 to December 2018. MethodsThe prevalence of NMOSD in Antioquia was determined using the Capture-Recapture Method. Eight centers in the Department of Antioquia for the care of patients with neurological diseases were included. The data was collected between 2016 and 2018. ResultsA total of 221 consultation histories, 169 patients with a diagnosis of NMOSD were identified. The prevalence was 4.03 cases/100,000 inhabitants (95% confidence interval (CI) 3.3–4.8) of whom (87.5%), were women and the predominant race was Mestizo (81.6%). The most frequent initial presentation was optic neuritis (ON) (50.9%). Most of the patients had motor or visual disability (86.4%) and the treatment most used was Rituximab (47.9%). ConclusionThe prevalence of NMOSD in Antioquia is one of the highest reported in the world, except for the French Antilles. More studies are required to know the prevalence of this disease in the Colombian population.

Incidence and prevalence of neuromyelitis optica spectrum disorders in Slovakia

ABSTRACT Objective : Neuromyelitis optica spectrum disorders (NMOSDs) are a group of rare, inflammatory, demyelinating diseases that affect the central nervous system. Neither the incidence nor the prevalence of NMOSD has been determined in Slovakia thus far. The aim of this study was to determine both the incidence and the prevalence of NMOSD in Slovakia using the 2015 International Panel of NMOSD diagnosis (IPND) criteria. Methods : We performed a population-based study in Slovakia to estimate both the incidence and the prevalence of NMOSD during the period from 1 January 2006 through 31 December 2019. NMOSD cases were reported from multiple sources and the diagnosis was subsequently verified using the IPND criteria by a joint commitee of three neurologists. The prevalence is reported as number of cases per 100,000 inhabitans and the incidence as number of new cases per 1,000,000 person-years. Age-adjusted rates to the WHO standard population 2005–2025 were also calculated. Results : We identified 63 NMOSD cases. The crude point-prevalence rate was 1.37 (95% CI 1.03–1.71) per 100,000 inhabitants. The crude indidence rate was 0.88 (95% CI 0.65–1.12) per 1,000,000 person-years. The age-adjusted point-prevalence rate was 1.42 (95% CI 1.07–1.84) per 100,000 persons and the age-adjusted incidence rate was 0.96 (95% CI 0.72–1.25) per 1,000,000 person-years. Conclusion : The NMOSD epidemiological situation in Slovakia is comparable to those reported from other Caucasian populations.

NMOSD and MS prevalence in the Indigenous populations of Australia and New Zealand.

We studied the prevalence of neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) in Indigenous populations of Australia and New Zealand with the aim of assessing potential differences. Cases of possible NMOSD and MS were collected from Australia and New Zealand. Clinical details, MR imaging, and serologic results were used to apply 2015 IPND diagnostic criteria for NMOSD and 2010 McDonald criteria for MS. Frequencies of self-determined ethnic ancestry were calculated for confirmed NMOSD, suspected NMOSD, and MS. Prevalence rates for NMOSD and MS according to ancestry were compared. There were 75 cases with NMOSD, 89 with suspected NMSOD, and 101 with MS. NMOSD cases were more likely to have Asian, Indigenous, or Other ancestry compared to suspected NMOSD or MS. There were no differences in the clinical phenotype of NMOSD seen in Indigenous compared to European ancestry populations. Per 100,000, the prevalence estimate for NMOSD in people with Māori ancestry was 1.50 (95% CI 0.52-2.49) which was similar to those with Asian ancestry 1.57 (95% CI 1.15-1.98). NMOSD prevalence in Australian Aboriginal and Torres Strait Islander populations was 0.38 (95% CI 0.00-0.80) per 100,000. The prevalence of NMOSD in the Māori population is similar to South East Asian countries, reflecting their historical origins. The prevalence of MS in this group is intermediate between those with South East Asian and European ancestry living in New Zealand. Both NMOSD and particularly MS appear to be uncommon in the Indigenous populations of Australia.

