Neuromyelitis Optica Spectrum Research Articles

NMO spectrum disorders: A single center experience

NMO spectrum disorders: A single center experience

B-101 Aquaporin 4 and Neuromyelitis Optica Spectrum Disorders

Abstract Background Neuromyelitis óptica (NMO) is a rare demyelinating inflammatory disease of the central nervous system, characterized by acute optic neuritis (ON) and transverse myelitis (TM). Previously, it was considered as a variant of multiple sclerosis (MS), however, there are now several clinical, laboratory, neuroimaging and pathological anatomical features that distinguish it from MS as the presence of a specific autoantibody in blood called IgG-NMO or anti-AQP4 that binds to water channels named aquaporin-4 (AQP4) expressed on astrocytes and plays a key role in the pathogenesis of this disease. Some patients remain seronegative for AQP4-IgG despite a definite diagnosis of NMO and the use of the finest methods for antibody detection. In anti-AQP4 seronegative forms, antibodies against myelin oligodendrocyte glycoprotein (MOG) may be identified. We have carried out a review of those patients with anti-AQP4-IgG detected in the autoimmunity laboratory during the last 3 years. Methods Each year we receive an average of 260 samples from patients with suspected demyelinating pathology with or without visual impairment. For the detection of antibodies against AQP-4 specifically HEK293 cells transfected were used as the standard substrate. Anti-MOG antibody can be detected in the same test. Measurement range: The dilution starting point for this measurement system is 1:10 (serum/plasma) and 1:1 (CSF, undiluted). Results A total of 5 patients were positive for anti AQP-4 antibody in serum samples, all women aged between 23 and 78 years. All patients except the youngest, who presented to the neurology department for loss of visual acuity, had other autoimmune diseases: systemic lupus erythematosus (SLE) in conjunction with rheumatoid arthritis, SLE in conjunction with Graves’ disease and hypothyroidism, the two oldest (59 and 78 years), respectively. The oldest patient was admitted to the internal medicine department for persistent nausea, vomiting and hiccups and was diagnosed as NMO spectrum disorder. In all cases, the oligoclonal immunoglobulin G bands study in CSF was negative. None of the CSF samples tested were positive for anti AQP-4 antibody. All patients were treated with methylprednisolone, but treatment with azathioprine or rituximab was necessary in 3 patients. Conclusion Titers of antibodies against AQP-4 are significantly higher in serum than in CSF, with serum being the most sensitive simple. NMO is more prevalent in women than men, with a female predominance usually higher than observed in MS. The median age of onset is also higher than MS, with a median of 35–45 years. Since the discovery of AQ-4 antibodies, there has been an increase in the number of clinical and radiological manifestations of NMO beyond involvement of the optic nerve and the spinal cord, including manifestations in the brain. It is important to recognise them in order to make an early diagnosis, to avoid unnecessary complementary tests and to establish the most suitable treatment. Persistent and intractable hiccups and nausea may occur in 17%–43% as in our oldest patient’s case. Associations with organ-specific autoimmune diseases such as autoimmune thyroiditis, and non-organ-specific diseases such as SLE and Sjögren’s syndrome have been described.

Devic’s Disease: The Confusing CNS Disease

Neuromyelitis optica spectrum is order (NMOSD) is a rare auto-antibody mediated disease of the central nervous system involving the central nervous system. The symptoms should alert the clinician to the diagnosis. Prompt diagnosis in the emergency department plays a crucial role as delay in diagnosis and initiation of management will lead to increased chances of morbidity and mortality. Approximately 80% of NMO patients have auto-antibodies against aquaporin-4, a water channel expressed on astrocytes. In this case report, we discuss a patient with NMO disease, the diagnosis and its management.

The Coexisting Neuromyelitis Optica Spectrum Disorder and Systemic Lupus Erythematosus: A Therapeutic Challenge.

