Comparison of cognitive functions among frail and prefrail older adults: a clinical perspective.

ABSTRACTObjective: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.

Brazilian consensus for diagnosis, management and treatment of hereditary transthyretin amyloidosis with peripheral neuropathy: second edition.

Hereditary transthyretin amyloidosis with peripheral neuropathy (ATTRv-PN) is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy with over 130 pathogenic variants identified in the TTR gene. Hereditary transthyretin amyloidosis with peripheral neuropathy is a disabling, progressive and life-threatening genetic condition that leads to death in ∼ 10 years if untreated. The prospects for ATTRv-PN have changed in the last decades, as it has become a treatable neuropathy. In addition to liver transplantation, initiated in 1990, there are now at least 3 drugs approved in many countries, including Brazil, and many more are being developed. The first Brazilian consensus on ATTRv-PN was held in the city of Fortaleza, Brazil, in June 2017. Given the new advances in the area over the last 5 years, the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology organized a second edition of the consensus. Each panelist was responsible for reviewing the literature and updating a section of the previous paper. Thereafter, the 18 panelists got together virtually after careful review of the draft, discussed each section of the text, and reached a consensus for the final version of the manuscript.

Polineuropatia amiloidótica familiar associada a transtirretina (ATTRv-PN) é uma polineuropatia sensitivo-motora e autonômica hereditária autossômica dominante com mais de 130 variantes patogênicas já identificadas no gene TTR. A ATTRv-PN é uma condição genética debilitante, progressiva e que ameaça a vida, levando à morte em ∼ 10 anos se não for tratada. Nas últimas décadas, a ATTRv-PN se tornou uma neuropatia tratável. Além do transplante de fígado, iniciado em 1990, temos agora 3 medicamentos modificadores de doença aprovados em muitos países, incluindo o Brasil, e muitas outras medicações estão em desenvolvimento. O primeiro consenso brasileiro em ATTRv-PN foi realizado em Fortaleza em junho de 2017. Devido aos novos avanços nesta área nos últimos 5 anos, o Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia organizou uma segunda edição do consenso. Cada panelista ficou responsável por rever a literatura e atualizar uma parte do manuscrito. Finalmente, os 18 panelistas se reuniram virtualmente após revisão da primeira versão, discutiram cada parte do artigo e chegaram a um consenso sobre a versão final do manuscrito.

Corneal confocal microscopy in patients with distal symmetric polyneuropathy compared to controls.

BACKGROUND: Diabetic neuropathy (DN) is a very common clinical condition throughout the world. The diagnostic tests currently recommended have low sensitivity, such as electromyography, or are invasive, such as skin biopsy. New techniques have been developed to identify the early involvement of the peripheral nerve. With the advent of corneal confocal microscopy (CCM), a reduction in corneal innervation in patients with DN has been observed. OBJECTIVE: To compare, through CCM, diabetic patients with symptomatic distal symmetric polyneuropathy (DSP) and controls. METHODS: In the present study, through CCM, we compared the morphological changes in the sub-basal epithelial corneal plexus of 35 diabetic patients with symptomatic DSP with 55 controls. Moreover, we sought to determine a pattern of change regarding the severity stages of DSP, comparing the clinical, laboratory, and nerve-conduction (NC) variables. RESULTS: Differences between the control and diabetic groups were observed for the following variables, respectively: age (44.9 ± 13.24 years versus 57.02 ± 10.4 years; p < 0.001); fiber density (29.7 ± 10.2 versus 16.6 ± 10.2; p < 0.001); number of fibers (4.76 ± 1.30 versus 3.14 ± 1.63; p < 0.001); number of Langerhans cells (4.64 ± 8.05 versus 7.49 ± 10.3; p = 0.035); tortuosity (p < 0.05); and thickness (p < 0.05). Furthermore, inverse relationships were found regarding fiber density and age (p < 0.01) and fiber density and the severity of the disease (p < 0.05). A positive relationship between the conduction velocity of the fibular nerve and fiber density (p < 0.05) was also observed. CONCLUSION: Corneal confocal microscopy proved to be a fast, noninvasive and reproducible method for the diagnosis, staging, and monitoring of diabetic DSP.

