scholarly journals Clinical and Prognostic Aspects of Patients With The Neuromyelitis Optic Spectrum Disorder (NMOSD) From a Cohort in Northeast Brazil

Thiago Gonçalves Fukuda ◽  
Ivã Taiuan Fialho Silva ◽  
Tayla Samanta Silva dos Santos ◽  
Marcos Baruch Portela Filho ◽  
Fernanda Ferreira de Abreu ◽  

Abstract Introduction: Neuromyelitis optic spectrum disorders (NMOSD) is a rare inflammatory and demyelinating disease of the central nervous system (CNS) more frequent in women and Afro-descendants. No previous epidemiological or prognostic study has been conducted in the region of the state of Bahia, Brazilian Northeast. Objective: To evaluate clinical and prognostic aspects in patients with NMOSD from a cohort in northeastern Brazil. Material and Methods: A single-center retrospective study was conducted with consecutive patients diagnosed with NMOSD. Clinical and epidemiological characteristics were described. The degree of disability was expressed by the Expanded Disability Status Scale (EDSS). Worsening disability were analyzed through negative binomial regression adjusted for disease duration. Results: Ninety-one patients were included, 72 (79.1%) female and 67 (73.6%) afro descendants. Mean age at onset was 36 (± 14) years and 73.3% were anti-aquaporin-4 antibody positive. Isolated transverse myelitis (32.9%) and isolated optic neuritis (22.4%) were the most frequent initial clinical syndromes. After multivariate analysis, optic neuritis (RR = 0.15; 95% CI=0.03 – 0.59; p = 0.008) and dyslipidemia (RR = 0.07; 95% CI=0.04 – 0.40; p < 0.001) were associated with slower disease progression. Area postrema involvement (RR = 29.69; 95% CI=3.40 – 226.07; p = 0.002) and age at onset (RR = 1.05; 95% CI=1.00 – 1.10; p = 0.037) were associated with faster disease progression. Conclusions: In the first clinical and prognostic study in northeastern Brazil, we identified area postrema involvement, age at onset, optic neuritis at fist syndrome and dyslipidemia as the main prognostic factors associated with disease progression.

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Marco A. Lana-Peixoto ◽  
Natália Talim

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.

2009 ◽  
Vol 15 (7) ◽  
pp. 802-810 ◽  
EA Yeh ◽  
B Weinstock-Guttman ◽  
N Lincoff ◽  
J Reynolds ◽  
A Weinstock ◽  

Purpose To evaluate retinal nerve fiber layer thickness (RNFLT) using optical coherence tomography (OCT) in children with acquired demyelinating diseases. Methods This is a cross-sectional study of patients seen between 2006–2008 at the Pediatric MS Center of the Jacobs Neurological Institute. Consensus definitions for pediatric demyelinating disease were followed. All children received OCT testing and assessment of visual acuity (VA) using Snellen and low contrast letter acuity (LCLA) charts. Results Thirty-eight children diagnosed with acquired demyelinating disease, 15 healthy controls, and five children with other neurological disorders (OND) were included. Average RNFLT in healthy controls was 107 ± 12 μm( n = 30) versus 108 ± 5 μm ( n = 10) in OND controls. In children with multiple sclerosis, average RNFLT ± SD was 99 ± 14 μm in unaffected ( n = 24) versus 83 ± 12 μmin eyes affected by optic neuritis (“affected eyes”) ( n = 10). Average RNFLT in children with acute disseminated encephalomyelitis and transverse myelitis was 102 ± 15 μm in unaffected ( n = 18) versus 67 ± 17 μm in affected eyes ( n = 6). In children with optic neuritis (ON), average RNFLT ± SD was 97 ± 13 μm in unaffected ( n = 5) versus 89 ± 12 μm in affected eyes ( n = 9). Differences between children with demyelinating disease and controls and between ON and nonON eyes were statistically significant ( P < 0.001). Bivariate correlations of RNFLT with LCLA ( P = 0.002) and VA ( P < 0.001) were significant. Conclusions OCT may be a valuable tool for the assessment and monitoring of anterior optic pathway dysfunction in children with demyelinating diseases.