Neuromyelitis Optica (NMO), or Devic's disease, is an immune-mediated, usually relapsing, central nervous system (CNS) demyelination disorder associated with optic neuritis and transverse myelitis. It is characterised by the presence of longitudinally extensive transverse myelitis (LETM) and antibodies against water channel aquaporin-4 (AQP4-immunoglobulin G [IgG]). The term NMO spectrum disorder (NMOSD) includes patients with limited forms of NMO who are at risk of recurrence. Often patients with NMO or NMOSD have an associated systemic autoimmune disease, most commonly systemic lupus erythematosus (SLE) or Sjogren syndrome (SS) or a related profile of non-organ-specific autoantibodies. The intriguing aspect of coexisting NMOSD and SLE is whether they are independent diseases that can coexist with each other or the serological findings specific to both diseases in a patient is a non-specific finding of no prognostic or therapeutic concern. We have presented two cases of NMOSD coexisting with SLE and based upon the existing evidence in the literature we present that the two conditions are independent of each other, and, at times, it can throw a therapeutic challenge to any clinician.

Radiological identification of neuromyelitis optica in a patient presented with unexplained weight loss and generalized weakness: a case report and literature review.

A 29-year-old female presented to the hospital with the complaint of blurry vision in her right eye and left eye ptosis for 2 days. Two months ago, the patient had a history of generalized fatigue with continuous documented fever with an average of 38.5°C, which was relieved by acetaminophen and ibuprofen. She also complained of continuous hiccups that increased at night and interfered with her sleep pattern and breathing; they lasted for 3 weeks and disappeared suddenly. She had also developed episodes of vomiting and could not tolerate food intake due to which she lost 6kg within 3 weeks. She was later diagnosed with neuromyelitis optica (NMO) using radiological neuroimaging. Early and correct diagnosis, followed by urgent treatment for acute exacerbations and the prevention of further relapses, are essential for treating NMO spectrum illnesses since they entail significant morbidity and, occasionally, fatality. The patient mentioned here represents a typical example of NMO disease. This case emphasizes the presence of this disease in our environment and the importance of accurately diagnosing this ailment, even in a context with minimal resources, to prevent disability.

Complement Turning Violent in Ultra-Rare Genetic Disorder

Abstract Sandhoff disease (SD) is an ultra-rare lysosomal storage disorder (LSD), which affects ~ 1/1000, 000 live birth. SD is caused by genetic deficiency of beta (β) Hexosaminidase and resulting excess central nervous system (CNS) synthesis of GM2 ganglioside (GM2) and its impact on neuron death. The exact mechanisms underlying such GM2-driven neuron death are unknown in SD. Glucosylceramide (GC) induced complement 5a (C5a) and its C5aR1 activation causes tissue inflammation in experimental and clinical Gaucher disease. Additionally, C5a-C5aR1 axis was found to sparks CNS inflammation and neurodegeneration in several brain diseases including, intracerebral hemorrhage, traumatic brain injury, myasthenia gravis, amyotrophic lateral sclerosis, neuromyelitis optica spectrum, Alzheimer, and Huntington’s diseases. Here, we determined the increased brain levels of C5a and C5aR1 in a beta (β) hexosaminidase inhibitor (HABI)-induced experimental mouse model of SD, when compared to vehicle (PBS) injected WT control mice. Also, HABI-induced experimental mouse model of SD showed increased microglial cells activation, massive brain generation of pro-inflammatory cytokines and loss of neurons, when compared to vehicle injected WT mice. To assess the relevance of C5a-C5aR1 axis activation for brain inflammation in SD, we targeted β hexosaminidase with HAB-I in WT and C5aR1 −/−mice. Strikingly, as compared to HABI-injected WT mice, HABI-injected C5aR1 −/−mice caused marked reduction in microglial cell activation, brain production of pro-inflammatory cytokines, and the loss of neurons. These data suggest that the C5a-C5aR1 axis is a critical driver of neuroinflammation and neurodegeneration in SD. No Funding

