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...
Introduction. Observation of open-ring enhancement in magnetic resonance imaging (MRI) is considered a specificity marker for diagnosing pseudotumoral multiple sclerosis (MS). This finding is of great value in the differential diagnosis of tumefactive lesions. Case Report. We describe a 55-year-old white woman, with previous history of ovarian cancer and recent history of fatigue and bilateral retroorbital pain. Important bilateral visual impairment evolved over one month. Physical examination detected the presence of right homonymous hemianopia. Cranial MRI showed an expanding lesion with open-ring enhancement. Given the range of diagnostic possibilities, a stereotactic biopsy was performed, and histopathological examination was consistent with an active demyelinating disease. The patient was treated with 1 g of methylprednisolone and symptoms improved following a significant reduction in the lesion. Conclusions. We highlight the MRI results suggestive of pseudotumoral MS, especially open-ring enhancement, which is an important radiologic aspect to diagnosis and can assist in avoiding unnecessary biopsies.
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.
RESUMO: Os traumas sobre a coluna resultantes de projetil de arma de fogo (PAF) são lesões geralmente graves e muitas vezes com baixo potencial para recuperação neurológica. A indicação cirúrgica destas lesões ainda é motivo de controvérsia. O objetivo deste artigo é identificar fatores no pré e trans-operatório que irão influenciar na recuperação neurológica destes pacientes. Realizamos estudo retrospectivo de 45 pacientes submetidos a laminectomia para trauma raquimedular por PAF, avaliando os seguintes fatores: nível da lesão, apresentação clínica, uso de glicocorticóide no pré-operatório, presença de lesão dural, momento cirúrgico e relação entre escala de Frankel pré e pós-operatória. Observamos que os fatores mais importantes para a recuperação neurológica foram o nível da lesão (53% dos pacientes com lesão lombar melhoraram após a cirurgia) e a apresentação clínica pré-operatória (pacientes com síndrome de cauda equina obtiveram melhora em 60% dos casos), sendo que as demais variáveis não apresentaram significância estatística. Em 71% dos casos, a dor pré-operatória foi aliviada com o procedimento cirúrgico. PALAVRAS-CHAVE: trauma raquimedular, projetil de arma de fogo, prognóstico.Prognostic factors related to gunshot wounds to the spine in patients submited to laminectomy ABSTRACT -The spinal trauma related to civilian gunshot missile still remains a serious neurological event that carries a dismal prognosis almost in all cases. Its surgical indication also is a mather of discution. Our goal is to identify the aspects that could influence the prognosis after surgery to this kind of lesions. We conducted a retrospective study of 45 consecutive patients submitted to laminectomy at Hospital de Base do Distrito Federal (Brasília, Brazil), testing the following aspects: initial neurological status, level of the deficit, surgical timing, use of methilprednisolone and presence of dural tearing. Among those, the initial clinical presentation and the level of the lesion (60% of the patients with cauda equina syndrome and 53% of that with lesions in the lombar region improved their neurological status after laminectomy) were the most important factors affecting the outcome. Seventy percent of the patients experienced a pain relief after the surgical procedure.KEY-WORDS: spinal trauma, gunshot wound, prognosis.O traumatismo raquimedular (TRM) penetrante, seja ele provocado por projetil de arma de fogo (PAF) ou objetos perfurantes constitui lesão grave e geralmente associada a mau prognóstico neurológico 1 . Quando um projetil atinge a coluna vertebral provoca lesões primárias devido ao trauma mecânico direto e trauma térmico sobre as estruturas neurais e vasculares. Posteriormente pode desencadear lesões secundárias, tanto por alterações isquêmicas como hiperêmicas sobre o
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