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Introdução: Comprometimento cognitivo (CC) é comum no paciente renal crônico, porém pouco avaliado. A anemia e a deficiência de ferro podem contribuir para o CC. Objetivo: avaliar CC e sua associação com alterações no metabolismo do ferro na DRC pré-dialítica. Método: Estudo transversal com 54 pacientes entre 21 e 65 anos avaliados sociodemografica, clínica e laboratorialmente. Realizada triagem cognitiva completa, teste de rastreio de cognição global: Montreal Cognitive Assessment (MoCA), bateria de testes de memória, atenção, velocidade de processamento, fluência verbal e funções executivas, escalas de sono (Escala de Sonolência Diurna de Epworth, Questionário Clínico de Apnéia Obstrutiva do Sono de Berlin(AOS), Questionário de cinco perguntas de sintomas de Pernas Inquietas, depressão (Inventário de depressão de Beck, Mini-Plus para Episódio Depressivo Maior (DSM-IV) e de funcionalidade (Questionário de Atividades Funcionais de Pfeffer). Resultados: AOS esteve presente em 76,9%, pernas inquietas e sonolência diurna (35,2%) e sintomas depressivos (34,7%). O MoCA esteve alterado em 59,3%. Correlação de Pearson entre testes neuropsicológicos e Hemoglobina(Hb), índice de Saturação de Transferrina(IST), ferritina e PCRus, mostrou associação entre MoCA e Hb (r=0,310 e p=0,02). Regressão Linear utilizando três modelos ajustados por variáveis sociodemográficas e IST ou ferritina ou Hb: modelo 1 e 2-escolaridade e depressão se associaram ao MoCA, modelo 3-Hb e depressão se associaram ao MoCA. Conclusão: O nível sérico de Hb e a presença de depressão foram associadas com CC. Níveis mais baixos de ferritina se correlacionaram com o MoCA, enquanto não encontramos associação com os demais marcadores do metabolismo do ferro.

Introduction We compared peripapillary retinal nerve fiber layer and macular thickness measurements in patients with mild cognitive impairment (MCI) and control subjects using swept-source optical coherence tomography (SS-OCT). We also assessed the relationship between SS-OCT measurements and the severity of cognitive impairment. Methods Peripapillary retinal nerve fiber layer and macular thickness were measured in 23 patients and 24 control subjects using SS-OCT. Cognitive status was assessed using the Mini-Mental State Examination, the Montreal Cognitive Assessment, and the Pfeffer Questionnaire. Results Most inner retinal layer thickness parameters were significantly smaller in patients with MCI, especially macular ganglion cell complex thickness measurements. Mini-Mental State Examination and Montreal Cognitive Assessment findings were significantly correlated with most macular thickness parameters. Discussion The SS-OCT–measured inner retinal layers of patients with MCI displayed thinning, especially in the central macular area. SS-OCT technology can provide useful information on ocular involvement patterns and holds promise as an ocular biomarker in this patient population., Highlights • Inner retinal layer thickness was reduced in patients with mild cognitive impairment. • Macular ganglion cell layer complex was the most affected retinal layer. • Peripapillary retinal nerve fiber layer thickness was not significantly reduced. • Cognitive tests significantly correlated with macular parameters.

Introdução. No Brasil, a população com mais de 65 anos pode chegar a 25,5%, em 2060. Com o envelhecimento populacional ocorre um aumento da prevalência das doenças degenerativas, dentre as quais o comprometimento cognitivo (CC). Impacto semelhante é observado na doença renal crônica (DRC). Existem fatores fisiopatológicos comuns entre a DRC e o CC. Dentre pacientes com DRC, até 38% apresentam CC. Em relação à fisiopatologia das alterações cognitivas na DRC, os achados são controversos. Objetivo. Avaliar os estudos relacionados à disfunção cognitiva na população com DRC pré-dialítica. Método. Utilizamos as estratégias PRISMA. Selecionamos os unitermos “renal insufficiency, chronic” and “cognitive disfunction”, realizamos a revisão sistemática e não meta-análise dada a disparidade dos estudos. Utilizamos as bases de dados disponíveis de forma gratuita: PubMed, Scielo. A seleção dos artigos foi realizada por dois pesquisadores. Critérios de inclusão: apresentar resumo em língua portuguesa, inglesa, espanhola ou francesa; incluir pacientes em pré-diálise; utilizar avaliações clínicas e/ou laboratoriais e/ou de imagens. Resultados. Encontramos 116 artigos e avaliamos 51. Conclusão. O CC é frequente na DRC e a etiologia permanece em discussão. A maioria dos estudos é derivada de dados secundários de grandes coortes ou estudos transversais. Os instrumentos clínicos mais utilizados são o MEEM e o MoCA e de neuroimagem, a ressonância magnética. Poucos estudos avaliam a inflamação e as toxinas urêmicas. Os mecanismos do CC na DRC são diversos, nem sempre correlacionados, existindo uma grande lacuna no melhor entendimento da associação fisiopatológica destas duas importantes morbidades.

