Materiały źródłowe

• #1 Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7332882/

  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. […] 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. […] 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.

• #2 Neuromyelitis Optica – EyeWiki

  https://eyewiki.org/Neuromyelitis_Optica

  Neuromyelitis optica (NMO) is the second most common type of demyelinating disease. It has a higher prevalence in women, particularly those of childbearing age (15 to 40 years), with a female-to-male ratio of (9:1). […] Moreover, studies show around 20%47% of women develop their first symptoms of NMO during pregnancy or within a year of childbirth or miscarriage. […] NMOSD may coexist with Systemic Lupus Erythematosus, Sjgren Syndrome, or myasthenia gravis and presence of these diagnoses tends to strengthen the confidence in NMOSD diagnosis.

• #3 Neuromyelitis Optica Spectrum Disorders: Clinical Perspectives, Molecular Mechanisms, and Treatments

  https://www.mdpi.com/2076-3417/13/8/5029

  Neuromyelitis optica (NMO) is a rare autoimmune inflammatory disorder affecting the central nervous system (CNS), specifically the optic nerve and the spinal cord, with severe clinical manifestations, including optic neuritis (ON) and transverse myelitis. […] NMO was expanded to NMO spectrum disorder (NMOSD) because of its varied clinical phenotypes. NMOSD is characterized by the activation of the complement cascade, granulocyte, eosinophil and lymphocyte infiltration, oligodendrocyte, and astrocyte injury, demyelination, and neuronal loss. […] Most NMOSD patients exhibit acute inflammatory responses activated by autoantibodies of IgG against AQP4 in the optic nerve and spinal cord, CNS attack relapses, ON, and encephalitis of the diencephalon, area postrema, and brain stem. […] Classically, about 30% of NMOSD patients have been observed with severe neurological disability, ON, worse clinical outcomes, and the presence of oligoclonal bands in the CSF.

• #4 Worldwide prevalence of neuromyelitis optica spectrum disorder (NMOSD) and neuromyelitis optica (NMO): a systematic review and meta-analysis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36745300/

  Neuromyelitis optica spectrum disorder (NMOSD) is a progressive demyelinating disease of the central nervous system that has overlapping symptoms with multiple sclerosis (MS) but differs from it in a variety of ways. Previous studies have reported conflicting results trying to estimate the number of individuals affected by them which is why we designed this systematic review and meta-analysis to estimate the worldwide prevalence and incidence of NMOSD/NMO based on current evidence. […] The prevalence and incidence rates of NMOSD/NMO ranged from 0.07 to 10 and 0.029 to 0.880 per 100,000 population, respectively. The overall pooled prevalence of NMO per 100,000 population was 1.54 (I2: 98.4%, 95% CI: 1.13-1.96, P 0.001) based on the 2006 criteria, 1.51 (I2: 99.4%, 95% CI: 1.21-1.81, P 0.001) based on the 2015 criteria and 2.16 (I2: 89.4%, 95% CI: 1.46-2.86, P 0.001) based on the 2006/2015 criteria. The overall annual incidence of NMO per 100,000 population was 0.155 (I2: 95%, 95% CI: 0.115-0.195, P 0.001) based on the 2006 criteria and 0.278 (I2: 100%, 95% CI: 0.135-0.420, P 0.001) based on the 2015 criteria. The prevalence rates were highest in French West Indies and South Korea, and lowest in Cuba and Australia, based on the 2006 and 2015 criteria, respectively. Also, the highest annual incidence rates were obtained for Sweden and Slovak republic and the lowest for Cuba and Australia based on the 2006 and 2015 criteria, respectively. All estimated rates were higher among females compared to males. […] Several large-scale prospective studies are required to reach a comprehension of the epidemiological aspects of these notorious demyelinating conditions.

• #5 Neuromyelitis optica spectrum disorder – Wikipedia

  https://en.wikipedia.org/wiki/Neuromyelitis_optica_spectrum_disorder

  Prevalence varies by region, ranging from 0.5 to 10 cases per 100,000 people. […] NMO is more common in women than men, with women comprising over two-thirds of patients and more than 80% of those with the relapsing form of the disease. […] A retrospective study found that prevalence of neuromyelitis optica spectrum disorders was 1.5% among a random sample of neurological patients, with a MS:NMOSD ratio of 42:7. […] NMO is more common in Asians than Caucasians. […] The majority of NMO patients have no affected relatives, and it is generally regarded as a nonfamilial condition. […] Rarely, NMO may occur in the context of other autoimmune diseases (e.g. connective tissue disorders, paraneoplastic syndromes) or infectious diseases.