ANTECEDENTES: A neuropatia diabética (ND) é condição clínica muito frequente no mundo inteiro. Os testes diagnósticos atualmente preconizados são pouco sensíveis, como a eletroneuromiografia, ou invasivos, como a biópsia de pele. Novas técnicas de investigação complementares têm sido desenvolvidas a fim de identificar o acometimento precoce do nervo periférico. Com o advento da microscopia confocal de córnea (MCC), observou-se redução da inervação da córnea em pacientes com ND. OBJETIVO: Comparar, por meio da MCC, pacientes diabéticos com polineuropatia simétrica distal (PSD) sintomática e controles. MéTODOS: Neste estudo, por meio da MCC, comparamos as alterações morfológicas do plexo sub-basal epitelial da córnea de 35 pacientes diabéticos com PSD sintomática com 55 indivíduos controles. Além disso, buscamos determinar um padrão de alteração entre os estágios de gravidade da PSD, comparando variáveis clínicas, laboratoriais e de neurocondução. RESULTADOS: Diferenças entre os grupos controle e diabéticos foram verificadas com relação às seguintes variáveis, respectivamente: idade (44,9 ± 13,24 anos versus 57,02 ± 10,4 anos; p < 0,001); densidade das fibras (29,7 ± 10,2 versus 16,6 ± 10,2; p < 0,001); número de fibras (4,76 ± 1,30 versus 3,14 ± 1,63; p < 0,001); número de células de Langerhans (4,64 ± 8,05 versus 7,49 ± 10,3; p = 0,035); tortuosidade (p < 0,05), e espessura (p < 0,05). Além disso, relações inversamente proporcionais foram verificadas entre a densidade das fibras e a idade (p < 0,01), e entre a densidade das fibras e a gravidade da doença (p < 0,05). Observou-se ainda uma relação positiva entre a velocidade de condução do nervo fibular e a densidade das fibras (p < 0,05). CONCLUSãO: A MCC constitui um método rápido, não invasivo e reprodutível para o diagnóstico, o estadiamento, e o acompanhamento da PSD diabética.

Chronic inflammatory demyelinating polyneurophaty: assessment of the cognitive function and quality of life.

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.

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.

HIV-associated painful neuropathy: where are we?

BACKGROUND: After the advent of combination antiretroviral therapy, infection with the human immunodeficiency virus (HIV) ceased to be a devastating disease, but sensory neuropathy resulting from the permanence of the virus and the side effects of treatment have worsened the morbidities of these patients. OBJECTIVE: To investigate the quality of life of 64 HIV-positive patients: 24 with painful neuropathy (case group) and 40 without painful neuropathy (control group). The impact of other factors on quality of life was also assessed. METHODS: To assess painful neuropathy, the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale, Douleur Neuropathique 4 (DN4) questions and Neuropathy Disability Score (NDS) were used. The Short Form Health Survey (SF-36) scale was used to assess quality of life. Factors related or unrelated to HIV were obtained through the medical history and analysis on medical records. RESULTS: The quality of life of patients with neuropathic pain was worse in six of the eight domains of the SF-36 scale. The number of clinical manifestations related to HIV, length of time with detectable viral load since diagnosis, length of time since the diagnosis of HIV infection and length of time of HAART use had a negative impact on quality of life. Higher levels of CD4, education and family income had a positive impact. CONCLUSIONS: Painful neuropathy related to HIV is a factor that worsens the quality of life of patients infected with this virus and should be included in the clinical evaluation.

Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A.

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.

The Xbox/Kinect use in poststroke rehabilitation settings: a systematic review.

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.

Neurological complications associated with emerging viruses in Brazil.

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<0.001) in the incidence of GBS following the introduction of influenza A/H1N1 in 2009, dengue virus type 4 in 2013, and Zika virus in 2015. Of 50 patients hospitalized, 14 (28.0%) were diagnosed with arboviruses, 9 (18.0%) with other viruses, and the remainder with other causes of such neuropathies. Statistical models based on cases of emerging viruses accurately predicted neurological sequelae, such as GBS. CONCLUSION: The introduction of novel viruses increases the incidence of inflammatory neuropathies.

Children Born With Congenital Zika Syndrome Display Atypical Gross Motor Development and a Higher Risk for Cerebral Palsy.

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.