Background and Aims Mild cognitive impairment (MCI) consists of a decline of one or more cognitive domains, independent of functional impairment. There are common pathophysiological factors between chronic kidney disease (CKD) and MCI. The aim of this study was to evaluate the association of MCI in pre-dialysis CKD with inflammation markers and alterations of image exams (Nuclear Magnetic Resonance-MRI). Method Cross-sectional study, were evaluated non-dialysis CKD patients in categories 1 to 5 in a secondary care clinic, from 2013 to 2015. Inclusion Criteria: age ≥ 21 and ≤ 65 years and having signed the informed consent form. Non-inclusion criteria: previous history of stroke, degenerative and infectious diseases of the CNS, presence of delirium and / or psychotic disorders, previous history of mental retardation and cranioencephalic trauma, visual and auditory disorders that prevented the tests, HIV and / or AIDS, contraindication or intolerance to MRI. The project was approved by the Research Ethics Committee. MCI was assessed using the Montreal Cognitive Assessment (MoCA) and the estimated glomerular filtration rate (eGFR), using the CKDEPI formula. Socio-demographic and clinical data were collected from medical records. Laboratory data were collected a maximum of 3 months after the MoCA evaluation, as well as the measurement of IL4, IL6, IL17, TNF alpha and hs-CRP. The brain MRI scans were performed in a Siemens Avanto high-field device (1.5 Tesla), without the use of paramagnetic contrast. Fazekas scales were used to quantify white matter lesions, MTA scale to quantify hippocampal involvement, ACG scale for global cortical atrophy. Statistical analysis a descriptive analysis was performed, followed by a comparison of abnormal vs normal MoCA among all variables with the relevant tests; MoCA and eGFR were also correlated with all variables. Finally, we performed a linear regression using MoCA as a dependent variable, adjusting for relevant confounding variables. Results 111 patients were invited, 80 were included in the neuropsychological assessment and 56 patients performed all stages of the study. The mean age was 56.3 ± 8.3 years, 51.8% had an altered MoCA. Comparing those with altered vs normal MoCA, we observed that there were fewer years of schooling (p = 0.04), greater use of diuretics (p = 0.04) and AAS (p = 0.002); in addition to higher levels of IL6 (p = 0.02) and IL17 (p = 0.05) among those with altered MoCA. There was no correlation between MoCA and eGFR. MoCA correlated with IL6 (R-0.201, p = 0.04). There was a correlation between eGFR (IL4 (R- -0.467, p = 0.005; IL6 (R- -0.652, p Conclusion The prevalence of changes in MoCA in this population was 51.8%, the eGFR was correlated with inflammation, MoCA was negatively correlated with IL6. There was no association between MRI findings and eGFR or inflammatory variables, and eGFR was not a predictor of MoCA values, while IL17 and IL6 were.

