Persistent MOG-IgG positivity is a predictor of recurrence in MOG-IgG-associated optic neuritis, encephalitis and myelitis.

BACKGROUND: MOG-IgG-associated optic neuritis, encephalitis and myelitis (MONEM) is a recently recognized group of inflammatory central nervous system (CNS) disorders distinct from multiple sclerosis and neuromyelitis optica spectrum disorders. Limited data are available regarding the predictors of relapse in this condition. OBJECTIVE: We aimed to evaluate the longitudinal serostatus of patients with MOG-IgG and to correlate serostatus with long-term clinical outcomes. METHODS: Of 574 consecutive patients who presented with demyelinating inflammatory CNS disorders, we included 31 patients who were MOG-IgG-positive. Patients with MOG-IgG were followed up from 2011 to 2017 at the School of Medicine, University of São Paulo, Brazil. RESULTS: Relapsing disease occurred in 23 out of 31 patients (74%), while 8 (26%) exhibited a monophasic course. All monophasic patients, as well as the majority of relapsing patients, became seronegative during clinical remission. Patients exhibiting disease activity in the last 2 years were more likely to remain positive, with higher medium titres than those found in patients in clinical remission. CONCLUSION: MOG-IgG patients usually present with a relapsing course, and the risk of relapse was associated with longitudinally persistent MOG-IgG seropositivity. In contrast, patients who experienced a single attack became spontaneously seronegative for MOG-IgG during long-term follow-up.

Anti-MOG (Myelin Oligodendrocyte Glycoprotein)-Positive Severe Optic Neuritis with Optic Disc Ischaemia and Macular Star.

A 44-year-old man presented with severe right visual loss. The right fundus examination showed marked optic disc oedema associated with partial macular star. Serological blood tests for infectious agents were all negative. Serum aquaporin-4 antibody was negative but anti-MOG (myelin oligodendrocyte glycoprotein) was positive. Magnetic resonance revealed extensive lesion in right optic nerve. There was no visual improvement after intravenous therapy. Patient had no further attacks after follow-up. Optic disc oedema with macular star is found in several infectious and non-inflammatory disorders, but it has not been reported in optic neuritis (ON) associated with autoantibodies to myelin oligodendrocyte glycoprotein (anti-MOG).

MOG-IgG-Associated Optic Neuritis, Encephalitis, and Myelitis: Lessons Learned From Neuromyelitis Optica Spectrum Disorder.

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been found in some cases diagnosed as seronegative neuromyelitis optica spectrum disorder (NMOSD). MOG-IgG allowed the identification of a subgroup with a clinical course distinct from that of NMOSD patients who are seropositive for aquaporin-4-IgG antibodies. MOG-IgG is associated with a wider clinical phenotype, not limited to NMOSD, with the majority of cases presenting with optic neuritis (ON), encephalitis with brain demyelinating lesions, and/or myelitis. Therefore, we propose the term MOG-IgG-associated Optic Neuritis, Encephalitis, and Myelitis (MONEM). Depending on the clinical characteristics, these patients may currently be diagnosed with NMOSD, acute disseminated encephalomyelitis, pediatric multiple sclerosis, transverse myelitis, or ON. With specific cell-based assays, MOG-IgG is emerging as a potential biomarker of inflammatory disorders of the central nervous system. We review the growing body of evidence on MONEM, focusing on its clinical aspects.

[Analysis of electrocardiographic recordings associated with acute myocardial infarction]. / Análise de registros eletrocardiográficos associados ao infarto agudo do miocárdio.

OBJECTIVE: Evaluate correlations between variations in eletrocardiogram (ECG) recordings and acute myocardial infarction. METHODS: Use of a low-cost software to digitalize printed and/or ".pdf" file format ECG recordings. Calculation of ST-segment area and amplitudes of the J and Y points. RESULTS: The amplitude of the Y point holds maximum correlation with troponin concentration. ST-segment elevation is not a good statistical indicator of myocardial infarction severity. There is a strong negative correlation between the amplitude of the J point and the amount of magnesium ions, but no statistical correlation with sodium or calcium ions. Neither method for calculating the ST-segment area (pixel counts and interpolation) indicated any significant differences in the results. CONCLUSION: The software used proved to be functional and cost-effective. Y point amplitude is a sensitive marker of myocardial infarction, and is also a calculation method both simpler to use and less subject to error than the calculation of the ST-segment elevation area.

Mutational screening of 320 Brazilian patients with autosomal dominant spinocerebellar ataxia.

Autosomal dominant spinocerebellar ataxias (SCAs) are a clinical and genetically heterogeneous group of debilitating neurodegenerative diseases that are related to at least 36 different genetic loci; they are clinically characterized by progressive cerebellar ataxia and are frequently accompanied by other neurological and non-neurological manifestations. The relative frequency of SCA varies greatly among different regions, presumably because of a founder effect or local ethnicities. Between July 1998 and May 2012, we investigated 320 Brazilian patients with an SCA phenotype who belonged to 150 unrelated families with an autosomal dominant inheritance pattern and 23 sporadic patients from 13 Brazilian states. A total of 265 patients (82.8%) belonging to 131 unrelated families (87.3%) were found to have a definite mutation, and SCA3 accounted for most of the familial cases (70.7%), followed by SCA7 (6%), SCA1 (5.3%), SCA2 (2.7%), SCA6 (1.3%), SCA8 (0.7%) and SCA10 (0.7%). In the Ribeirão Preto mesoregion, which is located in the northeast part of São Paulo State, the prevalence of SCA3 was approximately 5 per 100,000 inhabitants, which is the highest prevalence found in Brazil. No mutation was found in the SCA12, SCA17 and DRPLA genes, and all the sporadic cases remained without a molecular diagnosis. This study further characterizes the spectrum of SCA mutations found in Brazilian patients, which suggests the existence of regional differences and demonstrates the expansion of the SCA8 locus in Brazilian families.

Análise de registros eletrocardiográficos associados ao infarto agudo do miocárdio / Analysis of electrocardiographic recordings associated with acute myocardial infarction

OBJETIVO: Avaliar correlações entre as variações do eletrocar¡diograma (ECG) e o infarto agudo do miocárdio. MÉTODOS: Uso de software de baixo custo para digitalização de ECG impressos e/ou em formato "pdf". Cálculo de área do segmento ST e das amplitudes dos pontos J e Y RESULTADOS: A amplitude do ponto Y possui máxima correlação com a concentração da enzima troponina. O supradesnivelamento do segmento ST não se constitui bom indicador estatístico da gravidade do infarto. Existe uma forte correlação negativa entre a amplitude do ponto J e a quantidade de íons magnésio, mas nenhuma correlação estatística com os íons sódio ou cálcio. Os dois métodos de cálculo da área do segmento ST (contagem de pixels e interpolação) não mostraram diferenças significativas nos resultados. CONCLUSÃO: O software utilizado mostrou-se viável do ponto de vista econômico e funcional. A amplitude do ponto Y é um marcador sensível à ocorrência do infarto, tendo cálculo mais simples e menos sujeito a erros do que o cálculo da área de supradesnivelamento do segmento ST.