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Background Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed. Objectives To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters. Methods Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36). Results The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, p = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, p < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps. Conclusion There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways. Resumo Antecedentes Estudos foram realizados com o objetivo de avaliar a qualidade de vida (QV) de pacientes acometidos pela polirradiculopatia desmielinizante inflamatória crônica (PDIC). No entanto, a questão cognitiva ainda é pouco abordada. Objetivos Avaliar a QV e o comprometimento cognitivo em pacientes com PDIC bem como se existe correlação entre esses parâmetros. Métodos Sete pacientes com PDIC e sete controles pareados foram submetidos a: miniexame do estado mental (MEEM); avaliação cognitiva de Montreal (MoCA); teste de substituição de símbolo de dígito/cópia de símbolo (DSST); escala de gravidade da fadiga (FSS);Beck depressive inventory-I (BDI-I) e um short-form of health survey (SF-36). Resultados A média de idade dos pacientes foi de 50 anos (71,4% do sexo masculino). O MMSE e o MoCA não apresentaram diferença estatística entre os grupos. Os pacientes apresentaram resultados superiores no domínio memória do MoCA (5 vs. 2, p = 0,013). No DSST, observamos uma tendência de os pacientes serem mais lentos. Houve forte correlação negativa entre os níveis de fadiga e o domínio vitalidade (SF-36). Não houve correlação significativa entre níveis de depressão e QV. Não houve correlação entre depressão e os resultados obtidos nos testes cognitivos. Níveis elevados de depressão foram observados nos pacientes (15,28 vs. 3,42, p < 0,001). Um total de 57% apresentou fadiga intensa, 28,8% dor autorreferida, e 57,1% queixam-se de câimbras. Conclusão Não há comprometimento cognitivo nos pacientes estudados. Observamos somente uma tendência de lentificação na velocidade de processamento. Para melhor avaliar as alterações encontradas, será necessário estudo com um número maior de indivíduos. A PDIC afeta de diferentes formas o nível de QV de seus portadores.

Objective: To systematically analyze quantitative data about the effects of religion/spirituality and the well-being/quality of life of cancer patients. The second aim was to hypothesize a neurophysiological model of the association between religion/spirituality and the brain. Methods: This study met the PRISMA Statement and was registered at PROSPERO database. Randomized and Controlled trials investigating religion/spirituality and well-being/quality of life of cancer patients were included. Based on neuroimaging and neurophysiology studies, a neuroanatomical model was developed to hypothesize the relationship between neuroscience and religion/spirituality. Results: A large effect size was found on the improvement of well-being/quality of life (SMD = 3.90 [2.43-5.38], p < 0.01). Heterogeneity was high among studies (I2 = 98%, p < 0.01). Specific regions of the brain, such as the temporal lobes, amygdalae and hippocampus, regions from the limbic system, were hypothesized to take part in the religion/spirituality phenomena and the well-being/quality of life improvement. Conclusion: Religion/spirituality intervention, mainly the Islamic, promotes an improvement on well-being/quality of life of cancer patients.

