ImportanceDifferential diagnosis of patients with seronegative demyelinating central nervous system (CNS) disease is challenging. In this regard, evidence suggests that immunoglobulin (Ig) A plays a role in the pathogenesis of different autoimmune diseases. Yet little is known about the presence and clinical relevance of IgA antibodies against myelin oligodendrocyte glycoprotein (MOG) in CNS demyelination.ObjectiveTo investigate the frequency of MOG-IgA and associated clinical features in patients with demyelinating CNS disease and healthy controls.Design, Setting, and ParticipantsThis longitudinal study comprised 1 discovery and 1 confirmation cohort derived from 5 centers. Participants included patients with suspected or confirmed demyelinating diseases and healthy controls. MOG-IgA, MOG-IgG, and MOG-IgM were measured in serum samples and cerebrospinal fluid (CSF) of patients, who were assessed from September 2012 to April 2022.Main Outcomes and MeasuresFrequency and clinical features of patients who were seropositive for MOG-IgA and double-seronegative for aquaporin 4 (AQP4) IgG and MOG-IgG.ResultsAfter the exclusion of 5 participants with coexisting AQP4-IgG and MOG-IgA, MOG-IgG, and/or MOG-IgM, 1339 patients and 110 healthy controls were included; the median follow-up time was 39 months (range, 0-227 months). Of included patients with isolated MOG-IgA, 11 of 18 were female (61%), and the median age was 31.5 years (range, 3-76 years). Among patients double-seronegative for AQP4-IgG and MOG-IgG (1126/1339; 84%), isolated MOG-IgA was identified in 3 of 50 patients (6%) with neuromyelitis optica spectrum disorder, 5 of 228 patients (2%) with other CNS demyelinating diseases, and 10 of 848 patients (1%) with multiple sclerosis but in none of the healthy controls (0/110). The most common disease manifestation in patients seropositive for isolated MOG-IgA was myelitis (11/17 [65%]), followed by more frequent brainstem syndrome (7/16 [44%] vs 14/75 [19%], respectively; P = .048), and infrequent manifestation of optic neuritis (4/15 [27%] vs 46/73 [63%], respectively; P = .02) vs patients with MOG-IgG. Among patients fulfilling 2017 McDonald criteria for multiple sclerosis, MOG-IgA was associated with less frequent CSF-specific oligoclonal bands (4/9 [44%] vs 325/351 [93%], respectively; P < .001) vs patients with multiple sclerosis who were MOG-IgG/IgA seronegative. Further, most patients with isolated MOG-IgA presented clinical attacks after recent infection or vaccination (7/11 [64%]).Conclusion and RelevanceIn this study, MOG-specific IgA was identified in a subgroup of patients who were double-seronegative for AQP4-/MOG-IgG, suggesting that MOG-IgA may be a novel diagnostic biomarker for patients with CNS demyelination.

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Introdução: Um quarto dos pacientes com acidente vascular cerebral (AVC) despertam com os déficits neurológicos, uma condição denominada wake-up stroke (WUS). A ressonância magnética (RM) vem sendo cada vez mais utilizada na fase aguda desses casos para estimar de forma confiável que o evento cerebrovascular ocorreu em menos de 4.5 horas, tornando esses pacientes candidatos à trombólise endovenosa. O objetivo deste estudo é relatar o primeiro caso de WUS trombolisado na Irmandade da Santa Casa de Misericórdia de São Paulo e difundir essa importante modalidade de tratamento. Relato do Caso: Paciente do sexo feminino, de 73 anos, admitida com relato de ter despertado com novo déficit neurológico focal, após ter ido dormir assintomática. Ao exame neurológico, apresentava acometimento de múltiplos nervos cranianos, disartria e ataxia à esquerda. RM de crânio evidenciou lesão em região pontina com aumento do sinal na sequência de imagem ponderada em difusão (DWI) e ausência de imagem correspondente na sequência de recuperação de inversão atenuada por fluido (FLAIR), caracterizando o mismatch DWI-FLAIR. A paciente foi submetida à trombólise endovenosa, com melhora completa dos déficits até o final da internação. Conclusão: O caso descrito é comum na prática clínica, porém o uso do trombolítico guiado pela RM ainda é pouco empregado. O conhecimento de que técnicas de neuroimagem podem ser utilizadas como indicativo de janela terapêutica para trombólise é de fundamental importância, uma vez que pode beneficiar uma parcela significativa de casos selecionados, melhorando consideravelmente o desfecho funcional. Palavras chave: Acidente vascular cerebral, Trombólise terapêutica, AlteplaseABSTRACTIntroduction: One in every four patients with ischemic stroke awakens with the neurologic deficits, a condition named wake-up stroke (WUS). Magnetic resonance imaging (MRI) has been increasingly used in the acute phase of these cases to reliably estimate that the event occurred in less than 4.5 hours, identifying those that could benefit from reperfusion treatment. The aim of this study is to report the first case of WUS treated with alteplase in the Santa Casa of Sao Paulo Hospital and to disseminate this important treatment modality. Case Report: A 73-year-old woman admitted with a complaint of waking-up with a new focal neurologic symptom after going to sleep asymptomatic. On neurologic exam, she presented multiple cranial nerves disfunctions, dysarthria and left ataxia. MRI showed a left pontine lesion with increased signal in diffusion weighted imaging (DWI) sequence and the absence of a corresponding image in fluid-attenuated inversion recovery (FLAIR) sequence, characterizing the DWI-FLAIR mismatch. The patient underwent intravenous thrombolysis with complete resolution of neurologic deficits before hospital discharge. Conclusion: The reported case is common in daily practice; however, MRI-guided thrombolysis is not generally performed. Knowledge that neuroimaging techniques can be used to determine the therapeutic window for thrombolysis is of fundamental importance since it can benefit a substantial group of selected patients by considerably improving their functional outcomes. Keywords: Stroke, Thrombolytic therapy, Alteplase

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