Structural imaging of the brain is the most widely used diagnostic tool for investigating neurodegenerative diseases. More advanced structural imaging techniques have been applied to early or prodromic phases, but they are expensive and not widely available. Therefore, it is highly desirable to search for noninvasive, easily accessible, low-cost clinical biomarkers suitable for large-scale population screening, in order to focus on making diagnoses at the earliest stages of the disease. In this scenario, imaging studies focusing on the structures of the retina have increasingly been used for evaluating neurodegenerative diseases. The retina shares embryological, histological, biochemical, microvascular and neurotransmitter similarities with the cerebral cortex, thus making it a uniquely promising biomarker for neurodegenerative diseases. Optical coherence tomography is a modern noninvasive imaging technique that provides high-resolution two-dimensional cross-sectional images and quantitative reproducible three-dimensional volumetric measurements of the optic nerve head and retina. This technology is widely used in ophthalmology practice for diagnosing and following up several eye diseases, such as glaucoma, diabetic retinopathy and age-related macular degeneration. Its clinical impact on neurodegenerative diseases has raised enormous interest over recent years, as several clinical studies have demonstrated that these diseases give rise to reduced thickness of the inner retinal nerve fiber layer, mainly composed of retinal ganglion cells and their axons. In this review, we aimed to address the clinical utility of optical coherence tomography for diagnosing and evaluating different neurodegenerative diseases, to show the potential of this noninvasive and easily accessible method. RESUMO A avaliação estrutural do cérebro, feita por meio dos exames de neuroimagem, é a forma mais utilizada de ferramenta diagnóstica e de acompanhamento das doenças neurodegenerativas. Técnicas de imagem mais sofisticadas podem ser necessárias especialmente nas fases mais precoces, antes mesmo do surgimento de quaisquer sintomas, porém costumam ser caras e pouco acessíveis. Sendo assim, é de fundamental importância a busca de biomarcadores não invasivos, de fácil acesso e baixo custo, que possam ser utilizados para rastreio populacional e diagnóstico mais precoce. Nesse cenário, o número de estudos com ênfase em técnicas de imagem para avaliação estrutural da retina em pacientes com doenças neurodegenerativas tem aumentado nos últimos anos. A retina apresenta similaridade embriológica, histológica, bioquímica, microvascular e neurotransmissora com o córtex cerebral, tornando-se assim um biomarcador único e promissor nas doenças neurodegenerativas. A tomografia de coerência óptica é uma moderna técnica de imagem não invasiva que gera imagens seccionais bidimensionais de alta resolução e medidas volumétricas tridimensionais reprodutivas do disco óptico e da mácula. Essa tecnologia é amplamente utilizada na prática oftalmológica para o diagnóstico e o seguimento de diversas doenças oculares, como glaucoma, retinopatia diabética e degeneração macular relacionada à idade. A redução da espessura da camada de fibras nervosas da retina e das camadas de células ganglionares em pacientes com doenças neurodegenerativas foi demonstrada em diversos estudos clínicos nos últimos anos. Nesta revisão, abordamos as principais aplicações clínicas da tomografia de coerência óptica nas doenças neurodegenerativas e discutimos o seu papel como potencial biomarcador nessas afecções.

Context: COVID19 has better known respiratory impacts than cardiovascular1 and high D-dimer as the most significant coagulation parameter2 . Otherwise, paradoxical embolism due to Patent Foramen Ovale (PFO) and Ischaemic Cerebral Vascular Accident (iCVA) mechanisms associated are poorly documented3, 4 . We aimed to report an associated case of iCVA and PFO and highlight COVID19 hypercoagulability triggering thromboembolisms. Case report: CTT, 76y, female, hypertensive, former-smoker was hospitalized with right-hemiplegia, transcortical aphasia, dysarthria by iCVA and evolved with headache, odynophagia, fever, chills. RT-PCR-SARS-Cov2: positive; chestCT: 25% bilateral pulmonary involvement, ground-glass opacities. 8days later, was transferred to ICU-COVID-HU-UFJF; D-dimer=827. In 30days, went to ward with O2-dependent pulmonary sequelae; CT-angiography excluded Pulmonary Thromboembolism. Searched iCVA mechanism, ECO detected PFO=2.8mm and Interatrial Septum aneurysm. Prescribed anticoagulants. Subsequently, presented right-clonus, further previous RankinScale=5. Requested brain-MRI pointed lobar hemorrhage with mass effect in recent iCVA’s territory (left-Middle Cerebral Artery). After 10days, a head-CT evidenced partial resorption and reduced mass effect. Patient was discharged taking rivaroxabana. Conclusions: Retrospectively, we suspected that COVID19 hypercoagulability triggered Deep Vein Thrombosis and the consequent PFO paradoxical embolism, which caused iCVA. Therefore, vascular pathologies in COVID19 deserve further studies. Treatment for secondary prevention in iCVA by PFO is uncertain3 .