INTRO/BACKGROUND The complications of Diabetes Mellitus (DM) are traditionally categorized as micro and macrovascular disorders. Among them, diabetic polyneuropathy (DPN) is one of the most common, presenting with or without associated neuropathic pain, and its morbidity exerts a significant impact on the quality of life (QOL) of these patients. About 50% of individuals with type 2 DM (T2DM) suffer from this condition and the distal symmetric polyneuropathy (DSPN) constitutes its most frequent clinical form. OBJECTIVE: To demonstrate the effect of symptomatic DSPN on the QOL of T2DM patients in a sample of the Brazilian population, correlating clinical and electrophysiological findings, besides comparing the results obtained by the Medical Outcomes Study Questionaire 36-Item Short Form Health Survey (SF-36) among patients with painful and non-painful diabetic DSPN. METHODS: This study comprised 25 outpatients with DSPN and T2DM submitted to a detailed anamnesis to identify clinical and demographic characteristics, besides comorbidities and complications of DM. Clinical evaluation was performed through neurological physical examination, in addition to specific scales for neuropathy as the Neuropathy Disability Score (NDS). In order to assess the health-related quality of life (HRQoL) of these patients, the SF-36 translated and adapted for the Brazilian population was applied. Nerve conduction study (NCS) was performed for the examined nerves (motor part of peroneal nerve and sensory part of the sural nerve). The following parameters were assessed: motor conduction velocity (MCV), amplitude of the compound muscle action potentials (CMAP) and amplitude of the sensory nerve action potentials (SNAP). RESULTS: Role Physical (RP) domain of SF-36 was significantly related to some of the clinical and electrophysiological factors measured. RP had an inverse and significant relationship with the NDS values (Rho: -0.44), showing the impact of neuropathy severity on these patients’ QOL. The sural nerve SNAP and peroneal nerve MCV showed a significant and positive relationship with RP (Rho: 0,52 and 0,36, respectively). The Mental Health (MH) domain showed a statistically significant difference between those patients with pain and without pain (p = 0.002), and patients without pain had higher mean values, as well as a higher minimum and maximum value. The Role Emotional (RE) domain also showed a significant difference between patients with and without pain, and patients with pain had a lower mean value (p = 0.04). For all other domains, patients with pain showed lower mean values than those without pain, however without statistical difference in the test performed. Between DM complications, only nephropathy presented statistically different RP scores from those without nephropathy (p = 0.02). CONCLUSION: There was a significant inverse relationship between the severity of DSPN and the QOL of the evaluated patients, as evidenced by lower values in the SF-36 specific RP domain, as polyneuropathy becomes more severe. This domain also presented significantly lower values in patients with associated nephropathy. The presence of pain negatively affected the QOL of patients with painful DPN, who presented significantly lower mean values in the MH and RE domains when compared to patients without pain.

Objective To test the hypotheses that emerging viruses are associated with neurological hospitalizations and that statistical models can be used to predict neurological sequelae from viral infections. Methods An ecological study was carried out to observe time trends in the number of hospitalizations with inflammatory polyneuropathy and Guillain‐Barré syndrome (GBS) in the state of Rio de Janeiro from 1997 to 2017. Increases in GBS from month to month were assessed using a Farrington test. In addition, a cross‐sectional study was conducted analyzing 50 adults hospitalized for inflammatory polyneuropathies from 2015 to 2017. The extent to which Zika virus symptoms explained GBS hospitalizations was evaluated using a calibration test. Results There were significant increases (Farrington test, P, The introduction of novel viruses increases the incidence of inflammatory neuropathies in Brazil.

Background: Charcot-Marie-Tooth disease type 1A (CMT1A) is the most common form of hereditary neuropathy. Objective: To investigate the prevalence and characteristics of pain in patients with CMT1A. Methods: Nineteen patients with a diagnosis of CMT1A were evaluated between September 2018 and October 2019, and other causes of neuropathy were ruled out. The following tools were used for the pain assessment: neurological assessment, LANSS, DN4, clinical evaluation, VAS, CMTNS2 and SF-36. Statistical analysis was performed using prevalence analysis, t test, chi-square test and Spearman's rho. Results: The prevalence of pain was 84.2% in the sample of this study, with moderate intensity and nociceptive characteristics according to the LANSS scale (75%) and clinical evaluation (50%), but differing from DN4, which found neuropathic pain in the majority of the patients (56.2%). Mixed pain was also observed in 43.7% of the patients, according to clinical criteria. There was a statistically significant correlation between pain intensity and SF-36, thus demonstrating that the lower the pain was, the lower the impairment was, in all domains. Conclusion: Pain is a prevalent and important symptom in CMT1A, with moderate intensity and nociceptive characteristics according to two tools, but neuropathic pain is also present, and there may even be a mixed pattern of pain. The correlation of the pain with SF-36 suggests that pain relief could provide improvements to the quality of life of these individuals. RESUMO Introdução: A doença de Charcot-Marie-Tooth tipo 1 A (CMT1A) é a forma mais comum de neuropatia hereditária. Objetivo: Investigar a prevalência e as características de dor nos pacientes com a doença de CMT1A. Métodos: Dezenove pacientes com diagnóstico de CMT1A foram avaliados de setembro 2018 a outubro de 2019, e outras causas de neuropatia foram excluídas. As seguintes ferramentas foram utilizadas para avaliar a dor: avaliação neurológica, LANSS, DN4, avaliação clínica, EVA, CMTNS2 e SF-36. A análise estatística foi realizada pelo teste de análise de prevalência, bem como pelos testes T, do qui-quadrado e rô de Sperman. Resultados: A prevalência de dor foi de 84,2% na amostra do estudo, com intensidade moderada e características nociceptivas de acordo com a escala LANSS (75%) e a avaliação clínica (50%), diferentemente da escala DN4, que encontrou dor neuropática na maioria dos pacientes (56,2%). Dor mista também foi verificada em 43,7% dos pacientes, de acordo com os critérios clínicos. Houve significância estatística da correlação entre a intensidade da dor e o SF-36, demonstrando que quanto menor a dor, menor o comprometimento em todos os domínios. Conclusão: A dor é um sintoma prevalente e relevante na CMT1A, com intensidade moderada e características nociceptivas de acordo com duas ferramentas, mas dor neuropática também está presente, e ainda pode haver padrão misto de dor. A correlação da dor com SF-36 sugere que o alívio da dor pode proporcionar melhorias na qualidade de vida desses indivíduos.

