ABSTRACT Objective To describe the personal protective equipment training strategies during the beginning of the pandemic and to investigate the association between training and COVID-19 infection among healthcare workers. Methods This cross-sectional study conducted between March and May 2020 included 7,142 healthcare professionals who were eligible for both online and face-to-face simulation-based training on personal protective equipment use. Simulation training attendance was assessed by reviewing the attendance list, and the COVID-19 sick leave records recovered from the institutional RT-PCR database used to grant sick leave. The association between personal protective equipment training and COVID-19 was investigated using logistic regression, adjusted for sociodemographic and occupational characteristics. Results The mean age was 36.9 years (± 8.3), and 72.6% of participants were female. A total of 5,502 (77.0%) professionals were trained: 3,012 (54.7%) through online training, 691 (12.6%) through face-to-face training, and 1,799 (32.7%) through both strategies. During the study period, 584 (8.2%) COVID-19 cases were diagnosed among these professionals. The number of positive RT-PCR tests was 180 (11.0%) for untrained professionals, 245 (8.1%) for those trained only online, 35 (5.1%) for those trained face-to-face, and 124 (6.9%) for those trained with both strategies (p<0.001). Participants who received face-to-face training had a 0.43 lower risk of contracting COVID-19. Conclusion Personal protective equipment training decreased the odds of COVID-19 among healthcare professionals, with face-to-face simulation-based training being most effective.

RESUMO As encefalites autoimunes (EAI) são a principal causa de encefalite não-infecciosa. As manifestações neurológicas são variadas, incluindo alterações comportamentais ou psiquiátricas, disautonomia, transtornos do movimento e epilepsia. Habitualmente a instalação dos sintomas ocorre em até 6 semanas, de forma aguda ou subaguda. As EAI podem ser desencadeadas por tumores, quadros infecciosos virais ou ainda apresentar etiologia criptogênica. Este artigo revisa as principais EAI, estratégias de diagnóstico e tratamento.

ABSTRACT Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.

RESUMO O transplante de órgãos sólidos é um importante avanço no tratamento de doenças crônicas renal, hepática e cardíaca. Esta terapia tem aumentado a sobrevida e melhorado a qualidade de vida dos pacientes. Novas técnicas cirúrgicas e imunossupressores tem sido desenvolvidos para alcançar melhores desfechos. Entretanto, a variedade de complicações neurológicas que acompanham o transplante de órgãos sólidos é ampla, e carrega significado prognóstico. Pacientes podem ter acometimento do sistema nervoso central ou periférico devido a múltiplas causas que podem variar conforme o tempo após a realização da cirurgia, órgão transplantado e grau e tipo de terapia de imunossupressão. Manifestações neurológicas após o transplante de órgãos sólidos representam um desafio diagnóstico para médicos especialistas apesar de extensa investigação. O objetivo desta revisão é oferecer uma abordagem prática para ajudar neurologistas e clínicos a avaliar e manejar pacientes com transplante de órgãos sólidos que se apresentem com manifestações neurológicas agudas ou crônicas.

ABSTRACT Solid organ transplantation is a significant development in the treatment of chronic kidney, liver, heart and lung diseases. This therapeutic approach has increased patient survival and improved quality of life. New surgical techniques and immunosuppressive drugs have been developed to achieve better outcomes. However, the variety of neurological complications following solid organ transplantation is broad and carries prognostic significance. Patients may have involvement of the central or peripheral nervous system due to multiple causes that can vary depending on time of onset after the surgical procedure, the transplanted organ, and the intensity and type of immunosuppressive therapy. Neurological manifestations following solid organ transplantation pose a diagnostic challenge to medical specialists despite extensive investigation. This review aimed to provide a practical approach to help neurologists and clinicians assess and manage solid organ transplant patients presenting with acute or chronic neurological manifestations.