Background: Alzheimer’s disease (AD) represents one of the main causes of cognitive and functional decline in the world. Concomitant with pharmacological treatment, the practice of aerobic exercises (AE) can help in the symptomatic control of the disease. Objectives: To evaluate the effects of AE on activities of daily living and cognition in patients with AD. Methods: A systematic review was undertaken. EMBASE, Pubmed and BVS databases were searched using the terms “Alzheimer disease”, “Alzheimer syndrome” and “Alzheimer dementia”; “aerobic” and “exercise”. The inclusion criteria were: randomized controlled trials from 2016 to 2021, English language studies and human studies. Among 854 studies found, six were included in the review. Results: The potential benefits of AE training in AD patients are: improvement of functioning, quality of life and cognitive performance; better control of neuropsychiatric symptoms and possible reduction of systemic inflammation. Conclusions: AEs are associated with cognitive and functional performance gain in AD, probably related to synaptic plasticity optimization and improvement of the feeling of well-being. Although AEs may improve cognitive and neuropsychiatric symptoms, the response to treatment is individual. Future longitudinal studies with larger cohorts and functional neuroimaging studies are required for a better understanding of the real benefit of AE in AD.

Background: Headache is the most frequent neurological complaint in the population and the group of tension-type headaches (TTH) is the most prevalent subtype. Nevertheless, more information about the clinical features of headaches in patients attended at specialized centers are demanded. Methods: Cross-sectional, descriptive study. Data from patients referred to an outpatient specialized headache center from 2018 to 2019 were analyzed and clinical and epidemiological information was collected. This study was authorized by the research ethics committee of the HUUFJF (CAAE 03530818.9.0000.5133). Results: Data from 153 patients were assessed. The mean age of patients was 45,6 years and most cases were women (80,4%). The most frequent diagnosis were migraine (49,7%), TTH (22,8%) and temporomandibular disorders (8,5%). The prevalence of chronic headaches was 46,6%. Analgesic abuse was identified in 32% of participants, with a higher prevalence in women (Fischer’s exact test, P=0,05). Prophylactic treatment was used by 84,3% of the subjects. Pain was self-reported mild in 21,6% of cases, moderate in 30,1% and severe in 47,1%. The pain severity was inversely proportional to age (P=0,012). The most frequent associated symptoms were photophobia (57,5%), phonophobia (56,9%), nausea/ vomiting (47,1%). Conclusions: The findings show important differences in the prevalence of headache cases in specialized centers compared to the general population. Given the high prevalence of analgesic abuse reported, the development of effective educational programs for patients and healthcare providers at primary and secondary health services, might reduce the social burden of chronic headaches and decrease the demand for consultations on specialized headache clinics.

A doença de Behçet é uma doença sistêmica, sem marcador específico, cuja descrição clássica é de uma tríade clínica de úlceras genitais, aftas orais e lesão ocular (uveíte). O envolvimento do sistema nervoso (neuro-Behçet) é relatado em 4% a 29% dos indivíduos acometidos pela doença. Relatamos dois casos de envolvimento do sistema nervoso, em um homem e em uma mulher. No primeiro, houve acometimento do terceiro nervo craniano à direita e comprometimento neuropsicológico. O segundo caso caracterizou-se por cefaléia, baixa acuidade visual, astenia, eritema nodoso, trombose venosa profunda e edema de papila