Central nervous system demyelinating diseases in Hong Kong

AbstractObjectiveTo give an overview and update on central nervous system inflammatory demyelinating diseases in Hong Kong.FindingsUsing retrospective analysis of records from the Multiple Sclerosis (MS) Specialist Panel, the prevalence of MS in Hong Kong was estimated to be 6.9 per 100,000, while the annual incidence were 0.86 (SD = 0.135, 95% CI = 0.76 to 0.96) and 0.16 (SD = 0.11, 95% CI = 0.08‐0.23) per 100,000 for adult and pediatric populations, respectively. Preliminary data from the Hong Kong MS Society's NMOSD (neuromyelitis optica spectrum disorders) Registry showed that the local prevalence of NMOSD was 3.3 per 100 000, with a seropositivity rate of 80%. MS and NMOSD both had a female preponderance, and MS had younger disease onset age. NMOSD patients had more long‐term disability in terms of EDSS (expanded disability status scale) but MS patients had more cognitive impairment. Radiological features that discriminated MS and NMOSD were similar to other populations, and brain atrophy was observed in both diseases. The number of available treatment options for MS in Hong Kong has greatly increased in the past decade, including injectables, oral disease‐modifying agents, and immune‐reconstitution therapies. For NMOSD, corticosteroids and oral immunosuppressants were the mainstay of treatment.SummaryThe updated prevalence of MS and NMOSD in Hong Kong was higher than previously reported, and comparable with other Asian countries. The clinical, laboratory and imaging features of MS and NMOSD in Hong Kong were similar to those in neighboring regions.

Клинико-эпидемиологическая характеристика заболеваний спектра оптиконевромиелита в Республике Татарстан

The purpose — to study the clinical and epidemiological characteristics of neuromyelitis optica spectrum disorders (NMOSD) in the Republic of Tatarstan (RT). Material and methods. A retrospective analysis of the patients with NMOSD (according to 2015 diagnostic criteria), observed in our Centre in 2017–2021 (n = 35), was carried out. The parameters analyzed were demographic features, age of the disease onset, first clinical manifestations and course of disease, anti-AQP4 serum status, disease modifying therapy, and EDSS on baseline and follow up visits. Results and conclusions. We found that the prevalence of NMOSD in RT in 2021 was 0.9/100,000, the averaged (for 2017–2020) incidence was 0.83/1,000,000 per year. Among 35 patients (average age 43,4 (12,7) years old), 24 (69%) were female and 11 (31%) were male. The most common clinical manifestations at onset included optical neuritis and/or myelitis (94%). Most cases (83%) had relapsing course. Positive anti-AQP4 serum status was revealed in 49% of patients. The probably monophasic disease (n = 6) was observed predominantly in anti-AQP4 negative patients (5 vs. 1). DMT was prescribed to all patients with relapsing NMOSD, the most commonly used agents included corticosteroids, azathioprine and mitoxantron (49, 20, and 11% accordingly). The rate of remission rate for all DMT varied from 35 to 57%. Ultimately all patients with relapsing NMOSD showed EDSS increase over the follow-up period, which was more prominent among anti-AQP4 positive patients (from 3,8 to 5,4 over 4,4 (3,3) years, compared to 2,9 to 3,8 over 4,0 (3,0) years accordingly).

Clinical and epidemiological aspects of neuromyelitis optic spectrum diseases in the russian population

To present clinical and epidemiological aspects of neuromyelitis optica spectrum disorders (NMOSD) in the Russian Federation. We studied 142 patients who met diagnostic criteria of 2015 for NMOSD. Sex, age at disease onset, presence or absence of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), mail clinical symptoms, oligoclonal IgG, therapy for the treatment of exacerbations and prevention of exacerbations, compliance with 2006 diagnostic criteria were assessed. The prevalence of women is 4.26:1, the most frequent age at disease onset is 18-29 years (36% of cases). The laboratory aspects of the disease are characterized and approaches to the treatment and prevention of exacerbations of NMOSD in patients of the Russian population are evaluated. Approaches to diagnostics are compared depending on the applied diagnostic criteria (34% of patients do not meet neuromyelitis optica 2006 diagnostic criteria). A prognosis for the prevalence of NMOSD in the Russian population has been proposed: 0.45-4.21/100000. This is the first published data on clinical and epidemiological characteristics of NMOSD in the Russian Federation.

Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide.