Is the "lactormone" a key-factor for exercise-related neuroplasticity? A hypothesis based on an alternative lactate neurobiological pathway.

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.

Pain in Charcot-Marie-Tooth disease: an update.

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.

Brazilian consensus for diagnosis, management and treatment of transthyretin familial amyloid polyneuropathy.

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.

Definition and diagnosis of small fiber neuropathy: consensus from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology.

The aim of this study was to describe the results of a Brazilian Consensus on Small Fiber Neuropathy (SFN). Fifteen neurologists (members of the Brazilian Academy of Neurology) reviewed a preliminary draft. Eleven panelists got together in the city of Fortaleza to discuss and finish the text for the manuscript submission. Small fiber neuropathy can be defined as a subtype of neuropathy characterized by selective involvement of unmyelinated or thinly myelinated sensory fibers. Its clinical picture includes both negative and positive manifestations: sensory (pain/dysesthesias/pruritus) or combined sensory and autonomic complaints, associated with an almost entirely normal neurological examination. Standard electromyography is normal. A growing list of medical conditions is associated with SFN. The classification of SFN may also serve as a useful terminology to uncover minor discrepancies in the normal values from different neurophysiology laboratories. Several techniques may disclose sensory and/or autonomic impairment. Further studies are necessary to refine these techniques and develop specific therapies.

Brazilian consensus for diagnosis, management and treatment of hereditary transthyretin amyloidosis with peripheral neuropathy: second edition / Consenso brasileiro para o diagnóstico, manejo e tratamento da polineuropatia amiloidótica familiar associada à transtirretina: segunda edição

Abstract Hereditary transthyretin amyloidosis with peripheral neuropathy (ATTRv-PN) is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy with over 130 pathogenic variants identified in the TTR gene. Hereditary transthyretin amyloidosis with peripheral neuropathy is a disabling, progressive and life-threatening genetic condition that leads to death in ~ 10 years if untreated. The prospects for ATTRv-PN have changed in the last decades, as it has become a treatable neuropathy. In addition to liver transplantation, initiated in 1990, there are now at least 3 drugs approved in many countries, including Brazil, and many more are being developed. The first Brazilian consensus on ATTRv-PN was held in the city of Fortaleza, Brazil, in June 2017. Given the new advances in the area over the last 5 years, the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology organized a second edition of the consensus. Each panelist was responsible for reviewing the literature and updating a section of the previous paper. Thereafter, the 18 panelists got together virtually after careful review of the draft, discussed each section of the text, and reached a consensus for the final version of the manuscript.

Resumo Polineuropatia amiloidótica familiar associada a transtirretina (ATTRv-PN) é uma polineuropatia sensitivo-motora e autonômica hereditária autossômica dominante com mais de 130 variantes patogênicas já identificadas no gene TTR. A ATTRv-PN é uma condição genética debilitante, progressiva e que ameaça a vida, levando à morte em ~ 10 anos se não for tratada. Nas últimas décadas, a ATTRv-PN se tornou uma neuropatia tratável. Além do transplante de fígado, iniciado em 1990, temos agora 3 medicamentos modificadores de doença aprovados em muitos países, incluindo o Brasil, e muitas outras medicações estão em desenvolvimento. O primeiro consenso brasileiro em ATTRv-PN foi realizado em Fortaleza em junho de 2017. Devido aos novos avanços nesta área nos últimos 5 anos, o Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia organizou uma segunda edição do consenso. Cada panelista ficou responsável por rever a literatura e atualizar uma parte do manuscrito. Finalmente, os 18 panelistas se reuniram virtualmente após revisão da primeira versão, discutiram cada parte do artigo e chegaram a um consenso sobre a versão final do manuscrito.