Massimo Fabiani ◽  
Alberto Mateo-Urdiales ◽  
Xanthi Andrianou ◽  
Antonino Bella ◽  
Martina Del Manso ◽  

Background International literature suggests that disadvantaged groups are at higher risk of morbidity and mortality from SARS-CoV-2 infection due to poorer living/working conditions and barriers to healthcare access. Yet, to date, there is no evidence of this disproportionate impact on non-national individuals, including economic migrants, short-term travellers, and refugees. Methods We analysed data from the Italian surveillance system of all COVID-19 laboratory-confirmed cases tested positive from the beginning of the outbreak (20th of February) to the 19th of July 2020. We used multilevel negative-binomial regression models to compare the case-fatality rate and the rate of admission to hospital and intensive care unit (ICU) between Italian and non-Italian nationals. The analysis was adjusted for differences in demographic characteristics, pre-existing comorbidities, and period of diagnosis. Results We analysed 213,180 COVID-19 cases, including 15,974 (7.5%) non-Italian nationals. We found that, compared to Italian cases, non-Italian cases were diagnosed at a later date and were more likely to be hospitalised [(adjusted relative risk (ARR)=1.39, 95% confidence interval (CI): 1.33-1.44)] and admitted to ICU (ARR=1.19, 95% CI: 1.07-1.32), with differences being more pronounced in those coming from countries with lower HDI. We also observed an increased risk of death in non-Italian cases from low-HDI countries (ARR=1.32, 95% CI: 1.01-1.75). Conclusions A delayed diagnosis in non-Italian cases could explain their worse outcomes compared to Italian cases. Ensuring early access to diagnosis and treatment to non-Italians could facilitate the control of SARS-CoV-2 transmission and improve health outcomes in all people living in Italy, regardless of nationality.

2018 ◽  
Vol 25 (3) ◽  
pp. 325-329 ◽  
Carlos R Camara-Lemarroy ◽  
Jodie M Burton

In this topical review, we discuss the history of the area postrema syndrome, with special attention given to early studies aimed at identifying the area postrema and its function, possible early cases of the syndrome and its current relevance in neuroimmunology and demyelinating diseases. In 1896, Retzius named a structure in the posterior medulla oblongata as the area postrema. The work of Borison in the middle of the 20th century led to the elucidation of its function as a “vomiting center.” The historical medical literature is filled with excellent examples that could be described as “area postrema syndrome.” While severe and bilateral optic neuritis and transverse myelitis still constitute the classic components of neuromyelitis optica spectrum disorder (NMOSD), intractable vomiting and hiccups due to area postrema involvement is now recognized as essentially pathognomonic, indeed a shiny pearl in neuroimmunology and demyelinating diseases.

2014 ◽  
Vol 7 ◽  
pp. CCRep.S15177 ◽  
Mohammad Adawi ◽  
Bishara Bisharat ◽  
Abdalla Bowirrat

Neuromyelitis optica (NMO) is usually a relapsing demyelinating disease of the central nervous system associated with optic neuritis, transverse myelitis involving three or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody. NMO is often mistaken for multiple sclerosis and there are relatively sporadic publications about NMO and overlapping systemic or organ-specific autoimmune diseases, such as systemic lupus erythematosus (SLE). We described a unique case of a 25-year-old Arab young woman who was diagnosed with SLE, depending on clinical, laboratory investigations and after she had fulfilled the diagnostic criteria for SLE and had presented the following findings: constitutional findings (fatigue, fever, and arthralgia); dermatologic finding (photosensitivity and butterfly rash); chronic renal failure (proteinuria up to 400 mg in 24 hours); hematologic and antinuclear antibodies (positivity for antinuclear factor (ANF), anti-double-stranded DNA antibodies, direct Coombs, ANA and anti-DNA, low C4 and C3, aCL by IgG and IgM). Recently, she presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiological, and laboratory findings especially seropositivity for NMO-IgG were compatible with NMO. Accurate diagnosis is critical to facilitate initiation of immunosuppressive therapy for attack prevention. This case illustrates that NMO may be associated with SLE.