Introdução: O Acidente Vascular Cerebral (AVC) é uma das maiores causas de morte e incapacidade adquirida em todo o mundo. Além da alta mortalidade, o AVC é um importante causador de déficits neurológicos irreversíveis que necessitam de reabilitação, sendo que aproximadamente 70% dos pacientes não retomarão ao seu trabalho e 30% necessitarão de auxílio para caminhar. Entender o perfil epidemiológico e a contribuição de cada fator de risco é essencial para estabelecer políticas locais específicas para diminuir a incidência da doença e prevalência de complicações que ocorrem devido ao AVC, tanto para o bem-estar a curto e a longo prazo da população. A prevenção pode ser feita em até 90% dos casos de AVC, visto que estes podem ser atribuídos a fatores de risco modificáveis. Objetivo: Conscientizar e orientar a população da cidade de Juiz de Fora, Minas Gerais acerca dos fatores de risco modificáveis do AVC e, consequentemente, sobre sua prevenção. Relato de experiência: No mês de outubro de 2018 foram realizadas palestras educativas e orientações a respeito do AVC e seus fatores de risco. Além disso, os pacientes do serviço de Neurologia, seus acompanhantes e transeuntes locais tiveram dois dos fatores de risco modificáveis associados ao AVC avaliados: a pressão arterial sistêmica e a circunferência abdominal. Aqueles com alterações foram orientados e encaminhados a procurar um serviço específico. Conclusão: A educação da população sobre as formas de prevenção de doenças cardiovasculares, em especial o acidente vascular cerebral, que é uma doença conhecida por apresentar tantos fatores causais modificáveis, é essencial para evitar o aumento da incidência dessas patologias tão prevalentes no país.

A 69-year-old man presented with a reduced level of consciousness and left hemiparesis immediately after a percutaneous CT-guided transthoracic needle lung biopsy, performed to investigate an interstitial pulmonary disease. CT scan of the head (figure 1A, B) showed small, 1–2 mm sparse and linearly arranged hypodensities, suggesting air emboli in the anterior and posterior territories of the right cerebral hemisphere circulation, without ischaemic lesions. However, MR brain imaging (figure 1C, D) ultimately confirmed stroke secondary to gas embolisation in the same territory. Echocardiography, carotid and vertebral arterial duplex scans, and routine laboratory tests were normal. We gave him aspirin, but his left leg weakness persisted. He died 1 year later from pulmonary complications of the primary …

Objetivo. Relatar um caso de coinfecção por neurotoxoplasmose e neurocisticercose em mulher acometida pela AIDS. Método. Relato de caso prospectivo, descritivo e contemporâneo de paciente do sexo feminino, 36 anos, com quadro clínico compatível com síndrome de hipertensão intracraniana. Os exames complementares diagnostica­ram neurotoxoplasmose e a sorologia anti-HIV foi positiva. Evolui, após semanas, com crises convulsivas e exames subsidiários de imagem demonstrando neurocisticercose. Resultados. As infecções oportunis­tas relacionadas à AIDS são frequentes. A neurotoxoplasmose é cau­sada pelo protozoário Toxoplasma gondii e principal causa de lesão intracraniana expansiva em pacientes com AIDS. A neurocisticercose, provocada por cisticercos (Cysticercus cellulosae ou C. racemosus), formas larvárias da Taenia solium, é bastante prevalente em nosso meio. Conclusão. Após suspeição clínico-radiológica de neurotoxo­plasmose, torna-se imperativo realização de teste anti-HIV, devido sua elevada frequência neste grupo de pacientes. Já a comorbidade neurocisticercose e AIDS é achado excepcional, resultado mais prova­velmente de mera coincidência, sem qualquer vínculo predisponente entre si.

Multiple sclerosis (MS) starting in childhood and adolescence poses a challenge for diagnosis and management of the disease. The aim of the present study was to assess the characteristics of early onset MS in Brazilian patients. Methods Retrospective data collection from specialized MS units. Results From 20 MS units in 11 Brazilian states, 117 cases of MS starting before the age of 18 years were collected. These patients had an average of 10 years of disease duration, still typically with low disability and one relapse every 2.5 years. The mean age for disease onset was 13.7 years. Conclusion The present study introduces a large series of Brazilian cases of pediatric MS. Although some patients presented a very severe form of MS, on the whole the group of patients with MS starting in childhood or adolescence presented a relatively mild form of this disease in Brazil.