Corneal confocal microscopy in patients with distal symmetric polyneuropathy compared to controls / Microscopia confocal de córnea em pacientes com polineuropatia simétrica distal comparados a controles

Abstract Background Diabetic neuropathy (DN) is a very common clinical condition throughout the world. The diagnostic tests currently recommended have low sensitivity, such as electromyography, or are invasive, such as skin biopsy. New techniques have been developed to identify the early involvement of the peripheral nerve. With the advent of corneal confocal microscopy (CCM), a reduction in corneal innervation in patients with DN has been observed. Objective To compare, through CCM, diabetic patients with symptomatic distal symmetric polyneuropathy (DSP) and controls. Methods In the present study, through CCM, we compared the morphological changes in the sub-basal epithelial corneal plexus of 35 diabetic patients with symptomatic DSP with 55 controls. Moreover, we sought to determine a pattern of change regarding the severity stages of DSP, comparing the clinical, laboratory, and nerve-conduction (NC) variables. Results Differences between the control and diabetic groups were observed for the following variables, respectively: age (44.9 ± 13.24 years versus 57.02 ± 10.4 years; p< 0.001); fiber density (29.7 ± 10.2 versus 16.6 ± 10.2; p< 0.001); number of fibers (4.76 ± 1.30 versus 3.14 ± 1.63; p< 0.001); number of Langerhans cells (4.64 ± 8.05 versus 7.49 ± 10.3; p= 0.035); tortuosity (p< 0.05); and thickness (p< 0.05). Furthermore, inverse relationships were found regarding fiber density and age (p< 0.01) and fiber density and the severity of the disease (p< 0.05). A positive relationship between the conduction velocity of the fibular nerve and fiber density (p< 0.05) was also observed. Conclusion Corneal confocal microscopy proved to be a fast, noninvasive and reproducible method for the diagnosis, staging, and monitoring of diabetic DSP.

Resumo Antecedentes A neuropatia diabética (ND) é condição clínica muito frequente no mundo inteiro. Os testes diagnósticos atualmente preconizados são pouco sensíveis, como a eletroneuromiografia, ou invasivos, como a biópsia de pele. Novas técnicas de investigação complementares têm sido desenvolvidas a fim de identificar o acometimento precoce do nervo periférico. Com o advento da microscopia confocal de córnea (MCC), observou-se redução da inervação da córnea em pacientes com ND. Objetivo Comparar, por meio da MCC, pacientes diabéticos com polineuropatia simétrica distal (PSD) sintomática e controles. Métodos Neste estudo, por meio da MCC, comparamos as alterações morfológicas do plexo sub-basal epitelial da córnea de 35 pacientes diabéticos com PSD sintomática com 55 indivíduos controles. Além disso, buscamos determinar um padrão de alteração entre os estágios de gravidade da PSD, comparando variáveis clínicas, laboratoriais e de neurocondução. Resultados Diferenças entre os grupos controle e diabéticos foram verificadas com relação às seguintes variáveis, respectivamente: idade (44,9 ± 13,24 anos versus 57,02 ± 10,4 anos; p< 0,001); densidade das fibras (29,7 ± 10,2 versus 16,6 ± 10,2; p< 0,001); número de fibras (4,76 ± 1,30 versus 3,14 ± 1,63; p< 0,001); número de células de Langerhans (4,64 ± 8,05 versus 7,49 ± 10,3; p= 0,035); tortuosidade (p< 0,05), e espessura (p < 0,05). Além disso, relações inversamente proporcionais foram verificadas entre a densidade das fibras e a idade (p< 0,01), e entre a densidade das fibras e a gravidade da doença (p< 0,05). Observou-se ainda uma relação positiva entre a velocidade de condução do nervo fibular e a densidade das fibras (p< 0,05). Conclusão A MCC constitui um método rápido, não invasivo e reprodutível para o diagnóstico, o estadiamento, e o acompanhamento da PSD diabética.

Chronic inflammatory demyelinating polyneurophaty: assessment of the cognitive function and quality of life / Polineuropatia desmielinizante inflamatória crônica: avaliação da função cognitiva e qualidade de vida

Abstract 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.

Microcephaly and arthrogryposis multiplex congenita: The full-blown CNS spectrum in newborns with ZIKV infection.

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.

Influence of Islamic religion and spirituality on the well-being and quality of life of cancer patients: a meta-analysis and a hypothetical model of cerebral mechanisms / Influência da religião e da espiritualidade islâmica no bem-estar e na qualidade de vida de pacientes com câncer: uma metanálise e um modelo hipotético de mecanismos cerebrais

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 wellbeing/quality of life of cancer patients.

