Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.

SummaryWe performed a systematic review on the neurological complications of chikungunya virus. Such complications are being reported increasingly, owing primarily to the scale of recent epidemics but also to a growing understanding of the virus' neurovirulence. We performed a thorough literature search using PubMed and Scopus databases, summating the data on all published reports of neurological disease associated with chikungunya virus. We appraised the data for each major condition in adults, children, and neonates, as well as evaluating the latest evidence on disease pathogenesis and management strategies. The review provides a comprehensive summary for clinicians, public health officials, and researchers tackling the challenges associated with this important emerging pathogen.

Less than a hundred cases of pregnancies in women with neuromyelitis optica (NMO) have been published in the world. The aim of the present study was to add the Brazilian experience to this subject. Cases of women with NMO who became pregnant, or who developed NMO soon after pregnancy, were included. Retrospective analysis of medical data from these patients was carried out by the neurologist responsible for the case. Seventeen cases of pregnancies (16 full-term pregnancies, one miscarriage) were identified. The relapse rate of demyelinating events in the first trimester after pregnancy was significantly higher than at any other time. Disability progression was significantly worse 1 year after delivery. Pregnancy negatively influenced the disease course of NMO in these women. These results are similar to those of other authors, although the total number of cases so far described is still small. Obstetricians must be aware of the potential complications of a pregnancy in a woman who has NMO.

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Background Since 2015, the arthropod-borne viruses (arboviruses) Zika and chikungunya have spread across the Americas causing outbreaks, accompanied by increases in immune-mediated and infectious neurological disease. The spectrum of neurological manifestations linked to these viruses, and the importance of dual infection, are not known fully. We aimed to investigate whether neurological presentations differed according to the infecting arbovirus, and whether patients with dual infection had a different disease spectrum or severity. Methods We report a prospective observational study done during epidemics of Zika and chikungunya viruses in Recife, Pernambuco, a dengue-endemic area of Brazil. We recruited adults aged 18 years or older referred to Hospital da Restauração, a secondary-level and tertiary-level hospital, with suspected acute neurological disease and a history of suspected arboviral infection. We looked for evidence of Zika, chikungunya, or dengue infection by viral RNA or specific IgM antibodies in serum or CSF. We grouped patients according to their arbovirus laboratory diagnosis and then compared demographic and clinical characteristics. Findings Between Dec 4, 2014, and Dec 4, 2016, 1410 patients were admitted to the hospital neurology service; 201 (14%) had symptoms consistent with arbovirus infection and sufficient samples for diagnostic testing and were included in the study. The median age was 48 years (IQR 34–60), and 106 (53%) were women. 148 (74%) of 201 patients had laboratory evidence of arboviral infection. 98 (49%) of them had a single viral infection (41 [20%] had Zika, 55 [27%] had chikungunya, and two [1%] had dengue infection), whereas 50 (25%) had evidence of dual infection, mostly with Zika and chikungunya viruses (46 [23%] patients). Patients positive for arbovirus infection presented with a broad range of CNS and peripheral nervous system (PNS) disease. Chikungunya infection was more often associated with CNS disease (26 [47%] of 55 patients with chikungunya infection vs six [15%] of 41 with Zika infection; p=0·0008), especially myelitis (12 [22%] patients). Zika infection was more often associated with PNS disease (26 [63%] of 41 patients with Zika infection vs nine [16%] of 55 with chikungunya infection; p≤0·0001), particularly Guillain-Barré syndrome (25 [61%] patients). Patients with Guillain-Barré syndrome who had Zika and chikungunya dual infection had more aggressive disease, requiring intensive care support and longer hospital stays, than those with mono-infection (median 24 days [IQR 20–30] vs 17 days [10–20]; p=0·0028). Eight (17%) of 46 patients with Zika and chikungunya dual infection had a stroke or transient ischaemic attack, compared with five (6%) of 96 patients with Zika or chikungunya mono-infection (p=0·047). Interpretation There is a wide and overlapping spectrum of neurologica...

Zika virus (ZIKV) is now considered an emerging flavivirosis, with a first large outbreak registered in the Yap Islands in 2007. In 2013, a new outbreak was reported in the French Polynesia, with associated cases of neurological complications including Guillain-Barré syndrome (GBS). The incidence of GBS has increased in Brazil since 2015, what is speculated to be secondary to the ZIKV infection outbreak. The gold-standard test for detection of acute ZIKV infection is the polymerase-chain reaction technique, an essay largely unavailable in Brazil. The diagnosis of GBS is feasible even in resource-limited areas using the criteria proposed by the GBS Classification Group, which is based solely on clinical grounds. Further understanding on the relationship of ZIKV with neurological complications is a research urgency.

Objective: To present the results of prospective and retrospective studies on multiple sclerosis patients cognitive dysfunctions, as well as to discuss the batteries of neuropsychological tests used in these investigations. Method: A systematic review was performed involving 40 studies published from 1997 to 2009, in PubMed, Scopus, Ovid, ISI Web of Knowledge, Scientific Electronic Library on line (Scielo) and Latin-American and Caribbean Center of Health Sciences Informations database. Clear description of multiple sclerosis patients cognitive deficits evaluation, study design, sample size; inclusion-exclusion and discontinuation criteria; instruments for neuropsychological evaluation, diagnostic methods, evaluated functions and detailed statistical analysis had been the inclusion criteria. results: There is consensus on cognitive impairment of multiple sclerosis patients, especially on memory, speed processing, executive function, attention and concentration domains. One has identified use of 23 batteries and 74 neuropsychological tests, despite the recommendation of Consortium of Multiple Sclerosis Centers to the application of MACFIMS battery. considerations: The absence of the uniformization for multiple sclerosis patients cognitive evaluation battery has precluded to achieve evidences to recommend its incorporation on diagnostic routine. Nevertheless this tendency is already outlined. Key words: multiple sclerosis, cognitive dysfunctions, neuropsychological batteries.Déficits cognitivos na esclerose múltipla: uma revisão sistemática resuMO Objetivo: Apresentar os resultados de estudos prospectivos e retrospectivos sobre disfunções cognitivas em pacientes com esclerose múltipla, assim como discutir as baterias de testes neuropsicológicos empregadas em tais investigações. Método: Uma revisão sistemática foi realizada envolvendo 40 estudos publicados no período de 1997 a 2009, nas bases de dados PubMed, Scopus, Ovid, ISI Web of Knowledge, Scientific Electronic Library on line (Scielo) e Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde. Descrição clara de: avaliação de déficits cognitivos em pacientes com esclerose múltipla, desenho do estudo, tamanho amostral, critérios de inclusão, exclusão e descontinuação, instrumentos de avaliação neuropsicológica, métodos diagnósticos, funções avaliadas e detalhamento da análise estatística foram critérios de inclusão. resultados: Há consenso quanto ao comprometimento cognitivo de pacientes com esclerose múltipla, especialmente nos domínios de memória, velocidade de processamento, função executiva, atenção e concentração. Identificou-se o emprego de 23 baterias e 74 testes neuropsicológicos, apesar da recomendação do Consórcio de Centros de esclerose múltipla para emprego da bateria MACFIMS. considerações: A falta de uniformização da bateria de avaliação cognitiva desses pacientes está impedindo que se obtenham evidências para recomendar sua incorporação como rotina diagnóstica. Mesmo assim essa tendência já se delineia. Palavras-chave: escl...

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