Neuromielitis óptica y enfermedad anti-MOG

The classic term neuromyelitis optica (NMO) has been replaced by NMO spectrum disorder (NMOSD) as a result of the identification of the diagnostic marker of the disease (IgG-AQP4) and the inclusion of features such as area postrema syndrome or diencephalic disturbances, among others. The article includes the diagnostic criteria for IgG-AQP4 positive and negative NMOSD and describes the treatments used in the acute phase and to prevent relapses (namely, oral steroid-sparing drugs, rituximab, tocilizumab, and drugs specifically developed for the disease). Some of the patients with seronegative IgG-AQP4 NMOSD will show positivity for the IgG-MOG (myelin oligodendrocyte glycoprotein) marker, which can also manifest as recurrent optic neuritis, acute disseminated encephalomyelitis, or conus medullaris syndrome, among others. The possibility of recurrences in some cases of persistently seropositive anti-MOG associated disease prompts the chronic use of immunomodulators in a similar way to TENMO, with the particularity of the usefulness of immunoglobulins in children. Finally, the epidemiological, clinical, paraclinical and therapeutic differences between multiple sclerosis, TENMO and anti-MOG disease are described.

More Than Meets the Eye: A Closer Look At Demyelinating Diseases In Zambia (P4-8.015)

<h3>Objective:</h3> Describe demographic and clinical characteristics of Zambian adults with MS and neuromyelitis optica (NMO). <h3>Background:</h3> MS and other demyelinating diseases are reportedly rare in sub-Saharan Africa and remain understudied. <h3>Design/Methods:</h3> Adults diagnosed with either MS, NMO, NMO spectrum disorder (NMOSD), or clinically isolated syndrome (CIS) at the only neurology outpatient clinic in Zambia were eligible to participate from October 2019 to February 2022. An MS-trained nurse administered structured questionnaires regarding sociodemographic characteristics, and each participant also underwent a comprehensive neurological history and examination by a neurologist. Finally, plasma 25-hydroxyvitamin D levels were obtained. For analysis, the cohort was dichotimized into a MS/CIS disease group and NMO/NMOSD disease group. Descriptive statistics of the cohort are presented and compared between both groups. <h3>Results:</h3> Amongst the 34 participants, mean age was 36 + 9 years, 65% (n=22) were female, 90% were Black-African, 10% were of Southeast Asian decent, 50% had MS/CIS, and 50% had NMO/NMOSD. The average age was 34 + 11 years in the MS/CIS group and 37 + 7 years in the NMO/NMOSD group (p=0.28). Females constituted 65% (n=11) of both disease groups. Median time to diagnosis was 242 days (interquartile range IQR: 91–974) and did not differ significantly between the groups. The majority (82%) of the NMO/NMOSD group presented with bilateral optic neuritis. Among the MS/CIS group, median EDSS was 4 (IQR: 2.25–4.25), median Disease Steps were 2 (IQR: 1–2), and 59% (n=10) had an abnormal gait at enrollment. Median 25-hydroxyvitamin D level was 29 (IQR: 24–46) ng/mL in the overall cohort but did not differ by disease group or supplementation status. <h3>Conclusions:</h3> In this sub-Saharan African cohort of adults with demyelinating diseases, MS and NMO were equally prevalent. Delays in diagnosis resulted in high levels of disability in both groups. <b>Disclosure:</b> Dr. Mortel has nothing to disclose. Dr. Braun has received research support from Zambart. Dr. Chishimba has nothing to disclose. Dr. Chomba has nothing to disclose. Dr. Mutete has nothing to disclose. NALUCA MWENDAWELI has nothing to disclose. Dr. Namangala has nothing to disclose. Dr. Zimba has nothing to disclose. The institution of Dr. Saylor has received research support from National Institutes of Health. The institution of Dr. Saylor has received research support from National Multiple Sclerosis Society. The institution of Dr. Saylor has received research support from American Academy of Neurology. The institution of Dr. Saylor has received research support from United States Department of State. Dr. Saylor has a non-compensated relationship as a Member of multiple committees and task forces focused on improving access to MS medications to people across the world with Multiple Sclerosis International Federation that is relevant to AAN interests or activities. Dr. Saylor has a non-compensated relationship as a Member of the Neurology and COVID19 committee with World Health Organization that is relevant to AAN interests or activities. Dr. Saylor has a non-compensated relationship as a Member of the International Outreach Committee, Junior and Early Career Membership Committee, and Educational Innovation Commitees with American Neurological Association that is relevant to AAN interests or activities.

