Clinical Features of COVID-19 on Patients With Neuromyelitis Optica Spectrum Disorders.

BACKGROUND AND OBJECTIVES: To describe the clinical features and disease outcomes of coronavirus disease 2019 (COVID-19) in patients with neuromyelitis optica spectrum disorder (NMOSD). METHODS: The 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. RESULTS: Among 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. DISCUSSION: Most 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.

Acquired hemophilia A and other autoimmune diseases after alemtuzumab therapy for multiple sclerosis: A report of two cases.

Alemtuzumab (ALZ) is an anti-CD52 monoclonal antibody used to treat recurrent remittent multiple sclerosis (RRMS). After ALZ infusion, there is a depletion of T and B cells expressing CD52, while the stem cells and innate immune cells are spared. Longitudinal studies with long periods of follow-ups have reported ALZ-associated autoimmune diseases, such as thrombocytopenic purpura and thyroiditis. We report two patients who developed autoimmune hemophilia A or acquired hemophilia (AHA) after ALZ infusion, one of whom developed severe vitiligo. To the best of our knowledge, these two cases of ALZ-associated AHA are the first two cases to be reported in Brazil, and the fourth and fifth AHA cases to be reported worldwide. AHA is a potential life-threatening disease if not diagnosed and treated in a timely manner. The development of AHA should be cited as a possible adverse event, and specific coagulation tests must be part of the official recommendations for patient follow-ups.

Nine-year follow up after hematopoietic stem cell transplantation in five multiple sclerosis patients.

Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system. Its treatment has focused on inflammation control as early as possible to avoid disability. Autologous hematopoietic stem cell transplantation (AHSCT) has been used for treating MS since 1996, with recent decisive results regarding benefits in long-term efficacy. Five patients followed up at an MS center in Belo Horizonte, Brazil, who had relapsing-remitting MS with high disease activity, underwent AHSCT between 2009 and 2011. They were evaluated clinically, with magnetic resonance imaging, and by the EDSS every six months after transplantation, up to July 2018. The patients were four women and one man, with ages ranging from 25-50 years, and time since disease onset ranging from 4-17 years at the time of the procedure. Four patients improved, one patient was stabilized, and all patients were free of disease activity after 5-9 years. Through improving patient selection and decreasing the time from disease onset, AHSCT could stop epitope spreading and disease progression. Despite multiple other therapeutic choices being approved for relapsing-remitting MS, AHSCT continues to be a treatment to consider for aggressive MS disease.

Nine-year follow up after hematopoietic stem cell transplantation in five multiple sclerosis patients / Nove anos de seguimento após transplante autólogo de células tronco hematopoiéticas em cinco pacientes com esclerose múltipla

ABSTRACT Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system. Its treatment has focused on inflammation control as early as possible to avoid disability. Autologous hematopoietic stem cell transplantation (AHSCT) has been used for treating MS since 1996, with recent decisive results regarding benefits in long-term efficacy. Five patients followed up at an MS center in Belo Horizonte, Brazil, who had relapsing-remitting MS with high disease activity, underwent AHSCT between 2009 and 2011. They were evaluated clinically, with magnetic resonance imaging, and by the EDSS every six months after transplantation, up to July 2018. The patients were four women and one man, with ages ranging from 25-50 years, and time since disease onset ranging from 4-17 years at the time of the procedure. Four patients improved, one patient was stabilized, and all patients were free of disease activity after 5-9 years. Through improving patient selection and decreasing the time from disease onset, AHSCT could stop epitope spreading and disease progression. Despite multiple other therapeutic choices being approved for relapsing-remitting MS, AHSCT continues to be a treatment to consider for aggressive MS disease.

RESUMO A esclerose múltipla é uma doença imunomediada do sistema nervoso central. Seu tratamento tem sido focado no controle da inflamação o mais cedo possível para evitar incapacidade. O transplante autólogo de células tronco hematopoiéticas (TACTH) vem sendo usado para tratar esclerose múltipla desde 1996, e recentes resultados foram decisivos a respeito do benefício na eficácia a longo prazo. Cinco pacientes seguidos num centro de esclerose múltipla de Belo Horizonte, Brasil, que apresentavam forma clínica remitente recorrente com alta atividade de doença foram submetidos a esse tratamento de 2009 a 2011. Após o transplante foram avaliados clinicamente e com ressonância magnética, e escala de EDSS, a cada seis meses até julho de 2018. Os pacientes eram quatro mulheres e um homem, com idade entre 25 e 50 anos e tempo de doença variando de 4 a 17 anos na época do procedimento. Quatro pacientes melhoraram após a primeira avaliação e um manteve-se estável. Todos os pacientes permaneceram sem evidência de doença clínica ou radiológica de 5 a 9 anos após. Com uma seleção cada vez mais criteriosa de pacientes o TACTH pode interromper a disseminação do epítopo e controlar a progressão da doença. Apesar de várias outras opções terapêuticas aprovadas para esclerose múltipla o TACTH continua a ser uma forma de tratamento a ser considerada em casos de doença muito agressiva.