Background Varicella-zoster virus (VZV) is a human neurotropic virus that remains in a latent state within ganglionic neurons throughout the entire neuroaxis after the primary infection. When herpes zoster (HZ) leads to trigeminal involvement, the ophthalmic division is the most implicated. COVID-19 has emerged as a viral cause of severe acute respiratory syndrome that has spread all over the world in the last months. Co-infection with COVID-19 and other viruses has been reported, but sparsely, and involving the respiratory viruses. Methods The case of a co-infection of COVID-19 with VZV is reported, and the literature reviewed. Results A 39-year-old immunocompetent man presented with oligosymptomatic infection with COVID-19, which evolved to left facial HZ, affecting the three divisions of the trigeminal nerve. The co-infection was remotely registered, being the respiratory viruses, especially influenza, the most commonly cited association. However, the present case illustrates the emergence of a latent virus infection, which might be favored by the inflammatory response to the former agent (COVID-19). This reaction ascended from the nasal cavity, where trigeminal branches are also placed. Conclusions The emergence of latent VZV infection in this rare presentation might illustrate an effect, at least locally, of COVID-19. This virus possibly induced a retrograde reactivation of VZV in a young immunocompetent patient.

Importance: Congenital Zika syndrome virus infection is said to interfere in children’s development. Objective: evaluate gross motor trajectories and the frequency of cerebral palsy in children with congenital Zika syndrome. Design: Cohort study applying the Alberta Infant Motor Scale (AIMS) and the Bayley III Scales in infants from 6 to 18 months of age. Setting: The SARAH network, Rio de Janeiro. Participants: Thirty-nine infants whose diagnoses were established through clinical history, serology tests, and neuroimaging findings. Main outcomes and measures: Congenital Zika syndrome is associated with severe motor delays and is a risk factor to the diagnosis of cerebral palsy. Results: The Alberta Infant Motor Scale mean raw score at 6 months was 9.74 (SD 4.80) or equivalent to 2 to 3 months of motor developmental age. At the age of 12 months, 14.13 (SD 11.90), corresponding to 3 to 4 months of motor development age; the Bayley III Scales results correlated to the Alberta Infant Motor Scale ( P < .001) at this age. At 18 months, 15.77 (SD 13.80) or a motor development equivalent to 4 to 5 months of age. Thirty-five of 39 children (89.7%) met criteria for the diagnosis of cerebral palsy. Conclusions and relevance: Gross motor development marginally progresses from 6 to 18 months of age. These individuals also displayed a high frequency of cerebral palsy.