2006 ◽  
Vol 22 (4) ◽  
pp. 197-206 ◽  
Brian G. Weinshenker ◽  
Dean M. Wingerchuk ◽  
Sean J. Pittock ◽  
Claudia F. Lucchinetti ◽  
Vanda A. Lennon

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that principally targets the optic nerves and spinal cord and often leads to severe disability and occasionally life threatening respiratory failure. Although its clinical manifestations overlap with those of multiple sclerosis (MS), in established cases these two conditions can be distinguished on the basis of clinical, radiological, and routine spinal fluid studies. The diagnosis in early cases or limited forms of NMO is difficult. We recently discovered a unique IgG autoantibody (NMO-IgG) that is highly specific to patients with NMO and thus a valuable diagnostic aid. Its antigen, aquaporin-4 (AQP4), is the central nervous system’s predominant water channel protein. This antibody has not yet been proven to be pathogenic, but several facts suggest that it might be, including the similarity of the immunohistochemical pattern of NMO-(AQP4) IgG binding to mouse CNS tissues to the pattern of immune complex deposition in autopsied patients’ spinal cord tissue. The spectrum of diseases identified by NMO-IgG is broader than has previously been recognized clinically and includes incomplete forms of NMO, such as recurrent transverse myelitis without optic neuritis and recurrent optic neuritis without myelitis.

2020 ◽  
Vol 19 (6) ◽  
pp. 376-385
Md. A. Islam ◽  
Shoumik Kundu ◽  
Rosline Hassan

Multiple Sclerosis (MS) is the most common autoimmune demyelinating disease of the Central Nervous System (CNS). It is a multifactorial disease which develops in an immune-mediated way under the influences of both genetic and environmental factors. Demyelination is observed in the brain and spinal cord leading to neuro-axonal damage in patients with MS. Due to the infiltration of different immune cells such as T-cells, B-cells, monocytes and macrophages, focal lesions are observed in MS. Currently available medications treating MS are mainly based on two strategies; i) to ease specific symptoms or ii) to reduce disease progression. However, these medications tend to induce different adverse effects with limited therapeutic efficacy due to the protective function of the blood-brain barrier. Therefore, researchers have been working for the last four decades to discover better solutions by introducing gene therapy approaches in treating MS generally by following three strategies, i) prevention of specific symptoms, ii) halt or reverse disease progression and iii) heal CNS damage by promoting remyelination and axonal repair. In last two decades, there have been some remarkable successes of gene therapy approaches on the experimental mice model of MS - experimental autoimmune encephalomyelitis (EAE) which suggests that it is not far that the gene therapy approaches would start in human subjects ensuring the highest levels of safety and efficacy. In this review, we summarised the gene therapy approaches attempted in different animal models towards treating MS.

2021 ◽  
pp. jech-2020-215039 ◽  
Anders Malthe Bach-Mortensen ◽  
Michelle Degli Esposti

IntroductionThe COVID-19 pandemic has disproportionately impacted care homes and vulnerable populations, exacerbating existing health inequalities. However, the role of area deprivation in shaping the impacts of COVID-19 in care homes is poorly understood. We examine whether area deprivation is linked to higher rates of COVID-19 outbreaks and deaths among care home residents across upper tier local authorities in England (n=149).MethodsWe constructed a novel dataset from publicly available data. Using negative binomial regression models, we analysed the associations between area deprivation (Income Deprivation Affecting Older People Index (IDAOPI) and Index of Multiple Deprivation (IMD) extent) as the exposure and COVID-19 outbreaks, COVID-19-related deaths and all-cause deaths among care home residents as three separate outcomes—adjusting for population characteristics (size, age composition, ethnicity).ResultsCOVID-19 outbreaks in care homes did not vary by area deprivation. However, COVID-19-related deaths were more common in the most deprived quartiles of IDAOPI (incidence rate ratio (IRR): 1.23, 95% CI 1.04 to 1.47) and IMD extent (IRR: 1.16, 95% CI 1.00 to 1.34), compared with the least deprived quartiles.DiscussionThese findings suggest that area deprivation is a key risk factor in COVID-19 deaths among care home residents. Future research should look to replicate these results when more complete data become available.

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