• #6

  https://journals.lww.com/neur/fulltext/2022/70050/incidence_and_prevalence_of_neuromyelitis_optica.3.aspx

  Greater awareness of the disease globally and use of sensitive diagnostic criteria have improved epidemiological data for NMOSDs. […] The importance of using sensitive diagnostic criteria was evident from the results of studies that surveyed the same population by using both 2006 and 2015 criteria for NMOSDs. Incidence and prevalence of AQP4-IgG seropositive patients increased by several folds after applying the 2015 criteria. This review suggests that the worldwide prevalence of NMOSDs has not exceeded 5/100,000 despite using uniform diagnostic criteria, multiple sources for case ascertainment, and standard methods of surveys, which is in direct contrast to the uneven distribution of MS.

• #7 Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7332882/

  A limitation regarding incidence calculation is that, if a new antibody test becomes available in the study region, or when there is increased awareness among clinicians, then the number of newly diagnosed cases in that particular year will be higher, leading to a higher incidence rate, even though the disease could have started many years earlier in some cases. […] Among Whites, the annual incidence of NMOSD is generally reported to be around 0.50-0.8/million.

• #8 Orphanet: Neuromyelitis optica spectrum disorder

  https://www.orpha.net/en/disease/detail/71211

  A rare inflammatory disease of the central nervous system characterized mainly by attacks of uni- or bilateral optic neuritis (ON) and acute myelitis. […] Neuromyelitis optica spectrum disorder (NMOSD) has a worldwide distribution and estimated prevalence of 1-5/100,000. The average annual incidence is 1/770,000 worldwide. Nearly 90% of affected individuals are female. […] Fewer than 5% of individuals report relatives with NMOSD; polygenic inheritance is thought to be responsible for the small excess of familial cases relative to expected.

• #9 Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7332882/

  The prevalence range of NMOSD is ~0.54/100,000, and may be up to 10/100,000 in certain racial groups. […] Over the past two decades, population-based studies of NMOSD have provided important insights into its prevalence. […] Inter-racial variation in prevalence, as summarized in Table 1, is notable and consistent across geographical regions. […] The study in Hokkaido, Japan recorded a prevalence of 4.1/100,000, while the Japanese nationwide survey estimated a prevalence of 3.42/100,000. […] More studies, especially from China, Taiwan, and Hong Kong will be useful to further inform the prevalence of NMOSD among the East Asians. […] Population-based studies over the past two decades showed that Blacks also have a higher NMOSD prevalence than Whites. […] In recent nationwide and region-wide studies, the prevalence of NMOSD among Whites has consistently been ~1/100,000.

• #10 Neuromyelitis optica spectrum disorder | MedLink Neurology

  https://www.medlink.com/articles/neuromyelitis-optica-spectrum-disorders

  Neuromyelitis optica spectrum disorder is a rare disease. In a systematic review from 2021, the worldwide incidence of neuromyelitis optica spectrum disorder in adults varies from 0.029 to 0.7 per 100,000 persons-years. The prevalence was estimated to be 0.34 to 10 per 100,000 persons 0.06 to 0.22 per 100,000 in children. The highest incidence and prevalence are found in persons of African heritage followed by Asians. […] A 2016 comparative study investigated the incidence and prevalence of neuromyelitis optica spectrum disorder and anti-AQP4 IgG seropositive status in patients with a prior diagnosis of inflammatory demyelinating disease. These patients were either residents of Olmsted County, Minnesota (82% Caucasian) or residents of Martinique (90% Afro-Caribbean). The authors found the prevalence in Martinique to be 10 per 100,000 but in Olmsted County only 3.9 per 100,000. The prevalence is estimated to be 3.42 per 100,000 in Japan, 3.56 per 100,000 in South Korea, and 3.31 per 100,000 among Chinese in Malaysia. In multiple recent nationwide studies, the prevalence of neuromyelitis optica spectrum disorder among Caucasian has been approximately 1 out of 100,000. […] Neuromyelitis optica spectrum disorder occurs in the pediatric population with an average age of onset of 7 to 14 years. Neuromyelitis optica spectrum disorder is more common in women, accounting for more than 80% of cases, although in children there is no difference in gender.