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon inflammatory disease of the central nervous system, manifesting clinically as optic neuritis, myelitis, and certain brain and brainstem syndromes. Cases clinically diagnosed as NMOSD may include aquaporin 4 (AQP4)-antibody-seropositive autoimmune astrocytopathic disease, myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive inflammatory demyelinating disease, and double-seronegative disease. AQP4-antibody disease has a high female-to-male ratio (up to 9:1), and its mean age at onset of ~40 years is later than that seen in multiple sclerosis. For MOG-antibody disease, its gender ratio is closer to 1:1, and it is more common in children than in adults. Its clinical phenotypes differ but overlap with those of AQP4-antibody disease and include acute disseminated encephalomyelitis, brainstem and cerebral cortical encephalitis, as well as optic neuritis and myelitis. Double-seronegative disease requires further research and clarification. Population-based studies over the past two decades report the prevalence and incidence of NMOSD in different populations worldwide. One relevant finding is the varying prevalence observed in different racial groups. Consistently, the prevalence of NMOSD among Whites is ~1/100,000 population, with an annual incidence of <1/million population. Among East Asians, the prevalence is higher, at ~3.5/100,000 population, while the prevalence in Blacks may be up to 10/100,000 population. For MOG-antibody disease, hospital-based studies largely do not observe any significant racial preponderance so far. This disorder comprises a significant proportion of NMOSD cases that are AQP4-antibody-seronegative. A recent Dutch nationwide study reported the annual incidence of MOG-antibody disease as 1.6/million population (adult: 1.3/million, children: 3.1/million). Clinical and radiological differences between AQP4-antibody and MOG-antibody associated diseases have led to interest in the revisions of NMOSD definition and expanded stratification based on detection of a specific autoantibody biomarker. More population-based studies in different geographical regions and racial groups will be useful to further inform the prevalence and incidence of NMOSD and their antibody-specific subgroups. Accessibility to AQP4-antibody and MOG-antibody testing, which is limited in many centers, is a challenge to overcome. Environmental and genetic studies will be useful accompaniments to identify other potential pathogenetic factors and specific biomarkers in NMOSD.

Prevalence and Incidence of Neuromyelitis Optica Spectrum Disorder in Korea: Population Based Study.

BackgroundAlthough neuromyelitis optica spectrum disorder (NMOSD) is known to be a rare disease, its prevalence and incidence have not yet been studied in Korea. We performed a population-based study to examine the prevalence and incidence of NMOSD in Korea using data from the Korean National Health Insurance (NHI) claims database.MethodsData from 2013 to 2017 were obtained, with a washout period set as 2013 and 2014. The prevalence and incidence of NMOSD in 2016 and 2017 were calculated using population census data. Subjects were divided into 5 groups at 15-year intervals, depending on the age at which the diagnostic code was entered. The relative risk (RR) for each age group was compared with the oldest (≥ 60 years) age group.ResultsThe overall prevalence was estimated to be 3.36 and 3.56 per 100,000 individuals, with an incidence of 0.41 and 0.65 per 100,000 individuals-year in 2016 and 2017, respectively. The mean age was 43.08 (standard deviation, 14.56) years, and the ratio of male to females was 1:4.7. The incidence was higher in female individuals aged between 30 and 59 years (RR, 2.8–3.05; P < 0.05).ConclusionNationwide prevalence of NMOSD in Korea was 3.36 and 3.56/100,000 and its incidence was 0.41 and 0.65/100,000-year in 2016 and 2017 respectively.

Letter to the Editor: Lack of Attention to Epidemiological Study of Neuromyelitis Optica Spectrum Disorder Compared with that of Multiple Sclerosis in Iran.

Neuromyelitis optica spectrum disorder (NMOSD) was once considered a type of multiple sclerosis (MS), but, due to differences in pathogenesis of these two diseases, NMOSD is currently considered as a totally separate entity and has different diagnostic criteria from MS.1 Whereas MS is a demyelinating disease of the central nervous system, NMOSD is an astrocytopathic disorder. The incidence of NMOSD is much lower than that of MS, and, according to various reports, it varies from 0.51 per 100,000 people to 4.4 per 100,000 people.2 Nevertheless, there are many clinical similarities between NMOSD and MS. Detection of NMOSD is crucial, because if it is not properly diagnosed it can threaten a patient's life. This distinction of NMOSD from MS is very important and emphasizes the 2017 McDonald criteria for MS diagnosis.1 It has been suggested that in areas with a high NMOSD rate, each patient with MS should also be checked for NMOSD.3 Therefore, recognizing areas with a high prevalence of NMOSD is key.Unfortunately, researchers have widely ignored epidemiological study of NMOSD in different regions of Iran. After performing a comprehensive survey on various sites including PubMed, Scopus, and Web of Science (ISI), only five epidemiological studies of the disease in Iran were found.24–7 One was carried out in Khuzestan5 and two others in Isfahan.67 Two articles have evaluated NMOSD in Iran and in Iranian patients24; however, they cannot show the overall epidemiology of the disease there and its distribution in different parts of the country. In the article by Sahraian and colleagues,4 only the clinical and demographic characteristics of 44 patients were studied; place of birth and place of residence of these patients were not discussed. The article by Eskandarieh and colleagues,2 which is the most comprehensive study of NMOSD in Iran, is merely an examination of the epidemic aspects of the disease in Tehran, contrary to the title. Moreover, information about other regions of Iran was not found (Figure 1).Therefore, taking the aforementioned studies into account, information on NMOSD is available only regarding three provinces, which cannot guide physicians given the prevalence of the disease in other regions of Iran. Meanwhile, according to a recent systematic study of the prevalence of MS in Iran,8 epidemiological studies of MS have been carried out in 31 provinces, which is truly significant. There is a need for more epidemiological studies of NMOSD in different regions of Iran, as such studies could help improve patient care.The author declares no conflicts of interest.