CNPq - Conselho Nacional de Desenvolvimento Científico e Tecnológico FAPEMIG - Fundação de Amparo à Pesquisa do Estado de Minas Gerais CAPES - Coordenação de Aperfeiçoamento de Pessoal de Nível Superior FAPERJ - Fundação Carlos Chagas Filho de Amparo à Pesquisa do Estado do Rio de Janeiro For many years lactate was seen as a metabolite from glucose metabolism. However, since the last century researchers have shown that this molecule has an important role on liver, muscle, and brain metabolism. Lactate traffics along whole body mediating many biological processes depending on specific situations. For example, glucose is the main substrate used during exercise but lactate released by striated skeletal muscle is used by own muscle as secondary fuel. On the other hand, neuronal firing in the brain is almost totally lactate-dependent. In addition, lactate has an important role on BDNF-mediated neuroplasticity. As this molecule has a pleiotropic role in the body, it was called as “lactormone” in 2009. Here we show basic concepts on peripheral and central metabolism and discuss neurobiological pathways of lactate, including an alternative hypothesis on lactate released during exercise.

Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology. The first and last authors produced a draft summarizing the main views on the subject and emailed the text to 10 other specialists. Relevant literature on this subject was reviewed by each participant and used for the individual review of the whole text. Each participant was expected to review the text and send a feedback review by e-mail. Thereafter, the 12 panelists got together at the city of Fortaleza, discussed the controversial points, and reached a consensus for the final text.

Objective:To compare cognitive function among frail and prefrail older adults.Design:Cross-sectional clinical study.Participants:Fifty-one non-institutionalized older individuals participated in this study.Measurements:Cognitive functions were evaluated through Mini-Mental State Examination (Global Cognition), Digit Span Forward (short-term memory), Digit Span Backward (working memory), Verbal Fluency Test (semantic memory/executive function). Data were compared using parametric and non-parametric bivariate tests. Binary logistic regression was used to test a frailty prediction model. Statistical significance was defined as p ≤ 0.01 to compare groups. In the regression model, the p value was set to be ≤0.05.Results:Statistically significant differences were observed in global cognition, and short-term memory between frail and prefrail individuals (p ≤ 0.01). Global cognition explained 14–19% of frailty's model.Conclusion:According to our findings, the evaluation of cognitive functions among older persons with frailty and prefrailty provides important complementary information to better manage frailty and its progression.

Charcot-Marie-Tooth (CMT) disease, the most common inherited peripheral neuropathy, has pain as one of its clinical features, yet it remains underdiagnosed and undertreated. This literature review assessed data related to pain from CMT to determine its prevalence, type and importance as a symptom, which, unlike other symptoms, is likely to be treated. The research encompassed 2007 to 2017 and included five articles that addressed pain from CMT. All of the papers concurred that pain is frequently present in CMT patients, yet its classification remains undefined as there has been no consensus in the literature about the mechanisms that cause it. RESUMO A doença de Charcot-Marie-Tooth (CMT), a neuropatia periférica hereditária mais comum, tem a dor como uma de suas características clínicas, a qual permanece subdiagnosticada e subtratada. Essa revisão de literatura avaliou os dados relacionados à dor em CMT com objetivo de observar sua prevalência, tipo e importância como sintoma que, em detrimento de outros, é possível ser tratado. O intervalo da pesquisa foi entre 2007 e 2017, através de cinco artigos abordando a dor em CMT. Todos os artigos concordam que a dor é frequente nos pacientes com a doença de CMT e a sua classificação permanece indefinida por não haver consenso na literatura sobre os mecanismos da dor.

The recent alarming statements concerning the newborn ZIKV-induced microcephaly epidemics in the Northeast of Brazil, released by the Brazilian Ministry of Health, as well as important international health agencies, such as the World Health Organization and the Pan American Health Organization, raised many "why and how" questions so far, that will hopefully be scientifically answered, as more researches in that regard come up in the long term. In this paper, we describe another potentially ZIKV-induced central nervous system and musculoskeletal disorder that has accompanied microcephaly in these children: atrhogryposis multiplex congenita. The goal is to bring up some hypotheses for possible underlying molecular mechanisms based on published data taken from animal models, such as ovine and cattle, which once infected by other types of arboviroses and viruses that also belong to the Flaviviridae family, presented, too, with the full-blown CNS spectrum of malformations at birth.