• #11 Epidemiology of neuromyelitis optica spectrum disorder and its prevalence and incidence worldwide | Max Delbrück Center

  https://www.mdc-berlin.de/research/publications/epidemiology-neuromyelitis-optica-spectrum-disorder-and-its-prevalence-and

  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. […] 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. […] 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.

• #12 NMOSD | Neuromyelitis Optica Spectrum Disorder | Epidemiology | SRNA

  https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/epidemiology/

  NMOSD can affect children as young as 2 years and adults as old as 90 years. […] While MS is more prevalent among Caucasians, NMOSD disproportionately affects those of African descent. […] It is more common in women. […] The onset of NMOSD varies from childhood to adulthood, and the average age of onset is about 40 (about 10 years later than that of MS). […] In a more recent study published by Mealy et al.,1 out of 187 patients from three academic centers in the United States, there were 14 patients with onset as a minor, with only 5-8 being pre-menses in their development. […] Children with NMOSD are more likely to be NMO-IgG seronegative.

• #13 Neuromyelitis optica spectrum disorder | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/neuromyelitis-optica-spectrum-disorder?case_id=neuromyelitis-optica

  Neuromyelitis optica spectrum disorder is typically found in patients somewhat older than those with multiple sclerosis, with an average age of presentation of 41 years, and there is an even stronger female predilection (F:M 6.5:1). It is found more frequently in patients of Asian, Indian, and African descent. […] Neuromyelitis optica spectrum disorder (NMOSD) is a severe demyelinating disease, which in seropositive cases, is caused by an autoantibody to the aquaporin-4 (AQP4) water channel.

• #14 What is Neuromyelitis Optica Spectrum Disorder (NMOSD)?

  https://www.nmosdwontstopme.com/what-is-nmosd

  Neuromyelitis optica spectrum disorder (NMOSD) is a rare and debilitating autoimmune disease caused by inflammation in the central nervous system (the optic nerve, brain stem and spinal cord). […] The median age of onset is 40 years, but NMOSD can occur at any age. […] Women may be nine times more likely to be impacted versus men. […] There is a two- to three-fold higher prevalence in Asian and African American populations. […] Within one year, 60% of those with NMOSD will have repeat attacks with no NMOSD treatment. […] After five years, four out of 10 of those with NMOSD may be legally blind in at least one eye, and two out of 10 may require a walker. […] Of those with optic neuritis (or vision challenges), 67% had partial or no recovery following an NMOSD attack. […] Epidemiology of neuromyelitis optica in the United States: A multicenter analysis. […] Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. […] Incidence and prevalence of NMOSD in Australia and New Zealand.

• #15 Neuromyelitis Optica (NMO): What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9858-neuromyelitis-optica-nmo

  Neuromyelitis optica (NMO) is a rare chronic condition that affects your eyesight and your ability to move. […] NMO is much more likely to affect females, who make up about 80% to 90% of cases. […] NMO overall is rare. The average number of people with NMO per 100,000 is between 0.3 and 4.4. That means there are between 1,000 and 14,600 cases in the United States and between 24,000 and 350,600 cases worldwide. […] Currently, there’s no evidence that NMOSD (NMO) can spread from person to person. […] NMO is manageable, and your healthcare provider will help guide you on how to take care of yourself. […] NMO causes attacks, which are flare-ups of symptoms. For people with AQP4 or MOG antibodies, NMO is a lifelong condition. […] In years past, NMO was a condition that had a very negative outlook. However, experts now know NMO is treatable, thanks to discoveries into the immune origins of this condition.

• #16 Neuromyelitis Optica Spectrum Disorders

  https://practicalneurology.com/articles/2019-feb/neuromyelitis-optica-spectrum-disorders

  Neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSD) are clinical syndromes traditionally defined by the presence of myelitis and optic neuritis (ON). The prevalence of NMOSD is roughly 4 to 10 per 100,000, with higher rates among those of African or Asian descent; onset occurs throughout the lifespan and 5 to 10 times more often in women than men. […] The International Panel for NMO Diagnosis (IPND) updated diagnostic criteria for NMOSD are shown in the Table. […] There are few population-based epidemiologic studies of MOG antibody-associated disease. In contrast to MS and NMOSD, the increased incidence in women vs men seems lower. […] The diagnostic criteria for NMOSD were updated in 2015 and account for both patients who are AQP4 seropositive and seronegative. Treatment recommendations have been largely based on retrospective studies and expert clinical experience with data supporting RTX, MMF, or AZA. Ongoing clinical trials may offer more evidence for using new therapies in the near future. It is critical that neurologists encountering patients with suspected demyelinating disorders consider NMOSD in the differential diagnosis, thereby avoiding delays in recognition and treatment.