Disautonomía gastrointestinal como manifestación de mielitis transversa: Reporte de caso

Inflammatory transverse myelitis is a rare condition that affects one or more levels of the spinal cord. Its etiology includes multiple sclerosis, infectious causes, or disorders within the spectrum of neuromyelitis optica. It presents acutely with motor, sensory, and/or dysautonomic symptoms, such as those related to the gastrointestinal and urinary systems. Diagnosis is based on symptomatology, evolution, and is confirmed by lumbar puncture, magnetic resonance imaging, and complete blood analysis. We present a clinical case of a patient with transverse myelitis who presented with gastrointestinal symptoms, motor symptoms, and was diagnosed with magnetic resonance imaging.

B cell targeted therapies in inflammatory autoimmune disease of the central nervous system.

Cumulative evidence along several lines indicates that B cells play an important role in the pathological course of multiple sclerosis (MS), neuromyelitisoptica spectrum disorders (NMOSD) and related CNS diseases. This has prompted extensive research in exploring the utility of targeting B cells to contain disease activity in these disorders. In this review, we first recapitulate the development of B cells from their origin in the bone marrow to their migration to the periphery, including the expression of therapy-relevant surface immunoglobulin isotypes. Not only the ability of B cells to produce cytokines and immunoglobulins seems to be essential in driving neuroinflammation, but also their regulatory functions strongly impact pathobiology. We then critically assess studies of B cell depleting therapies, including CD20 and CD19 targeting monoclonal antibodies, as well as the new class of B cell modulating substances, Bruton´s tyrosinekinase (BTK) inhibitors, in MS, NMOSD and MOGAD.

Prediction of Retinal Loss Based on Lesion of the Optic Nerve in Acute Stage of Optic-Neuritis

Aim: To assess the predictive features of optic neuritis in retinal loss through optic nerve lesion identification. Study design: Prospectively longitudinal study Place and duration of study: Ghulam Muhammad Mahar Medical College Sukkur and Shaheed Mohtarma Benazir Bhutto Medical University Larkana from 1st January 2021 to 30th June 2022. Methodology: One hundred and ten patients suffering from acute optic neuritis ≤8 weeks were enrolled. Retinal optical-coherence tomography was performed in all patients. Fifty five patients in the whole cohort while clinically isolated syndrome (CIS) 25 patients and relapsing remitting multiple sclerosis (RRMS) as well as neuromyelitis-optica spectrum disorders (NMOSD) and isolated optic neuritis as 20, 3 and 7 respectively. Brain optic nerve magnetic resonance imaging which included three-dimensional double inversion recovery sequence was also conducted at acute phase (M0) and then post 12 months (M12). Results: The mean age of the cases was 30.85±8.9 years. There was a higher frequency of males then females. The duration of the disease was found highest in RRMS cases with 50.31±77.45 months. The variance in estimated and standard error of the ganglion cell-inner plexiform layer volume change was significantly different in whole acute optic neuritis cases as well as the one with CIS and multiple sclerosisat cohort. Conclusion: Optic nerve-lesion length in prediction of retinal-neuroaxonal loss in cases of optic neuritis. The optic-nerve lesion length can also be considered as a biomarker for the process of retinal remodeling as well as visual impairment. Key words: Optic nerve lesion, Acute phase, Optic neuritis (ON), Retinal neuronal loss