Disability as a determinant of fatigue in MS patients.

INTRODUCTION: Fatigue is one of the most frequent and disabling symptoms in multiple sclerosis (MS). Central, psychological, and peripheral factors may contribute to the occurrence of fatigue. OBJECTIVES: The current study aimed to evaluate potential fatigue determinants in patients with relapsing-remitting MS with a low functional impairment. METHODS: We compared inflammatory markers, respiratory pressures, disability, and quality of life in 39 relapsing-remitting MS patients with and without fatigue. RESULTS: Patients with relapsing-remitting MS with fatigue had higher Expanded Disability Status Scale scores (p = 0.002). We observed a significant association between the results of the Guy Neurological Disability Scale, the Functional Assessment of MS Quality of Life Rating Scale and the presence of fatigue (p < 0.05). CONCLUSIONS: The degree of functional impairment is a determinant for the presence of fatigue in MS patients, but respiratory function and inflammatory markers are not.

Disability as a determinant of fatigue in MS patients / Incapacidade como determinante da fadiga em pacientes com EM

ABSTRACT Fatigue is one of the most frequent and disabling symptoms in multiple sclerosis (MS). Central, psychological, and peripheral factors may contribute to the occurrence of fatigue. Objectives: The current study aimed to evaluate potential fatigue determinants in patients with relapsing-remitting MS with a low functional impairment. Methods: We compared inflammatory markers, respiratory pressures, disability, and quality of life in 39 relapsing-remitting MS patients with and without fatigue. Results: Patients with relapsing-remitting MS with fatigue had higher Expanded Disability Status Scale scores (p = 0.002). We observed a significant association between the results of the Guy Neurological Disability Scale, the Functional Assessment of MS Quality of Life Rating Scale and the presence of fatigue (p < 0.05). Conclusions: The degree of functional impairment is a determinant for the presence of fatigue in MS patients, but respiratory function and inflammatory markers are not.

RESUMO A fadiga é um dos sintomas mais frequentes e incapacitantes na esclerose múltipla (EM). Fatores centrais, psicológicos e periféricos podem contribuir para a ocorrência de fadiga. Objetivos: O presente estudo teve como objetivo avaliar potenciais determinantes de fadiga em pacientes com EM remitente-recorrente (EMRR) com baixo nível de incapacidade funcional. Métodos: Foram comparados marcadores inflamatórios, pressões respiratórias, incapacidade e qualidade de vida em 39 pacientes com EMRR com e sem fadiga. Resultados: Pacientes com EMRR com fadiga apresentaram maior Escala de Incapacidade Funcional Expandida (p = 0,002). Observamos uma associação significativa entre os resultados da Escala de Incapacidade Neurológica de Guy e Escala de Avaliação da Qualidade de Vida Funcional com a presença de fadiga (valores de p < 0,05). Conclusão: O grau de comprometimento funcional, mas não a função respiratória e os marcadores inflamatórios, são determinantes para a presença de fadiga em pacientes com EM.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis.

The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis / Consenso Brasileiro para o Tratamento da Esclerose Múltipla: Academia Brasileira de Neurologia e Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla

ABSTRACT The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

RESUMO O crescent arsenal terapêutico na esclerose múltipla (EM) tem permitido tratamentos mais efetivos e personalizados, mas a escolha e o manejo das terapias modificadoras da doença (TMDs) tem se tornado cada vez mais complexos. Neste contexto, especialistas do Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla e do Departamento Científico de Neuroimunologia da Academia Brasileira de Neurologia reuniram-se para estabelecer este Consenso Brasileiro para o Tratamento da EM, baseados no entendimento de que neurologistas devem ter a possibilidade de prescrever TMDs para EM de acordo com o que é melhor para cada paciente, com base em evidências e práticas atualizadas. Por meio deste documento, propomos recomendações práticas para o tratamento da EM, com foco principal na escolha e no manejo das TMDs, e revisamos os argumentos que embasam as estratégias de tratamento na EM.

Guideline for multiple sclerosis treatment in Brazil: Consensus from the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology.