Post-polio syndrome (PPS) is a condition that affects polio survivors years after recovery from an initial acute attack of the poliomyelitis virus. Most often, polio survivors experience a gradual new weakening in muscles that were previously affected by the polio infection. The actual incidence of cardiovascular diseases (CVDs) in individuals suffering from PPS is not known. However, there is a reason to suspect that individuals with PPS might be at increased risk. Method A search for papers was made in the databases Bireme, Scielo and Pubmed with the following keywords: post polio syndrome, cardiorespiratory and rehabilitation in English, French and Spanish languages. Although we targeted only seek current studies on the topic in question, only the relevant (double-blind, randomized-controlled and consensus articles) were considered. Results and Discussion Certain features of PPS such as generalized fatigue, generalized and specific muscle weakness, joint and/or muscle pain may result in physical inactivity deconditioning obesity and dyslipidemia. Respiratory difficulties are common and may result in hypoxemia. Conclusion Only when evaluated and treated promptly, somE patients can obtain the full benefits of the use of respiratory muscles aids as far as quality of life is concerned. RESUMO Síndrome pós-polio (SPP) é uma condição que afeta sobreviventes da poliomielite aguda (PAA), anos após a recuperação de um ataque agudo inicial do vírus. Na maioria das vezes, os sobreviventes da polio começam a apresentar nova paresia gradual nos músculos que foram previamente afetados pela infecção. A incidência real de doenças cardiovasculares (DCV) em indivíduos que sofrem de SPP não é conhecida. Entretanto, há indícios para suspeitar de que sujeitos com SPP podem estar em maior risco. Método Realizou-se uma busca de artigos nas bases de dados: Bireme, Scielo e Pubmed, utilizando as seguintes palavras-chave: síndrome pós-poliomielite, função cardiorrespiratória e reabilitação, nos idiomas Inglês, Francês e Espanhol. Embora tenhamos selecionado um número expressivo de artigos, somente foram considerados os duplo-cegos, randomizados-controlados, além de consensos. Resultados e Discussão Certas características da SPP, tais como fadiga muscular, paresia, dor muscular e/ou articulares podem resultar em descondicionamento por inatividade física, além de obesidade e dislipidemia. Dificuldades respiratórias são comuns e podem resultar em hipoxemia. Conclusão Somente quando avaliados e tratados em tempo hábil, alguns pacientes são capazes de obter os benefícios do uso dos músculos respiratórios auxiliares em termos de qualidade de vida.

Problem statement: Peripheral neuropathy due to diabetes has been stu died for several decades. Until recently, we associate the involveme nt of peripheral nerves with an inappropriate glycemic control in the most advanced stages of the disease. Currently, it is considered that the onse t of the neural injury can occur in the initial phase of this metabolic abnormality, during the period of g lucose intolerance. Approach: The clinical aspects of the sensory neuropathy ass ociated to the impaired glucose tolerance were analyzed in 35 Brazilian patients. A ll patients met the American Diabetes Association (ADA) and the World Health Organization (WHO) criteria for glucose intolerance. Results: We studied 20 male and 15 female, with a mean age of 62.5, ran ging from 30-83 years. A distal symmetrical lower limb involvement with positive (neuropathic pain) a nd/or negative (reduced temperature and pinprick sensations) symptoms and clinical signs of autonomic neuropathy were seen in most patients. The glycemic levels were not related to the severity of the symptoms or to the presence of any specific symptom. Conclusion: The Impaired Glucose Tolerance (IGT) neuropathy can be included in the chronic axonal polyneuropathy and usually the patie nts suffer from chronic pain and disability before the diagnosis. The determination of the prevalence of s ymptoms is essential to recognize this disease. The early diagnosis and the aggressive treatment can be crucial for the control, development and progressi on of the small fiber neuropathy in glucose intolerant patients.