• #17 Neuromyelitis Optica (NMO) Treatment | Rush

  https://www.rush.edu/services/neuromyelitis-optica-nmo-services

  Over 40% of people with neuromyelitis optica can become legally blind in at least one eye within five years of the condition first appearing. […] Of confirmed diagnosed cases in the United States, the condition has appeared in African Americans and Asians twice as much as Caucasians and nine times more in women than men. […] It usually affects people between ages 30 and 50. […] Treatment is most effective when it begins as early as possible. Early intervention can slow or stop progression of the disease and prevent disabilities. […] Getting a second opinion for an NMO diagnosis or prescribed treatment can help you make decisions about your care with confidence. […] The life expectancy of patients with NMO will vary a great deal from person to person. Without treatment, it often leads to worsening disability and can shorten life expectancy. But with effective treatment, life expectancy for a patient with NMO is the same as a person who does not have the condition, and progression of disability can often be slowed or stopped, leading to greater quality of life.

• #18

  https://journals.lww.com/neur/fulltext/2022/70030/a_study_of_neuromyelitis_optica_spectrum_disorders.46.aspx

  Neuromyelitis optica (NMO) is a central demyelinating disorder, predominantly affecting the optic nerves and spinal cord and autoimmune basis. We aimed to analyze the clinical, laboratory, and imaging features associated with NMO spectrum disorders (NMOSD) according to the aquaporin 4 antibody (AQP4-Ab) serology status. […] AQP4-Ab positivity was 56.5%. The male to female ratio in the seropositive group was 1:7.7 and 1:1.2 in the seronegative group. The mean age of onset in seropositive patients was 36.8 years (vs 28.8 years in seronegative NMOSD patients). […] We found differences in age, gender, and prognosis between the two groups. Antibody status may be a guiding factor in deciding the treatment approach during the first attack of NMOSD. […] Neuromyelitis optica Spectrum disorder (NMOSD) is a common demyelinating disorder of CNS. The clinical features, CSF findings, radiological parameters and prognosis may differ, according to antibody status; reflecting the possibility of different etio-pathogenic mechanism. Antibody detection may guide us in the treatment approach of NMOSD, even during the first attack.

• #19

  https://link.springer.com/article/10.1007/s00415-023-11634-0

  While NMOSD must be considered in both sexes, there is a strong female preponderance irrespective of ethnicity, with a female:male ratio of up to 10:1 among AQP4-IgG-seropositive patients, who account for the vast majority of NMOSD, and up to 3:1 in seronegative patients. […] Although a diagnosis of NMOSD must be taken into account irrespective of ethnic origin, the overall incidence and prevalence of NMOSD is thought to be higher among non-whites than among whites, with the highest rates found for patients of African descent in some studies. […] Reported incidence and prevalence estimates for NMOSD vary substantially among studies from different regions of the world (0.280.73/100,000 person-years and 0.5210/100,000 persons, respectively), suggesting differences in genetic background and environmental factors across the populations analyzed.

• #20 Incidence of neuromyelitis optica spectrum disorders in China: a large cohort study using claim data | BMJ Open

  https://bmjopen.bmj.com/content/12/1/e048942

  The incidence rate in females was significantly higher than that in males (females: 0.68, 95% CI: 0.63 to 0.72; males: 0.15, 95% CI: 0.13 to 0.17, per 100 000 person-years; p0.001), with a female-to-male IRR of 4.52 (95% CI: 4.39 to 4.65). […] The incidence increased with age, peaking at 5564 years in females and 6574 years in males and then decreasing thereafter. […] No significant correlation was detected between the incidence rates in cities in 20 provinces and latitude (R=0.03, p=0.896). The incidence rates in cities located 20, 25, 30, 35 and 40 north of Earths equatorial plane were 0.53 (95% CI: 0.46 to 0.61), 0.41 (95% CI: 0.33 to 0.50), 0.40 (95% CI: 0.36 to 0.43), 0.54 (95% CI: 0.46 to 0.62) and 0.30 (95% CI: 0.26 to 0.35) per 100 000 person-years, respectively. […] The results provide information on the epidemiological profiles of NMOSD in Asia and help to provide insights into NMOSD susceptibility to better understand the condition.