Multiple sclerosis has become an ever-increasing challenge to neurologists. With the release of the latest medications on the market, Brazilian neurologists feel divided between following their patients' evolution in accordance with the strict rules established by the Brazilian Ministry of Health regarding drug distribution, or following disease progression and worsening in accordance with the evidence in the literature. Therefore, a systematic review of the main published treatment guidelines was conducted and an escalating therapy proposed for guiding multiple sclerosis patient treatment in Brazil.

Guideline for multiple sclerosis treatment in Brazil: Consensus from the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology / Orientações para tratamento da esclerose múltipla no Brasil: Consenso do Departamento Científico de Neuroimunologia da Academia Brasileira de Neurologia

ABSTRACT Multiple sclerosis has become an ever-increasing challenge to neurologists. With the release of the latest medications on the market, Brazilian neurologists feel divided between following their patients’ evolution in accordance with the strict rules established by the Brazilian Ministry of Health regarding drug distribution, or following disease progression and worsening in accordance with the evidence in the literature. Therefore, a systematic review of the main published treatment guidelines was conducted and an escalating therapy proposed for guiding multiple sclerosis patient treatment in Brazil.

RESUMO A esclerose múltipla vem se tornando um desafio crescente para os neurologistas. Com o lançamento de novos medicamentos no mercado, os neurologistas brasileiros se encontram divididos entre, apesar da evolução dos seus pacientes, seguir as regras restritas estabelecidas pelo Ministério da Saúde para distribuição de medicamentos, ou ao contrário, considerar a progressão e piora da doença, em concordância com as evidências da literatura. Devido a este impasse foi realizada uma revisão sistemática sobre as principais orientações de tratamento publicadas e foi proposto um escalonamento terapêutico para orientar o tratamento dos pacientes com esclerose múltipla no Brasil.

Determinants of quality of life in Brazilian patients with myasthenia gravis.

OBJECTIVES: The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. METHODS: This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. RESULTS: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. CONCLUSION: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.

Determinants of quality of life in Brazilian patients with myasthenia gravis

OBJECTIVES: The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. METHODS: This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. RESULTS: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. CONCLUSION: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.

Cerebrum-cervical arterial dissection in adults during sports and recreation / Dissecção arterial cérebro-cervical em adultos durante prática de esportes e recreação

Dissection of cervical arteries constitutes a medical emergency. Although relatively rarely, activities classified as sports and recreation may be a cause of arterial dissection independently of neck or head trauma. The purpose of the present paper was to present a series of cases of cerebrum-cervical arterial dissection in individuals during or soon after the practice of these sports activities. Methods Retrospective data on patients with arterial dissection related to sports and recreation. Results Forty-one cases were identified. The most frequently affected vessel was the vertebral artery. A large variety of activities had a temporal relationship to arterial dissection, and jogging was the most frequent of these. This is the largest case series in the literature. Conclusion Arterial dissection may be a complication from practicing sports.

A dissecção das artérias cervicais é uma emergência médica. Embora de forma relativamente rara, certas atividades descritas como esportes e recreação podem ser a causa de dissecção arterial independentemente de trauma de crânio ou cervical. O propósito do presente estudo é apresentar uma série de casos de dissecção de artérias cérebro-cervicais em indivíduos durante ou logo após a prática destas atividades desportivas. Métodos Dados retrospectivos de pacientes com dissecção arterial relacionada à prática de esportes e recreação. Resultados Quarenta e um casos foram identificados. A artéria mais frequentemente afetada foi a vertebral. Uma grande variedade de atividades teve relação temporal com a dissecção arterial, sendo a corrida a mais frequente delas. Esta é a maior série de casos da literatura. Conclusão Dissecção arterial pode ser uma complicação da prática de esportes.

Cerebrum-cervical arterial dissection in adults during sports and recreation.

UNLABELLED: Dissection of cervical arteries constitutes a medical emergency. Although relatively rarely, activities classified as sports and recreation may be a cause of arterial dissection independently of neck or head trauma. The purpose of the present paper was to present a series of cases of cerebrum-cervical arterial dissection in individuals during or soon after the practice of these sports activities. METHODS: Retrospective data on patients with arterial dissection related to sports and recreation. RESULTS: Forty-one cases were identified. The most frequently affected vessel was the vertebral artery. A large variety of activities had a temporal relationship to arterial dissection, and jogging was the most frequent of these. This is the largest case series in the literature. CONCLUSION: Arterial dissection may be a complication from practicing sports.

Clinical profile of patients with myasthenia gravis followed at the University Hospital, Federal University of Minas Gerais.

OBJECTIVE: to determine the clinical profile of patients with myasthenia gravis (MG); followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. METHODS: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. RESULTS: sixty nine patients were enrolled in the study. Fifty five (91%) subjects were female and the mean age (SD) was 37.6 (± 11.4) years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%), followed by the use of azathioprine (43%). There was no difference between thymectomized (42) and non-thymectomized (27) patients regarding disease severity and medication use. CONCLUSION: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature.