NEUROMYELITIS OPTICA SPECTRUM DISORDER (NMOSD) DENGAN ANTIBODI AQP4 POSITIF

Background: Neuromyelitis optica (NMO) is an in?ammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. Seropositive antiAQP4 differentiates NMO from MS and the presence of manifestation in the postrema, brainsteam or diencephalic areas extend to NMO Spectrum Disorder (NMOSD). Case Description: A 18 years old male complain sudden vision loss on his left eye since 2 weeks ago. The examination show the visual acuity on the right eye was 6/6 and LPBP on the left eye. Positive RAPD on the left eye, funduscopy and the OCT RNFL within normal limits. Head MRI focus orbita with contrast show optic neuritis. Patient was diagnosed with left eye retrobulbar optic neuritis and ONTT therapy was given. The visual acuity improved to 1/60 then therapy change to oral steroid. Four months later, the patient suddenly got vision loss on the right eye accompanied by paraparesis. The visual acuity on the right eye was NLP and the left eye was 1/300, with mid-dilated papil. The results of the OCT RNFL show on the right eye edema papil and left eye atrophy papil. An MRI of the head focus orbital and whole spain was reexamined followed by VEP examination and an AntiAQP4 which showed an NMOSD. He was given ONTT then continued with immunosuppressants. The visual acuity of the right eye improved to 3/60 and the left eye remained 1/300. Discussion: This patient first presented with complain on the left eye with clinical and supporting symptoms suggesting an optic neuritis. The presence of a new attack on the right eye with paraparesis is a clinical feature of NMO supported by MRI results and seropositive AQP4 indicates an NMOSD. Conclusion: Establishment of diagnosis and administration of therapy quickly and precisely can reduce the severity and risk of recurrence which leads to greater disability and blindness. Key Words: Neuromyelitis Optica, Neuromyelitis Optica Spectrum Disorder, AQP4

Literature Commentary

Welcome to a new era in "Lit Commentary!"  I want to thank Tariq Bhatti for the past years of partnering with me in this endeavor. It has been a pleasure to collaborate with him.  We now welcome 2 new colleagues to Lit Commentary, Deborah I. Friedman and Marc J. Dinkin, who will work along with me. This is our first "Rodeo" together and you can see we all have different approaches to our reviews. We look forward to finding our groove and get in to some appropriate banter as we ride along! -Mark L. Moster, MD. In this issue of JNO Drs. Marc J. Dinkin, Deborah I. Friedman and Mark L. Moster discuss the following 6 articles: Tu TM, Yi SJ, Koh JS, Saffari SE, Hoe RHM, Chen GJ, Chiew HJ, Tham CH, Seet CYH, Yong MH, Yong KP, Hui AC, Fan BE, Tan BY, Quek AML, Seet RCS, Yeo LLL, Tan K, Thirugnanam UN. Incidence of cerebral venous thrombosis following SARS-CoV-2 infection vs mRNA SARS-CoV-2 vaccination in Singapore. JAMA Netw Open. 2022;5:e222940.Douglas RS, Dailey R, Subramanian PS, Barbesino G, Ugradar S, Batten R, Qadeer RA, Cameron C. Proptosis and diplopia response with teprotumumab and placebo vs the recommended treatment regimen with intravenous methylprednisolone in moderate-to-severe thyroid eye disease: a meta-analysis and matching-adjusted indirect comparison. JAMA Ophthalmol. 2022;140:328-335.Mikolajczyk B, Ritter A, Larson C, Connett J, Olson J, McClelland C, Lee MS. Red desaturation prevalence and severity in healthy patients. Neurol Clin Pract. 2022;12:1-5.Landau Prat D, Liu GT, Avery RA, Ying GS, Chen Y, Tomlinson LA, Revere KE, Katowitz JA, Katowitz WR. Recovery of vision after optic nerve sheath fenestration in children and adolescents with elevated intracranial pressure. Am J Ophthalmol. 2021;237:173-182.Hummert MW, Schoppe LM, Bellmann-Strobl J, Siebert N, Paul F, Duchow A, Pellkofer H, Kümpfel T, Havla J, Jarius S, Wildemann B, Berthele A, Bergh FT, Pawlitzki M, Klotz L, Kleiter I, Stangel M, Gingele S, Weber MS, Faiss JH, Pul R, Walter A, Zettl UK, Senel M, Stellmann J-P, Häußler V, Hellwig K, Ayzenberg I, Aktas O, Ringelstein M, Schreiber-Katz O, Trebst C; on behalf of the Neuromyelitis Optica Study Group (NEMOS). Costs and health-related quality of life in patients with NMO spectrum disorders and MOG-antibody-associated disease: CHANCENMO study. Neurology. 2022;98:e1184-e1196.Pineles SL, Henderson RJ, Repka MX, Heidary G, Liu GT, Waldman AT, Borchert MS, Khanna S, Graves JS, Collinge JE, Conley JA, Davis PL, Kraker RT, Cotter SA, Holmes JM; Pediatric Eye Disease Investigator Group. The pediatric optic neuritis prospective outcomes study-two-year results. Ophthalmology. 2022:S0161-6420(22)00234-2. doi: 10.1016/j.ophtha.2022.03.021. Epub ahead of print. PMID: 35364222.