Introdução: Indivíduos com doença renal crônica (DRC) têm grande risco de desenvolver comprometimento cognitivo (CC), inicialmente leve (CCL), passível de identificação, mas ainda subdiagnosticado e subtratado. O Montreal Cognitive Assessment (MoCA) vem sendo indicado para rastreio de CCL na DRC. Objetivo: Avaliar o CCL em indivíduos com DRC pré-dialítica. Métodos: O estudo foi realizado em 72 indivíduos, não idosos, com DRC nos estágios pré-dialíticos. A avaliação neuropsicológica incluiu: o teste de cognição global MoCA; o teste do relógio (TDR); o Digit Span ordem direta (DOD) e inversa (DOI); o teste de fluência verbal (FV), fonêmica (FAS) e semântica (animais); o punho-borda-mão (PBM); e de memória 10 figuras. Resultados: A média de idade dos participantes foi de 56,74 ± 7,63 anos, com predominância de homens (55,6%), com escolaridade ≥ 4 anos (84,3%), a maioria com DRC 1, 2 e 3a e 3b (67,6%), hipertensa (93,1%) e diabética (52,1%). O CC (MoCA ≤ 24) foi observado em 73,6% dos usuários. Não encontramos associação das variáveis demográficas e clínicas com CC, mas tendência de associação com a idade (p = 0,07), com a escolaridade (p = 0,06) e com o DM (0,06). Os testes de função executiva, TDR, DOI e PBM, isoladamente, apresentaram boa sensibilidade e valor preditivo negativo comparados ao MoCA para a identificação de CC e, em conjunto, foram capazes de predizer o resultado do MoCA. Conclusão: O CCL é frequente em usuários não idosos com DRC pré-dialítica. O TDR, DOI e PBM associados são equivalentes ao MoCA na identificação do CC nessa população, sugerindo comprometimento de funções executivas. Introduction: Individuals with chronic kidney disease (CKD) are at higher risk of developing cognitive impairment (CI), initially mild (MCI), potentially identifiable, but still poorly diagnosed and treated. The Montreal Cognitive Assessment (MoCA) has been indicated for MCI screening in CKD. Objective: To assess MCI in patients with CKD not yet on dialysis. Methods: Study conducted in 72 non-elderly subjects with pre-dialysis CKD. The neuropsychological assessment included: The global cognitive assessment test MoCA; the clock drawing (CD); the digit span forward (DSF) and reverse (DSR); phonemic verbal (VF) fluency (FAS) and semantics (animals); the fist-edge-palm (FEP); and the memory 10 pictures. Results: The average age of the participants was 56.74 ± 7.63 years, with predominance of male sex (55.6%), mainly with ≥ 4 years of education (84.3%), with CKD cathegories 1, 2 and 3a and 3b (67.6%), hypertension (93.1%) and diabetes mellitus (52.1%). MCI (MoCA ≤ 24) was observed in 73.6% of the patients. We did not find association among MCI with demographic and clinical variables, but a tendency to association with age (p = 0.07), educational level (p = 0.06) and diabetes (0.06). The executive function tests CD, DS-reverse and FEP, individually were able to identify CI with good sensibility and negative predictive value compared to MoCA and together, showed the same capability to identify MCI when compared to MoCA. Conclusion: The MCI is common in non-elderly patients with CKD not yet on dialysis. Together, the CD, DSR and FEP showed similar performance in identify MCI in this population when compared to MoCA, suggesting impairment of executive functions.

