The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients’ demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect immunofluorescence (IIF) with a composite substrate of mouse tissues in 200 NMOSD cases was positive in people with NMO (95/162; 58.6%), longitudinally extensive transverse myelitis (10/30; 33.3%) and bilateral or recurrent optic neuritis (8/8; 100%). No association of NMO-IgG antibody positivity was found with gender, age at onset, ethnicity, early or late onset forms, disease course, or long-term severe disability. The relative frequency of NMO among relapsing-remitting MS (RRMS) + NMO cases in SA was 14.0%. Despite the high degree of miscegenation found in SA, MS affects three quarters of all patients with IIDD, mainly white young women who share similar clinical characteristics to those in Western populations in the northern hemisphere, with the exception of ethnicity; approximately one-third of all cases occur among non-white individuals. At the last assessment, the majority of RRMS patients showed mild disability, and the risk for secondary progression was significantly superior among those of African ethnicity. NMO comprises 11.8% of all IIDD cases in SA...
SUMMARY:The aim of the study was to establish morphometrically the infraorbital foramen (IOF) location in the sagittal and transversal plan of the skull and to analyze the variations, as well as verifying the symmetry with the contralateral IOF. 210 skulls were analyzed and as exclusion criterion, were not analyzed skulls of children. Digital paquimeter was used and had been carried through two bilateral measures: a)sagittal measure; from infraorbital margin until the superior margin of the IOF; b) transversal measure, of the lateral margin of piriformis opening until the medial margin of the IOF, using the transversal plan. These measures had formed an angle of 90º between itself. The data were analyzed by the Kolmogorov-Smirnov to verify the Gaussian distribution and and Person's test to evaluate the correlation, considering significantly the differences that had presented a p≤ 0,05. The mean±SD in the sagital measures were respectively; right side 6,71±1.70 mm and 6,83±1.83 mm for left side (p≤0.0001). Transversal measures were: right side 13,28±2.17mm and 13,31±2.19 mm for left side (p≤0.0001). Were observed supranumerary foramens of the IOF, with presence of the double IOF in 50 cases, being bilateral in 08 specimens, left and right side 21 specimens for each side.The IOF are not absolutely symmetrical and may present pair bilateral and or unilateral opening. The presence of the supranumerary foraminas, as well as, the morphometrics measures in this study may intervene with the anesthesia, thus, suggest that they must be considered in the anaesthesical evaluation and be remembered during the procedures used for the localization of the forame. (Chonghuo, 1993;Chen, 1997;Esper et al. 1997Esper et al. ,1998, require more precise understanding of the surrounding anatomy (Bolini & Del Sol; Karakas et al.). Data on morphometric measurements regarding reference points should reduce risks during procedures, surgical operations and anesthesia (Zide & Swift; Karakas et al.; Du Tolt & Nortje). Therefore, the purpose of this study was to conduct morphometric measurements of the IOF location by using the sagittal and transversal plan of the Brazilian skull as reference, as well as verifying the symmetry with the contralateral IOF.Exclusion criterion of the sample: It was adopted like exclusion criterion all the child skulls and the skulls which the piriformis opening was damaged, unilaterally or bilaterally.In all the samples the presence of right maxilar joined to the left maxilar was considered indispensable to have the absolutely certainty that the studied foramens belonged to the same original skull and the statistic could express reliable results. The variables age, sex and race were not considered. Statistical analysis:The softwareGraph Prism was used in the statistical evaluation of measurement results. From these measurements means and standard deviations (mean±SD) were calculated. The data were analyzed by the KolmogorovSmirnov to verify the Gaussian distribution and and Person's test to evaluate the correlati...
Background and ObjectivesTo describe the clinical features and disease outcomes of coronavirus disease 2019 (COVID-19) in patients with neuromyelitis optica spectrum disorder (NMOSD).MethodsThe Neuroimmunology Brazilian Study Group has set up the report of severe acute respiratory syndrome (SARS-CoV2) cases in patients with NMOSD (pwNMOSD) using a designed web-based case report form. All neuroimmunology outpatient centers and individual neurologists were invited to register their patients across the country. Data collected between March 19 and July 25, 2020, were uploaded at the REDONE.br platform. Inclusion criteria were as follows: (1) NMOSD diagnosis according to the 2015 International Panel Criteria and (2) confirmed SARS-CoV2 infection (reverse transcription-polymerase chain reaction or serology) or clinical suspicion of COVID-19, diagnosed according to Center for Disease Control / Council of State and Territorial Epidemiologists (CDC/CSTE) case definition. Demographic and NMOSD-related clinical data, comorbidities, disease-modifying therapy (DMT), COVID-19 clinical features, and severity were described.ResultsAmong the 2,061 pwNMOSD followed up by Brazilian neurologists involved on the registry of COVID-19 in pwNMOSD at the REDONE.br platform, 34 patients (29 women) aged 37 years (range 8–77), with disease onset at 31 years (range 4–69) and disease duration of 6 years (range 0.2–20.5), developed COVID-19 (18 confirmed and 16 probable cases). Most patients exhibited mild disease, being treated at home (77%); 4 patients required admission at intensive care units (severe cases); and 1 patient died. Five of 34 (15%) presented neurologic manifestations (relapse or pseudoexacerbation) during or after SARS-CoV2 infection.DiscussionMost NMOSD patients with COVID-19 presented mild disease forms. However, pwNMOSD had much higher odds of hospitalization and intensive care unit admission comparing with the general Brazilian population. The frequency of death was not clearly different. NMOSD disability, DMT type, and comorbidities were not associated with COVID-19 outcome. SARS-CoV2 infection was demonstrated as a risk factor for NMOSD relapses. Collaborative studies using shared NMOSD data are needed to suitably define factors related to COVID-19 severity and neurologic manifestations.
IntroductionNeuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system. NMOSD starting after the age of 50 years is considered a “late onset” (LO-NMOSD) and seems to be particularly aggressive. The objective of this paper is to present a series of 37 Brazilian patients with LO-NMOSD.MethodsRetrospective data collection from medical records of patients with LO-NMOSD seen at 14 Brazilian specialized units.ResultsThe ratio of women to men in the sample was 4.3 to 1. The patients were followed up for a median period of 4 years. Sex, age at disease onset, and ethnic background were not associated with the number of relapses or disability outcomes. Extensive longitudinal myelitis affected 86% of patients, while optic neuritis affected 70%, and brainstem syndromes were present in only 16% of these patients. Six patients are currently using some type of support for walking or are wheelchair-bound. Three have died. Therapeutic options for NMOSD were particularly complicated for these elderly patients, since medications for controlling NMOSD are, in essence, immunosuppressive. Long-term use of corticosteroids can be an issue when the patients have high blood pressure, diabetes mellitus, or dyslipidemia (conditions often seen in elderly individuals).ConclusionThis series of LO-NMOSD cases highlights the importance of prompt diagnosis and treatment for these patients.
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