Current Advancement of Immunomodulatory Drugs as Potential Pharmacotherapies for Autoimmunity Based Neurological Diseases.

Dramatic advancement has been made in recent decades to understand the basis of autoimmunity-mediated neurological diseases. These diseases create a strong influence on the central nervous system (CNS) and the peripheral nervous system (PNS), leading to various clinical manifestations and numerous symptoms. Multiple sclerosis (MS) is the most prevalent autoimmune neurological disease while NMO spectrum disorder (NMOSD) is less common. Furthermore, evidence supports the presence of autoimmune mechanisms contributing to the pathogenesis of amyotrophic lateral sclerosis (ALS), which is a neurodegenerative disorder characterized by the progressive death of motor neurons. Additionally, autoimmunity is believed to be involved in the basis of Alzheimer’s and Parkinson’s diseases. In recent years, the prevalence of autoimmune-based neurological disorders has been elevated and current findings strongly suggest the role of pharmacotherapies in controlling the progression of autoimmune diseases. Therefore, this review focused on the current advancement of immunomodulatory drugs as novel approaches in the management of autoimmune neurological diseases and their future outlook.

Immuno-pathogenesis of neuromyelitis optica and emerging therapies.

Neuromyelitis optica (NMO) is an inflammatory disease that resembles MS in the relapsing clinical course of optic neuritis and myelitis. Two decades of studies have revealed that autoantibodies, reactive to the water channel protein aquaporin 4 (AQP4)are detected in the core group of patients. These autoantibodies play a crucial role in the inflammatory pathology of NMO, involving proinflammatory cytokines, chemokines, and various inflammatory cells such as Th17 cells. Anti-AQP4 antibody-positive NMO differs fundamentally from MS, particularly in the responsiveness to therapies and the neuropathology accompanying destruction of astrocytes. Research into the immunological mechanism has led to the identification of possible targets of therapy, including complement pathway and interleukin-6 (IL-6) receptor signaling. Recent randomized controlled clinical trials have shown the remarkable efficacy of antibodies specific for complement C5, IL-6 receptor, and CD19+ B cells in prevention of NMO spectrum disorder relapses, although no such effects were found in anti-AQP4 antibody-negative patients. These results imply that anti-AQP4 antibody is a biomarker predicting the efficacy of therapies, and indicate the future direction towards "precision medicine."

Factors Influencing Social Participation Among Persons With Neuromyelitis Optica Spectrum Disorders: A Cross-Sectional Study.

BackgroundNeuromyelitis optica spectrum disorders seriously hinder social participation. Only clarifying the status of social participation and the factors most strongly influencing it can promote better-targeted support for those with neuromyelitis optica spectrum disorders.ObjectivesTo explore the factors influencing social participation among persons with neuromyelitis optica spectrum disorders.Methods138 persons with neuromyelitis optica were recruited by convenience sampling. They completed the Impact on Participation and Autonomy Questionnaire, and were rated using the Barthel index, the Beck Anxiety Inventory and the Beck Depression Inventory. The data were used to evaluate a generalized linear model predicting social participation.ResultsThe respondents mostly perceived good or very good possibilities of social participation, but participation outdoors and in social relations were considered more restricted. Ability in the activities of daily living, education level, bladder and bowel dysfunction, and depression were four significant predictors of social participation.ConclusionsStrengthening training in the activities of daily living, continuing education, giving positive psychological support and helping with bladder and bowel problems may help to promote social participation among those with neuromyelitis optica spectrum disorders.

A Case of Neuromyelitis Optica Spectrum Disorder of a Patient Who Present for Fungal Sinusitis

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system, characterized by repeatedly invading the brain, optic nerve, or spinal cord. In 2004, when a disease-specific antibody (aquaporin-4-immunoglobulin G) was discovered in the serum of an optic neuromyelitis patient, it was thought to be a separated disease from multiple sclerosis. Since 2004, however, the concept of the disease has been expanded to include NMO spectrum disorder (NMOSD). We experienced a rare case of NMOSD in a patient who presented with left visual disturbance and underwent endoscopic sinus surgery because of acute fungal sinusitis. We report this case with a review of the literature.