OBJETIVO: Analisar sistematicamente dados quantitativos sobre os efeitos da religião/espiritualidade e o bem-estar/qualidade de vida de pacientes com câncer. O segundo objetivo foi levantar a hipótese de um modelo neurofisiológico da associação entre religião/espiritualidade e o cérebro. MÉTODOS: Este estudo seguiu as recomendações do PRISMA e foi registrado no PROSPERO. Estudos randomizados e controlados investigando religião/espiritualidade e o bem-estar/qualidade de vida de pacientes com câncer foram incluídos. Com base em estudos de neuroimagem e neurofisiologia, um modelo neuroanatômico foi desenvolvido para hipotetizar relações entre neurociência e religião/espiritualidade. RESULTADOS: Um tamanho de efeito grande foi encontrado na melhoria do bem-estar/qualidade de vida (SMD = 3,90 [2,43-5,38], p < 0,01). A heterogeneidade foi alta entre os estudos (I2 = 98%, p < 0,01). Regiões específicas do cérebro, como lobos temporais, amídalas e hipocampo, regiões do sistema límbico, foram hipotetizadas como participantes dos fenômenos religião/espiritualidade e melhoria do bem-estar/qualidade de vida. CONCLUSÃO: A intervenção religiosa/espiritual, principalmente islâmica, promove melhora no bem-estar/qualidade de vida em pacientes com câncer.

HIV-associated painful neuropathy: where are we? / Neuropatia dolorosa associada ao HIV: onde estamos?

ABSTRACT Background: After the advent of combination antiretroviral therapy, infection with the human immunodeficiency virus (HIV) ceased to be a devastating disease, but sensory neuropathy resulting from the permanence of the virus and the side effects of treatment have worsened the morbidities of these patients. Objective: To investigate the quality of life of 64 HIV-positive patients: 24 with painful neuropathy (case group) and 40 without painful neuropathy (control group). The impact of other factors on quality of life was also assessed. Methods To assess painful neuropathy, the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale, Douleur Neuropathique 4 (DN4) questions and Neuropathy Disability Score (NDS) were used. The Short Form Health Survey (SF-36) scale was used to assess quality of life. Factors related or unrelated to HIV were obtained through the medical history and analysis on medical records. Results: The quality of life of patients with neuropathic pain was worse in six of the eight domains of the SF-36 scale. The number of clinical manifestations related to HIV, length of time with detectable viral load since diagnosis, length of time since the diagnosis of HIV infection and length of time of HAART use had a negative impact on quality of life. Higher levels of CD4, education and family income had a positive impact. Conclusions: Painful neuropathy related to HIV is a factor that worsens the quality of life of patients infected with this virus and should be included in the clinical evaluation.

RESUMO Antecedentes: Após o advento da terapia antirretroviral combinada a infecção pelo vírus da imunodeficiência humana (HIV) deixou de ser uma doença devastadora, porém a neuropatia sensitiva consequente à permanência do vírus e ao efeito colateral do tratamento piora a morbidade desses pacientes. Objetivo: Investigar a qualidade de vida de 64 pacientes com HIV, 24 com neuropatia dolorosa (grupo caso) e 40 sem neuropatia dolorosa (grupo controle). Avaliou-se também o impacto de outros fatores relacionados e não relacionados ao HIV na qualidade de vida. Métodos: Para avaliação da neuropatia dolorosa foram utilizadas as escalas Leeds Assessment of Neuropathic Symptoms and Signs (LANSS), Douleur Neuropathique 4 (DN4) e Escore de Comprometimento Neuropático (ECN). Para avaliação da qualidade de vida foi utilizada a escala Short Form Health Survey (SF-36). Fatores relacionados e não relacionados ao HIV foram obtidos através da anamnese e análise de prontuário. Resultados: A qualidade de vida dos pacientes com dor neuropática foi pior em 6 dos 8 domínios da escala SF-36. O número de manifestações clínicas relacionadas ao HIV, tempo de carga viral detectável desde o diagnóstico, tempo de diagnóstico da infecção pelo vírus e tempo de uso de TARVC impactaram negativamente na qualidade de vida. Maior nível de CD4, da escolaridade e da renda familiar impactaram positivamente. Conclusões: A neuropatia dolorosa relacionada ao HIV é fator de piora da qualidade de vida dos pacientes infectados por esse vírus devendo ser incluída na avaliação clínica desses pacientes.

Corneal confocal microscopy in a healthy Brazilian sample.

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.