Resumo Introdução: Indivíduos com doença renal crônica (DRC) têm grande risco de desenvolver comprometimento cognitivo (CC), inicialmente leve (CCL), passível de identificação, mas ainda subdiagnosticado e subtratado. O Montreal Cognitive Assessment (MoCA) vem sendo indicado para rastreio de CCL na DRC. Objetivo: Avaliar o CCL em indivíduos com DRC pré-dialítica. Métodos: O estudo foi realizado em 72 indivíduos, não idosos, com DRC nos estágios pré-dialíticos. A avaliação neuropsicológica incluiu: o teste de cognição global MoCA; o teste do relógio (TDR); o Digit Span ordem direta (DOD) e inversa (DOI); o teste de fluência verbal (FV), fonêmica (FAS) e semântica (animais); o punho-borda-mão (PBM); e de memória 10 figuras. Resultados: A média de idade dos participantes foi de 56,74 ± 7,63 anos, com predominância de homens (55,6%), com escolaridade ≥ 4 anos (84,3%), a maioria com DRC 1, 2 e 3a e 3b (67,6%), hipertensa (93,1%) e diabética (52,1%). O CC (MoCA ≤ 24) foi observado em 73,6% dos usuários. Não encontramos associação das variáveis demográficas e clínicas com CC, mas tendência de associação com a idade (p = 0,07), com a escolaridade (p = 0,06) e com o DM (0,06). Os testes de função executiva, TDR, DOI e PBM, isoladamente, apresentaram boa sensibilidade e valor preditivo negativo comparados ao MoCA para a identificação de CC e, em conjunto, foram capazes de predizer o resultado do MoCA. Conclusão: O CCL é frequente em usuários não idosos com DRC pré-dialítica. O TDR, DOI e PBM associados são equivalentes ao MoCA na identificação do CC nessa população, sugerindo comprometimento de funções executivas. Abstract Introduction: Individuals with chronic kidney disease (CKD) are at higher risk of developing cognitive impairment (CI), initially mild (MCI), potentially identifiable, but still poorly diagnosed and treated. The Montreal Cognitive Assessment (MoCA) has been indicated for MCI screening in CKD. Objective: To assess MCI in patients with CKD not yet on dialysis. Methods: Study conducted in 72 non-elderly subjects with pre-dialysis CKD. The neuropsychological assessment included: The global cognitive assessment test MoCA; the clock drawing (CD); the digit span forward (DSF) and reverse (DSR); phonemic verbal (VF) fluency (FAS) and semantics (animals); the fist-edge-palm (FEP); and the memory 10 pictures. Results: The average age of the participants was 56.74 ± 7.63 years, with predominance of male sex (55.6%), mainly with ≥ 4 years of education (84.3%), with CKD cathegories 1, 2 and 3a and 3b (67.6%), hypertension (93.1%) and diabetes mellitus (52.1%). MCI (MoCA ≤ 24) was observed in 73.6% of the patients. We did not find association among MCI with demographic and clinical variables, but a tendency to association with age (p = 0.07), educational level (p = 0.06) and diabetes (0.06). The executive function tests CD, DS-reverse and FEP, individually were able to identify CI with good sensibility and negative predictive value compared to MoCA and together, showed the same capability to identify MCI when compared to MoCA. Conclusion: The MCI is common in non-elderly patients with CKD not yet on dialysis. Together, the CD, DSR and FEP showed similar performance in identify MCI in this population when compared to MoCA, suggesting impairment of executive functions.

Purpose To evaluate the ability of frequency domain optical coherence tomography (fd-OCT) to estimate retinal neural loss in eyes with Alzheimer’s disease (AD). We also verified the existence of a correlation between AD-related cognitive impairment and macular and peripapillary retinal nerve fiber layer (RNFL) thickness measurements. Methods fd-OCT scans were obtained from 45 eyes of 24 patients with AD and 48 control eyes. Peripapillary RNFL, macular full-thickness and segmented inner macular thickness parameters were calculated. The inner macular parameters included macular retinal nerve fiber layer (mRNFL) thickness, ganglion cell layer (GCL) plus inner plexiform layer thickness (GCL+), and RNFL plus GCL+ thickness (GCL++). The Mini-Mental State Examination (MMSE) was used to assess cognition in all subjects. The two groups were compared and the relationship between MMSE scores and fd-OCT measurements was verified. Results Average, superior and inferior quadrant RNFL thickness parameters and all but one of the nine full-thickness macular measurements were significantly reduced in AD patients compared to controls. The segmented layers, GCL+ and GCL++ were significantly reduced in AD eyes. A significant correlation was found between most fd-OCT parameters (especially macular thickness measurements) and MMSE scores. Conclusions Most fd-OCT peripapillary RNFL and macular full-thickness and segmented inner retinal layers parameters were reduced in AD eyes compared to controls. Moreover, neuronal loss, especially as reflected in macular parameters, correlated well with cognitive impairment in AD. Our results suggest that fd-OCT could be a potentially useful diagnostic tool in the evaluation and follow-up of AD patients.