Prevalence and clinical features of neuromyelitis optica spectrum disorders in northern Japan

To clarify the prevalence and clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) in Japan and compare them with those in other ethnic populations. Data processing sheets were sent to all related institutions in northern Japan and were collected from April to May 2016. Prevalence was determined on March 31, 2016, using the 2015 International Panel for NMO Diagnosis criteria. The crude prevalence was 4.1/100,000 (95% confidence interval 2.2-6.9) for NMOSD in northern Japan, with a significantly higher number of female than male patients (female: male 12:2). The positivity for anti-aquaporin-4 antibody was 78.6%, and the mean age at onset was 45.2 years. All patients were subjected to preventive therapy in the form of treatment with steroids or immunosuppressive agents. Our results showed that the prevalence of NMOSD in the Japanese population is similar to that in Caucasians.

Epidemiology of NMOSD in Catalonia: Influence of the new 2015 criteria in incidence and prevalence estimates

Background: Population-based studies on neuromyelitis optica spectrum disorders (NMOSD) are limited, and it is unclear whether the rates have changed with the implementation of the new 2015 criteria. Objectives: To estimate the incidence and prevalence of NMOSD in Catalonia (Spain), using both the 2006 and the 2015 criteria. Methods: In this clinic-based retrospective study, patients diagnosed with NMOSD between 2006 and 2015 were identified using multiple sources, including direct contact to all Catalan hospitals, identification of cases through the Catalan Health Surveillance System, and registry of antibodies to aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) in a reference laboratory. The incidence rate was calculated for the period 1 January 2006–1 January 2016 and prevalence for the date 1 January 2016. Results: We identified 74 patients (by the 2015 criteria). Most patients were Caucasian (81%), and female (76%) with a median age at disease onset of 42 years (range, 10–76 years). In total, 54 (73%) patients were positive for AQP4-IgG, 11 (15%) double-seronegative, and 9 (12%) MOG-IgG-positive. Rates of incidence and prevalence (0.63/1,000,000 person-years and 0.89/100,000, respectively) were 1.5-fold higher than those reported by the 2006 criteria. Lowest rates were seen in children and elder people and highest in women and middle-aged people (40–59 years). The female predominance was lost in incident AQP4-IgG-seronegative children and AQP4-IgG-positive elder people. MOG-IgG and double-seronegativity contributed similarly but did not influence the long-term outcome. Conclusion: The new criteria increase the estimates, but NMOSD remains as a rare disease. The differences in age- and sex-specific estimates highlight the importance of the serologic classification.

Incidence and prevalence of NMOSD in Australia and New Zealand

ObjectivesWe have undertaken a clinic-based survey of neuromyelitis optica spectrum disorders (NMOSDs) in Australia and New Zealand to establish incidence and prevalence across the region and in populations of differing...

Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution

Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd is of unknown prevalence worldwide, we conducted a retrospective, cross-sectional study of 850 patients with demyelinating disorders hospitalized in North East Tuscany from 1998 to 2006 to examine the prevalence of NMO and related disorders among unselected consecutive neurological patients with inflammatory CNS diseases and to evaluate the clinical phenotype spectrum of identified cases. Clinical data were updated after at least 2 years of follow-up. An immunofluorescence technique was used to detect NMO-IgG on rat brain tissue. Sera from other 828 neurological patients, 65 non-neurological patients and 50 healthy donors served as controls. The prevalence of NMOsd was 1.5%, with a MS:NMOsd ratio of 42.7. Among 13 NMOsd patients, 77% had long spinal cord lesions, 38% had severe optic neuritis and 23% had brain or brainstem lesions. Only 56% had clinically definite NMO at follow-up. The final EDSS score ranged from 1 to 10, mainly depending on brainstem involvement occurrence. Our findings confirm a low prevalence of NMO and related disorders among demyelinating inflammatory diseases in a Caucasian population. Moreover, this study demonstrates an unexpectedly high prevalence of limited and atypical variants of this disease, not previously documented.