RESUMO Lúpus eritematoso sistêmico (LES) é uma doença autoimune crônica que envolve múltiplos órgãos e sistemas, caracterizada pela produção de auto anticorpos e lesão tecidual. A etiologia do LES é parcialmente conhecida e envolve interação entre fatores genéticos e ambientais. Até 50% dos pacientes com LES apresentam envolvimento neurológico no decorrer da doença. Manifestações neurológicas estão associadas a prejuízo na qualidade de vida e altas taxas de mortalidade e morbidade. Foram identificadas 19 síndromes neuropsiquiátricas em pacientes com LES, divididas entre manifestações do sistema nervoso central e periférico. O objetivo deste artigo é revisar as manifestações neuropsiquiátricas mais importantes. Serão abordadas as características clínicas, os aspectos radiológicos e opções de tratamento dos eventos neuropsiquiátricos.

ABSTRACT Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.

RESUMO A neurodegeneração com acúmulo cerebral de ferro (sigla em inglês NBIA) representa um grupo heterogêneo e complexo de doenças neurodegenerativas hereditárias, caracterizada pelo acúmulo cerebral de ferro, especialmente nos núcleos da base. O quadro clínico das NBIAs em geral inclui distúrbios do movimento, particularmente parkinsonismo e distonia, disfunção cognitiva, sinais piramidais e anormalidades da retina. As formas de NBIA descritas até o momento incluem neurodegeneração associada a pantothenase kinase (PKAN), neurodegeneração associada a phospholipase A2 (PLAN), neuroferritinopatia, aceruloplasminemia, neurodegeneração associada a beta-propeller protein (BPAN), síndrome de Kufor-Rakeb, neurodegeneração associada a mitochondrial membrane protein (MPAN), neurodegeneração associada a “fatty acid hydroxylase” (FAHN), neurodegeneração associada a coenzyme A synthase protein (CoPAN) e síndrome de Woodhouse-Sakati. Esta revisão é uma orientação para o diagnóstico das NBIAs, partindo das características clínicas e achados de neuroimagem, até a etiologia genética.

ABSTRACT Neurodegeneration with brain iron accumulation (NBIA) represents a heterogeneous and complex group of inherited neurodegenerative diseases, characterized by excessive iron accumulation, particularly in the basal ganglia. Common clinical features of NBIA include movement disorders, particularly parkinsonism and dystonia, cognitive dysfunction, pyramidal signs, and retinal abnormalities. The forms of NBIA described to date include pantothenase kinase-associated neurodegeneration (PKAN), phospholipase A2 associated neurodegeneration (PLAN), neuroferritinopathy, aceruloplasminemia, beta-propeller protein-associated neurodegeneration (BPAN), Kufor-Rakeb syndrome, mitochondrial membrane protein-associated neurodegeneration (MPAN), fatty acid hydroxylase-associated neurodegeneration (FAHN), coenzyme A synthase protein-associated neurodegeneration (CoPAN) and Woodhouse-Sakati syndrome. This review is a diagnostic approach for NBIA cases, from clinical features and brain imaging findings to the genetic etiology.

RESUMO As mielopatias não-traumáticas representam um grupo heterogêneo de doenças neurológicas. Poucos estudos descrevem os perfis clínico e epidemiológico sobre a experiência de serviços de referência. Objetivo Descrever as características clínicas de uma coorte de mielopatia não-traumática. Método Os registros clínicos de 166 pacientes atendidos entre 2001 e 2012 foram revisados e variáveis epidemiológicas, clínicas e radiológicas foram compiladas. Resultados O diagnóstico mais prevalente foi o de degeneração combinada subaguda (11,4%), seguido por mielopatia espondilótica cervical (9,6%), doenças desmielinizantes (9%), paraparesia espástica tropical (8,4%) e paraparesia espástica hereditária (8.4%). Aproximadamente 20% dos pacientes apresentaram mielopatia não-traumática de etiologia indeterminada, apesar da ampla investigação clínica, laboratorial e de neuroimagem. Conclusão Independentemente da ampla avaliação, muitos pacientes com mielopatia não-traumática apresentam etiologia incerta. Causas compressivas e deficiências nutricionais permanecem como etiologias importantes de mielopatias não-traumáticas.