Problem statement: Amyotrophic Lateral Sclerosis (ALS) is a progressi ve and degenerative disease that affects the anterior horn motor neurons of the spinal cord and pyramidal tracts. In Brazil, there are few epidemiological da ta on this disease. Recently, some important findings have been reported, allowing a better unde rstanding on the underlying processes of neuronal death, as well as the characteristics of t his population. To discuss the clinical and functional profile of a convenience sample of patie nts with ALS in Rio de Janeiro and Neurology Department-Federal Fluminense University to compare the data with studies of other regions and countries. Approach: We used the Severity and Functional Ability Scale (SFAS) as a clinical and functional indicator for ALS. The modified El Escor ial criteria were used to establish the diagnosis. The participants underwent five quarterly assessmen ts during the study period. The research took place at two University Hospitals (Hospital Univers itario Antonio Pedro-Universidade Federal Fluminense and Instituto de Neurologia Deolindo Couto-Universidade Federal do Rio de Janeiro) from March 2007 to December 2009. Results: Of the 98 recruited subjects, only 24 have complet ed all phases of the study. The average age was 52.7±4 .1 years. The time between the onset of first symptoms and seeking care services was 11.6±12.:4 months. The time between the first symptoms and the diagnosis was 20.5±8.4 months. Muscle weakness was identified as the initial symptom in most cases. Patients had impaired muscle strength, speech, swallowing, respiratory function and severity stages of SFAS. The disease had different forms of initial presentation (impaired speech, limbs strength, respiratory function or swallowing) , time to progression and clinical characteristics in our population. Conclusion: The knowledge on the individual clinical evolution in ALS is of paramount importance for the healthcare team to pro vide a correct treatment during the decline of

Objective This study aims to evaluate the characteristics of the corneal sub-basal plexus by performing in vivo confocal microscopy of healthy Brazilians to provide reference values for the Brazilian population. Method This study is an observational, cross-sectional, descriptive study comparing corneas from 55 healthy Brazilian individuals across the age span of 20-70 years. Results The average number of fibers was 5.35 ± 1.36, fiber density was 33.4 ± 8.5 fibers per field, and the mean number of Langerhans cells was 5.13 ± 8.10. A correlation between the average number of fibers and age showed an inverse relationship between the number and density of fibers and age for women (p < 0.05). In the multivariate analysis, each annual increase of age showed an average increase of 1.017 (95%CI: 1.008 to 1.026) in the number of Langerhans cells, adjusting for sex and thickness. Conclusion Compared to other samples, this Brazilian population showed a higher average number of fibers, though further studies with a larger sample should be performed. RESUMO Objetivo Este estudo tem como objetivo avaliar as características morfológicas do plexo sub-basal da córnea por microscopia confocal in vivo com indivíduos brasileiros saudáveis para fornecer uma referência para a população brasileira. Método Este trabalho é um estudo observacional, descritivo, transversal comparativo com microscopia confocal de córnea a partir de 55 indivíduos brasileiros saudáveis na faixa etária de 70 ± 20 anos de idade. Resultados Número médio de fibras foi 5,35 ± 1,36, a densidade foi de 33,4 ± 8,5 fibras por campo e de células de Langerhans foi 5,13 ± 8,10. Uma correlação entre o número médio de fibras e da idade dos indivíduos mostrou uma relação inversa entre o número e densidade de fibras, e idade para as mulheres, (p < 0,05). Na análise multivariada, cada aumento anual de idade apresentou um aumento médio de 1,017 (IC95%: 1,008-1,026) no número de células de Langerhans, ajustado para sexo e espessura. Conclusão Em comparação com outras amostras, esta população brasileira apresentou um maior número médio de fibras embora estudos com número maior de amostras necessitem ser realizados.

Thanks to the development of several new researches, the lifetime presented a significant increase, even so, we still have many obstacles to overcome − among them, manage and get responses regarding neurodegenerative diseases. Where we are in the understanding of neuroprotection? Do we really have protective therapies for diseases considered degeneratives such as amyotrophic lateral sclerosis and its variants, Parkinson’s disease, Alzheimer’s disease and many others? Neuroprotection is defined by many researches as interactions and interventions that can slow down or even inhibit the progression of neuronal degeneration process. We make some considerations on this neuroprotective effect.