Neuromyelitis optica spectrum: a case report

&lt;p class="abstract"&gt;Neuromyelitis optica (NMO) or Devic's disease is a rare inflammatory, demyelinating autoimmune disease of the central nervous system that primarily affects women, most commonly in their 30s and 40s. NMOSD has been linked to several different illnesses including autoimmune disorders. Recurrent bouts of optic neuritis, myelitis and brainstem disorders are caused by serum antibodies (Abs) against the aquaporin-4 (AQP4) water channel. No AQP4-Abs are detected in some patients with NMOSD symptoms, but Abs against myelin-oligodendrocyte-glycoprotein (MOG). The term MOG-encephalomyelitis is now commonly used to describe this clinical disorder. Long segments of inflammation in the spinal cord (myelitis), severe optic neuritis and bouts of persistent vomiting and hiccoughs are all characteristics symptoms. This was the case of 30 year old female who presented with a chief complaint of weakness of both lower limbs, unable to walk, tingling and numbness. Based on her MRI findings revealed that the patient was diagnosed with NMO spectrum. She was treated with immunosuppressants and corticosteroids.&lt;/p&gt;

Patient with Systemic Lupus Erythematosus who Presented with Area Postrema Syndrome after COVID-19 Vaccination

Area postrema syndrome is a disorder that is included within the neuromyelitis optica spectrum (NMOSD), a group of severe inflammatory disorders of the central nervous system that primarily affect the optic nerve and spinal cord. The cause of NMOSD is unknown, but it has been linked to infections, vaccines and associated with autoimmune diseases. We present the case of a patient with a history of systemic lupus erythematosus who presented with area postrema syndrome after vaccination with COVID-19.

Costs and Health-Related Quality of Life in Patients With NMO Spectrum Disorders and MOG-Antibody-Associated Disease: CHANCENMO Study.

Background and ObjectivesTo evaluate costs and health-related quality of life (HRQoL) of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD).MethodsIn this multicenter cross-sectional study, data on consumption of medical and nonmedical resources and work ability were assessed via patient questionnaires. Costs were analyzed in Euros for 2018 from the societal perspective. HRQoL was captured by the EuroQoL Group 5 Dimension 5 Level Scale (EQ-5D-5L) questionnaire. Clinical data were retrieved from the Neuromyelitis Optica Study Group (NEMOS) database.ResultsTwo hundred twelve patients (80% women, median age 50 [19–83] years, median disease duration 7 [0–43] years, median Expanded Disability Status Scale [EDSS] score 3.5 [0–8.5], 66% aquaporin-4 immunoglobulin G [IgG] positive, 22% MOG IgG positive, 12% double seronegative) were analyzed. The mean total annual per capita cost of illness accounted for €59,574 (95% CI 51,225–68,293 or US dollars [USD] 70,297, 95% CI 60,445–80,586), and the mean index value of the EQ-5D-5L was 0.693 (95% CI 0.65–0.73). The most important cost drivers were informal care costs (28% of total costs), indirect costs (23%), and drugs (16%), especially immunotherapeutics. Costs showed a positive correlation with disease severity (ρ = 0.56, 95% CI 0.45–0.65); in the EDSS score 6.5 to 8.5 subgroup, the mean annual costs were €129,687 (95% CI 101,946–160,336 or USD 153,031, 95% CI 120,296–189,196). The HRQoL revealed a negative correlation to disease severity (ρ = −0.69, 95% CI −0.76 to −0.61); in the EDSS score 6.5 to 8.5 subgroup, the EQ-5D-5L mean index value was 0.195 (95% CI 0.13–0.28). Neither antibody status nor disease duration influenced the total annual costs or HRQoL.DiscussionThese German data from the era without approved preventive immunotherapies show enormous effects of the diseases on costs and quality of life. An early and cost-effective therapy should be provided to prevent long-term disability and to preserve quality of life.