ABSTRACT Non-traumatic myelopathies represent a heterogeneous group of neurological conditions. Few studies report clinical and epidemiological profiles regarding the experience of referral services. Objective To describe clinical characteristics of a non-traumatic myelopathy cohort. Method Epidemiological, clinical, and radiological variables from 166 charts of patients assisted between 2001 and 2012 were compiled. Results The most prevalent diagnosis was subacute combined degeneration (11.4%), followed by cervical spondylotic myelopathy (9.6%), demyelinating disease (9%), tropical spastic paraparesis (8.4%) and hereditary spastic paraparesis (8.4%). Up to 20% of the patients presented non-traumatic myelopathy of undetermined etiology, despite the broad clinical, neuroimaging and laboratorial investigations. Conclusion Regardless an extensive evaluation, many patients with non-traumatic myelopathy of uncertain etiology. Compressive causes and nutritional deficiencies are important etiologies of non-traumatic myelopathies in our population.

A neurosarcoidose (NS) ocorre frequentemente no contexto de doença sistêmica. O diagnóstico é baseado na história clínica sugestiva de NS, presença de granulomas não-caseosos e achados anatomopatológicos, laboratoriais e radiológicos de sarcoidose. A NS causa manifestações neurológicas variadas, que apresentam, em geral, baixa resposta ao corticoide isoladamente e, portanto, necessitam uso de imunossupressores. Na NS, os linfócitos estão polarizados para resposta Th1 excessiva, levando à produção aumentada de TNF-alfa e IFN-gama, assim como IL-2 e IL-15. Infliximabe, um anticorpo monoclonal quimérico que neutraliza a atividade biológica do TNF-alfa, é uma nova opção no tratamento da NS. Revisou-se a fisiopatologia, as manifestações clínicas, o diagnóstico e o tratamento da NS para orientar neurologistas gerais.

Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.

As ataxias hereditárias compreendem grande espectro de doenças neurodegenerativas geneticamente determinadas, tendo como sintoma preponderante a ataxia de instalação progressiva. No sentido de avaliar a gravidade da ataxia cerebelar através de forma mais fácil e prática, foi proposta uma nova escala: a Escala para Avaliação e Graduação de Ataxia (SARA). O objetivo deste estudo foi traduzir e validar a SARA para o português brasileiro. MÉTODO: A escala SARA foi traduzida para o português brasileiro, analisada, traduzida novamente para o inglês e comparada com sua versão original. A escala foi aplicada em 30 pacientes. Além disso, nós aplicamos também a Escala Cooperativa Internacional para Graduação de Ataxia (ICARS) em todos os pacientes. RESULTADOS: A escala SARA foi traduzida para a versão brasileira com adequada consistência interna, mas uma correlação significativa com a escala ICARS não foi encontrada. CONCLUSÃO: A escala SARA foi traduzida e validada para o português brasileiro, demonstrando boa confiabilidade e validade.

The hereditary ataxias comprise a very large spectrum of genetically determined neurodegenerative disorders with progressive ataxia as the prominent symptom. In order to measure the severity of cerebellar ataxia in an easier and more practical way, it was proposed a new scale: the Scale for the Assessment and Rating of Ataxia (SARA). The objective of this study was to translate and validate SARA into Brazilian Portuguese. METHOD: The SARA was translated into Brazilian Portuguese, analyzed, back translated to English, and compared to the original version. It was applied to 30 patients. In addition to SARA, we applied the International Cooperative Ataxia Rating Scale (ICARS) in all subjects. RESULTS: SARA scale was translated into Brazilian version with adequate internal consistence, but a significant correlation between ICARS and SARA was not found. CONCLUSION: SARA was translated and validated into Brazilian Portuguese language, showing good reliability and validity.