Amyotrophic lateral sclerosis (ALS), Charcot’s disease or Lou Gehrig’s disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000. It causes progressive and cumulative physical disabilities, and leads to eventual death due to respiratory muscle failure. ALS is diverse in its presentation, course, and progression. We do not yet fully understand the causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. In this chapter, we will discuss the diagnosis, treatment, and how to cope with impaired function and end of life based on of our experience, guidelines, and clinical trials. Nowadays ALS seems to be a more complex disease than it did two decades – or even one decade – ago, but new insights have been plentiful. Clinical trials should be seen more as experiments on pathogenic mechanisms. A medication or combination of medications that targets more than one pathogenic pathway may slow disease progression in an additive or synergistic fashion.

Introduction. Classical amyotrophic lateral sclerosis (ALS) is not hard to diagnose, but when it comes to atypical forms of motor neuron disease (MND) which account for about 20% in clinical setting, we may face some difficulties in differentiating clearly between atypical forms of ALS/MND and other non-ALS diseases, such as multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy and cervical spondylosis. Association between neuropathy and ALS has been reported rarely. Method. We report a patient who presented with clinical/electrophysiological features and investigations suggestive of chronic neuropathy but who later progressed with anterior horn and pyramidal signs and received a final diagnosis of ALS according to the original El Escorial criteria. Conclusion. Our findings support the hypothesis that ALS is a multisystem neurodegenerative disorder that may occasionally include neuropathy among its non-motor features.

Clinical and pathological findings in leprosy are determined by the natural host immune response to Mycobacterium leprae. We previously described cases of painful neuropathy (PN) with no concurrent cause apart from a past history of leprosy successfully treated. Four leprosy previously treated patients who developed a PN years after multidrug therapy (MDT) are reported. The mean patient age was 52.75 years (47-64). The mean time interval of the recent neuropathy from the previous MDT was 19 years (12-26). A painful multiplex neuritis or polyneuropathy were observed respectively in two cases. Electrophysiological studies disclosed a sensory axonal neuropathy in two cases. Microvasculitis with no bacilli was seen in nerve biopsy. Neuropathic symptoms were improved with prednisone. We consider these cases as being a leprosy late-onset neuropathy (LLON) form of presentation. A delayed immune reaction could explain the late appearance of LLON. Dados clínicos e patológicos são determinados pela resposta imune ao Mycobacterium leprae. Já haviamos descrito previamente casos de neuropatia dolorosa (ND) sem causa associada exceto história de hanseníase tratada. Relatamos agora quatro pacientes previamente tratados que desenvolveram ND anos após multidrogaterapia (MDT). A média de idade foi de 52,75 anos (47-64). O intervalo de tempo para o aparecimento da neuropatia recente, em relação à MDT prévia, foi de 19 anos (12-26). Neurite múltipla ou polineuropatia dolorosa foram observadas em dois casos. Estudos eletrofisiológicos revelaram ocorrência de neuropatia sensitiva axonal em dois casos. Em biópsia de nervos, foi observada microvasculite sem a presença de bacilos. Os sintomas neuropáticos melhoraram com o uso de prednisona. Consideramos esses casos como sendo a forma de apresentação de neuropatia da hanseníase de início tardio (NHIT). Reação imune tardia poderia explicar o aparecimento da NHIT.

Objective: Our objective is to call attention to the new laboratory tolls for small-fiber peripheral neuropathies (SFPN)/neuropathic pain (NP) clinical assessment. Background SFPN are very frequent in clinical practice. Usually, these neuropathies are painful. The best tools to assess SFPN/NP are a comprehensive patient medical history and a well performed neurological examination. Secondly, appear the electrophysiological methods, and thirdly the imaging and the morphological ones. The 2009 revised guidelines on NP assessment proposed by the European Federation of Neurological Societies (EFNS), reinforce the previous recommendations (Cruccu G et al., 2010). They considered that amongst laboratory tests, laser evoked potentials (LEPs) are the best tool for assessing SFPN/NP, and skin biopsy for assessing distal loss of unmyelinated nerve fibers. Design/Methods: Standard techniques of contact heat evoked potentials (CHEPs), corneal confocal microscopy (CCM), and Meissner corpuscles (MCs) confocal microscopy were evaluated in the clinical assessment of patients presenting SFPN/NP by our different peripheral nerve centers. Results: CHEPs provided an objective approach to investigate cerebral responses to thermal stimuli mediated by Adelta fibers. In patients with skin denervation amplitudes of CHEPs were reduced. CCM is a non-invasive and easily reproducible clinical technique that disclosed early or severe damage of small-nerve fibers of the cornea in several disorders, including diabetic neuropathy. MCs are touch-pressure sensation receptors in glabrous skin. CM of MCs showed to be a potential for non-invasive detection and monitoring of SNF denervation or reinnervation. Normal data collections are in course for these new tools by ours groups. Conclusions: We have had the opportunity to assess all of the referred methods, including those proposed in the 2009 revised guidelines on NP assessment and we conclude that CHEPS, CCM and MCsCM are highly effective, non invasive and efficient tools in this field. Supported by: CNPq. Disclosure: Dr. Nascimento has nothing to disclose. Dr. De Freitas has nothing to disclose. Dr. Pupe has nothing to disclose. Dr. Dieckmann has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Valls-Sole has nothing to disclose. Dr. Schestatsky has nothing to disclose. Dr. Matta has nothing to disclose.