A 16-year-old boy presented with a 5-year history of progressive cognitive decline and behavioral change followed by generalized tonic-clonic and myoclonic seizures refractory to many anticonvulsants (valproic acid, phenobarbital, clonazepam, and topiramate) and cerebellar ataxia a year later. The son of consanguineous parents, he had a family history of 2 cousins who both had epilepsy and died at the ages of 15 and 25 years. Laboratory screening tests, including lactic acid, fundus examination, brain neuroimaging, and CSF, were normal. Skin biopsy (figure) revealed periodic acid–Schiff-positive intracellular polyglucosan inclusion bodies in myoepithelial and sweat gland duct cells. These findings are typical of Lafora disease, a fatal autosomal recessive disorder caused by mutations in one of 2 known genes, both located at chromosome 6: EPM2A , which encodes the protein laforin, and EPM2B , which encodes the protein malin. Differential diagnosis must be made with other causes of progressive myoclonic epilepsies, most commonly Unverricht-Lundborg disease (Baltic myoclonus), myoclonus epilepsy and ragged-red fibers syndrome, neuronal ceroid lipofuscinosis, and type 1 sialidosis. There is no treatment, and therapy is mainly supportive and symptomatic.1,2

A trombose venosa cerebral (TVC) é doença vascular com diferentes manifestações clínicas e várias causas possíveis (locais, sistêmicas ou idiopáticas). A trombose da veia de Galeno (TVG) é causa rara de TVC e geralmente está associada a alguma malformação vascular. Relatamos o caso de uma paciente de 16 anos que apresentou TVG sem malformação vascular, porém associada a trombose de seio reto e infarto venoso talâmico. Discutem-se também aspectos importantes do diagnóstico clínico, radiológico e laboratorial da TVC. Cerebral venous thrombosis (CVT) is a vascular disease with many clinical manifestations and possible etiologies (local, systemic or idiopathic). Galen vein thrombosis (GVT) is a rare cause of CVT and usually it is associated with some vascular malformation. We report a case of a 16 years old female patient with GVT without vascular malformation, but associated with straight sinus thrombosis and venous thalamic infarct. Relevant aspects of the clinical, radiological and laboratory diagnosis of CVT are also discussed.

Ossification of the posterior longitudinal ligament (OPLL) is a rare cause of myelopathy in non-Oriental populations and relatively unrecognized by general practitioners. A case of an Afro-Brazilian 54-years-old woman presenting with tetraparesis due to cervical OPLL is presented. Emphasis is made for the inclusion of OPLL in the differential diagnosis of compressive cervical myelopathy.

Cerebral venous thrombosis (CVT) is a vascular disease with many clinical manifestations and possible etiologies (local, systemic or idiopathic). Galen vein thrombosis (GVT) is a rare cause of CVT and usually it is associated with some vascular malformation. We report a case of a 16 years old female patient with GVT without vascular malformation, but associated with straight sinus thrombosis and venous thalamic infarct. Relevant aspects of the clinical, radiological and laboratory diagnosis of CVT are also discussed.

Meningoceles intra-sacrais são lesões raras, que podem determinar sintomas de compressão radicular. Descrevemos o caso de uma paciente de 18 anos, com queixa de incontinência urinária, acometida por esta patologia. Após avaliação neuro-urológica e estudo uro-dinâmico, foi demonstrada arreflexia do detrusor, sendo aventada a hipótese de disrafismo espinhal. O diagnóstico de meningocele intra-sacral oculta foi evidenciado através de ressonância magnética. A paciente foi submetida a abordagem cirúrgica com boa recuperação pós-operatória. No presente artigo, é feita breve revisão da literatura, e são discutidas as apresentações clínicas possíveis, os achados neuro-radiológicos e o tratamento cirúrgico. Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary incontinence. The neurological and urological evaluation revealed lack of detrusor muscle reflex. This finding supported the hypothesis of spinal disraphism. Magnetic resonance image revealed an intrasacral meningocele. The patient was submitted to surgical excision of the lesion without postoperative complications. In the present paper, the pathogenesis, clinical picture, neuroradiological findings and surgical treatment are discussed.