• #20 Incidence of neuromyelitis optica spectrum disorders in China: a large cohort study using claim data | BMJ Open

  https://bmjopen.bmj.com/content/12/1/e048942

  Objectives Population-based studies estimating the incidence of neuromyelitis optica spectrum disorders (NMOSDs) in Asia are limited, and the relationship between latitude and incidence has been scarcely investigated. We aimed to estimate the incidence of NMOSDs in Chinese adults and explore their relationship to latitude. […] The incidence of NMOSD in Chinese adults was 0.41 per 100 000 person-years. There is no latitude gradient observed. Sex and age influence the risk of NMOSD, suggesting the role of genetic, hormonal and other related factors in the pathophysiology. […] Based on a large cohort including 177 million people followed for 2 years, we identified 1313 newly diagnosed NMOSD cases, and the incidence of NMOSD in Chinese adults was estimated as 0.41 per 100 000 person-years.

• #21

• #22

  https://link.springer.com/article/10.1007/s00415-023-11634-0

  The proportion of patients with NMOSD among patients with inflammatory demyelinating disorders (IDD) varies among populations. While NMOSD accounts only for a small proportion of white adult patients with IDD, most of whom have classic MS, rates are much higher in some (but not all) Asian populations, at least in part reflecting the well-known lower prevalence of MS in Asia. […] Certain human leukocyte antigen (HLA) types are associated with an increased risk of developing AQP4-IgG-positive NMOSD (e.g., HLA-DRB1*03 in Europe, Brazil, and India; HLA-DPB1*0501 in southern Han Chinese; HLA-DPB1*0501 and HLA-DRB1*1602 in Japanese; and HLA- DRB1*04:04 and HLA- DRB1*10:01 in Muslim Arab Israelis).

• #23 A Meta-Analysis of Neuromyelitis Optica Epidemiology in Latin American Nations

  https://stars.library.ucf.edu/honorstheses/583/

  Neuromyelitis Optica (NMO) is a rare, autoimmune, neurodegenerative disease selectively affecting the optic nerves and spinal cord. Relapsing NMO is nine times more prevalent in women than in men and approximately one-quarter of NMO patients have symptoms of another autoimmune disorder (National Institute of Health, 2019). NMO affects roughly one percent of that of Multiple Sclerosis (4000-8000 patients total) in the United States, but prevalence rates are abnormally high in a handful of regions around the world, particularly among Latin America, where rates can reach up to 5/100,000 individuals. The results of this study predict that there is a connection between African genetics and NMO, but further studies will need to be conducted in more Latin America nations and other regions to determine prevalence rates as well as genetic analysis of affected individuals.

• #24

  https://journals.lww.com/neur/fulltext/2022/70050/incidence_and_prevalence_of_neuromyelitis_optica.3.aspx

  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. Incidence of NMOSDs was 0.040.25/100,000 in predominantly white and 0.341.31/100,000 in nonwhite populations. Prevalence was 0.701.91/100,000 in white and 0.864.52/100,000 in nonwhite populations. The 2015 IPND criteria significantly improved the incidence and prevalence rates for NMOSDs when compared to the Wingerchuk 2006 criteria. The prevalence of NMOSDs was estimated to be 5/100,000 globally. This review highlights the need for prospective population-based epidemiological studies and the importance of surveys in nonwhite populations around the globe.

• #25 How Common Is Neuromyelitis Optica?logo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na41013/2016/04/20/how-common-neuromyelitis-optica

  A population-based study suggests that the prevalence is less than 2% of all “demyelinating” disease in the U.S. […] Neuromyelitis optica (NMO) and NMO-spectrum disorder (NMOSD) are uncommon in the U.S., although epidemiologic studies are lacking. […] The calculated prevalences of NMO/NMOSD were 10 in 100,000 people in Martinique and 3.9 in 100,000 people in Olmsted County. […] The author estimated the prevalence of NMO/NMOSD in the U.S. to be 16,600 in 2011. […] These findings show that NMO is a rare disease, likely with fewer than 20,000 cases in the U.S. […] Of all demyelinating diseases, NMO/NMOSD constitutes <2%.