[Cannabinoids in neurology--Brazilian Academy of Neurology]. / Canabinoides e seu uso em neurologia--Academia Brasileira de Neurologia.

The use of cannabidiol in some neurological conditions was allowed by Conselho Regional de Medicina de São Paulo and by Agência Nacional de Vigilância Sanitária (ANVISA). Specialists on behalf of Academia Brasileira de Neurologia prepared a critical statement about use of cannabidiol and other cannabis derivatives in neurological diseases.

Clinical profile of patients with myasthenia gravis followed at the University Hospital, Federal University of Minas Gerais / Perfil clínico de pacientes com miastenia gravis em acompanhamento no Hospital das Clínicas, da Universidade Federal de Minas Gerais

Summary Objective: to determine the clinical profile of patients with myasthenia gravis (MG); followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. Methods: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. Results: sixty nine patients were enrolled in the study. Fifty five (91%) subjects were female and the mean age (SD) was 37.6 (±11.4) years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%), followed by the use of azathioprine (43%). There was no difference between thymectomized (42) and non-thymectomized (27) patients regarding disease severity and medication use. Conclusion: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature. .

Objetivo: determinar o perfil clínico dos pacientes com miastenia gravis (MG) atendidos na Clínica de Doenças Neuromusculares do Serviço de Neurologia do Hospital das Clínicas da Universidade Federal de Minas Gerais, e comparar com outras séries de casos brasileiras. Métodos: foram coletados dados clínicos e sociodemográficos dos pacientes, e realizada revisão sistemática de literatura, focando em estudos nacionais sobre o perfil clínico de pacientes com MG. Resultados: sessenta e nove pacientes participaram do estudo, sendo 55 (91%) do sexo feminino, e a média de idade (desvio-padrão) de 37,6 (±11,4) anos. O tempo médio de doença foi de 14,1 anos. Em relação ao tratamento, o uso isolado de prednisona foi a estratégia mais adotada (64%), seguida do uso de azatioprina (43,3%). Não houve diferença entre os pacientes timectomizados (47) e não timectomizados (22) no que diz respeito à gravidade da doença e ao tratamento. Conclusão: as características clínicas e sociodemográficas da presente amostra de pacientes com MG de um serviço universitário assemelham-se às características de outras séries brasileiras e da literatura internacional. .

Cannabinoids in neurology – Brazilian Academy of Neurology / Canabinoides e seu uso em neurologia – Academia Brasileira de Neurologia

O uso do canabidiol em algumas condições neurológicas foi liberado pelo Conselho Regional de Medicina de São Paulo e pela Agência Nacional de Vigilância Sanitária (ANVISA). Especialistas em nome da Academia Brasileira de Neurologia prepararam uma posição crítica sobre o uso do canabidiol e outros derivados da cannabis em doenças neurológicas.

The use of cannabidiol in some neurological conditions was allowed by Conselho Regional de Medicina de São Paulo and by Agência Nacional de Vigilância Sanitária (ANVISA). Specialists on behalf of Academia Brasileira de Neurologia prepared a critical statement about use of cannabidiol and other cannabis derivatives in neurological diseases.

Postpartum Treatment With Immunoglobulin Does Not Prevent Relapses of Multiple Sclerosis in the Mother.

Multiple sclerosis (MS) is a chronic, neurological, immune-mediated disease that can worsen in the postpartum period. There is no consensus on the use of immunoglobulin for prevention of disease relapses after delivery. We have shown that the controversial beneficial effect of immunoglobulin given immediately after birth could not be observed in patients with MS.

Supplementation and therapeutic use of vitamin D in patients with multiple sclerosis: consensus of the Scientific Department of Neuroimmunology of the Brazilian Academy of Neurology.

Multiple sclerosis (MS) is an inflammatory, autoimmune, demyelinating, and degenerative central nervous system disease. Even though the etiology of MS has not yet been fully elucidated, there is evidence that genetic and environmental factors interact to cause the disease. Among the main environmental factors studied, those more likely associated with MS include certain viruses, smoking, and hypovitaminosis D. This review aimed to determine whether there is evidence to recommend the use of vitamin D as monotherapy or as adjunct therapy in patients with MS. We searched PUBMED, EMBASE, COCHRANNE, and LILACS databases for studies published until September 9 th , 2013, using the keywords "multiple sclerosis", "vitamin D", and "clinical trial". There is no scientific evidence up to the production of this consensus for the use of vitamin D as monotherapy for MS in clinical practice.