Background: Active games based on virtual reality have been widely used in the rehabilitation of many clinical conditions. However, studies on the use of Xbox/Kinect are rare, and technology application in stroke treatment is not clear yet. Objective: To verify the outcomes (O) analyzed in randomized controlled trials (C; S) that investigated the use of Xbox/Kinect (I) in patients with stroke (P). Methods: This is a systematic literature review that meets PRISMA standards and the eligibility criteria according to the PICOS strategy. The search procedure was performed by two researchers. The research strategy was repeated in case of divergence. Effect size was calculated by Cohen's formula and Hopkins rank. The risk of individual bias was assessed using PEDro Score and Higgins Classification. Results: The main outcomes were postural balance and activities of daily living, with four studies addressing these variables. However, only one study showed the effect of Xbox/Kinect intervention on balance as large, as in two other studies evaluating manual dexterity and depression, respectively. Conclusion: The greater use of Xbox/Kinect in treating patients after stroke is in recovery of balance and motor function, and the evidence support its application. These findings enable the use of virtual reality technology through Xbox/Kinect in rehabilitation programs, focusing on postural balance and motor skills. However, conclusive results are still not possible. Therefore, caution in the use of this technology is required. RESUMO Introdução: Jogos ativos baseados em realidade virtual têm sido amplamente utilizados na reabilitação de muitas condições clínicas. No entanto, estudos sobre a utilização do Xbox/Kinect são raros, e não está clara a aplicabilidade da tecnologia no tratamento de pacientes que tiveram acidente vascular cerebral. Objetivo: Verificar os desfechos (O) analisados em ensaios clínicos randomizados e controlados (C; S), que investigaram a utilização do Xbox/Kinect (I) em pacientes que tiveram acidente vascular cerebral (P). Métodos: Trata-se de uma revisão sistemática da literatura que atende aos padrões do PRISMA e aos critérios de elegibilidade, de acordo com a estratégia PICOS. O procedimento de busca foi realizado por dois pesquisadores e, em caso de divergência, a estratégia de busca foi repetida. O tamanho do efeito foi calculado por meio da fórmula de Cohen e da escala de Hopkins. O risco de viés individual foi analisado utilizando o escore PEDro e a classificação de Higgins. Resultados: Os principais desfechos foram o equilíbrio postural e as atividades de vida diária, com quatro estudos abordando essas variáveis. No entanto, apenas um estudo mostrou o efeito da intervenção com Xbox/Kinect sobre o equilíbrio como sendo grande, assim como em dois outros artigos que avaliaram destreza manual e depressão, respectivamente. Conclusão: A utilização mais comum do Xbox/Kinect no tratamento de pacientes que tiveram acidente vascular cerebral acontece na recuperação do equilíbrio e da função motora, e as evidências apoiam o seu uso. Esses achados permitem o uso da tecnologia de realidade virtual por meio do Xbox/Kinect em programas de reabilitação, com foco no equilíbrio postural e nas habilidades motoras. Porém, resultados conclusivos ainda não são possíveis, o que exige cautela no uso dessa tecnologia.