• #26 Neuromyelitis Optica Spectrum Disorder: From Basic Research to Clinical Perspectives

  https://www.mdpi.com/1422-0067/23/14/7908

  Neuromyelitis optica spectrum disorder (NMOSD) is a common cause of optic neuritis (ON) in Taiwan. In 2015, the prevalence of NMOSD was 1.47/100,000, and the age-standardized annual incidence rate was 0.61/100,000 person-years. The reported prevalence of NMOSD in different racial groups is approximately 1/100,000 in White individuals, 3.5/100,000 in Asians, and 10/100,000 in Black individuals. Currently, the clinical diagnosis of NMOSD is mainly based on the detection of serum NMO-IgG (AQP4-IgG) antibodies and the presence of core symptoms included in the diagnostic criteria developed by the International Panel for NMO Diagnosis in 2015. The revised criteria that replaced the previous 2006 criteria for NMO diagnosis resulted in a significant increase in the diagnostic sensitivity of NMOSD by 76% (62% in the AQP4-IgG-positive group and 14% in the seronegative group). The rate of seropositivity for myelin oligodendrocyte glycoprotein (MOG-IgG) antibodies in AQP4-IgG-seronegative patients with NMOSD was reported to reach up to 41.6%.

• #27

  https://journals.lww.com/neur/fulltext/2022/70030/a_study_of_neuromyelitis_optica_spectrum_disorders.46.aspx

  NMO occurs worldwide and have been described from different geographical regions. […] To the best of our knowledge, so far, this is the only long-term, comparative study of NMOSD patients from India. […] In this single-center observational study, 46 patients met NMO and NMOSD criteria, according to the 2006 and 2014 definitions. Out of the 46 patients, 56.5% were tested positive for AQP4-Ab. […] We reanalyzed the data according to the antibody status. There were differences in clinical features, imaging findings, and recovery patterns between seropositive and seronegative groups. […] The relapse rate among AQP4 positive NMOSD was 84.6% as compared with 55% in the seronegative group. […] The recovery rate in the seropositive cases was significantly lesser (15%) than seronegative cases (40%).

• #28

  https://journals.lww.com/neur/fulltext/2022/70030/a_study_of_neuromyelitis_optica_spectrum_disorders.46.aspx

  The differences between the two subgroups of NMOSD may point to accept the theory of different etiopathogenic mechanism. Frequent relapse and poor functional recovery, accumulating in more severe disability in short course, in AQP4-Ab positive NMOSD patients argue against demyelination. Antibody status may be the decisive factor in guiding the treatment approach in NMOSD, even during the first attack of NMOSD.

• #29 Assessment of disability and disease burden in neuromyelitis optica spectrum disorders in the CIRCLES Cohort | Scientific Reports

  https://www.nature.com/articles/s41598-024-75013-z

  Neuromyelitis optica spectrum disorders (NMOSD) comprise autoimmune diseases imposing substantial disability. […] The incidence of NMOSD is relative to geographical location, race, and ethnicity, where those of African and Asian descent are at a higher risk than other populations. Incidence estimates range from 0.05 to 10 per 100,000 people, have distinct geographical distributions, and indicate that females are disproportionately affected, at a ratio of up to 9:1. […] The present study was designed to quantitatively compare disability assessment instruments used to evaluate patients with NMOSD. […] The current study provides quantitative evidence supporting the need for an NMOSD-specific disability assessment instrument.

• #30 Research Advances in Neuromyelitis Optica Spectrum Disorder

  https://www.imrpress.com/journal/JIN/special_issues/neuromyelltis_optica_spectrum_disorder

  NMOSD is a major inflammatory demyelinating disorder of the CNS with a complex autoimmune etiology that has yet to be fully understood. […] This disorder has multifocal clinical manifestations and despite its reportedly low frequency, has become a topic of great international interest. […] Major challenges remain in the diagnosis and management of NMOSD, and its prevalence in non-white Caucasian populations is the opposite to that of Multiple Sclerosis (MS). […] Your contribution to this special issue will enhance and disseminate knowledge of different aspects of NMOSD, including basic research, epidemiology, clinical aspects, diagnosis including neuroimaging, and treatment. […] Keywords: Neuromyelltis Optica Spectrum Disorder, AQP-4 IgG antibody, NMO epidemiology, neuroimagen, NMO therapy.

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