Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ or system. Neuropsychiatric and pulmonary involvement can occur in 40 and 50% of patients respectively, and may occur in several different clinical forms. While the main neuropsychiatric manifestations are represented by cognitive impairment, organic cerebral syndromes, delirium, psychosis, seizures, and peripheral neuropathies, the main forms of pulmonary involvement are pleurisy with or without pleural effusion, pneumonitis, interstitial disease, pulmonary hypertension, and alveolar hemorrhage. The authors report the case of a 49-year-old woman whose first manifestation of SLE was represented by two rare manifestations: rapidly progressive cognitive impairment, which was associated with respiratory failure caused by the shrinking lung syndrome. The authors call attention to the under-diagnosis of lupus pulmonary complications and its association with severe cognitive impairment that often necessitates aggressive treatment

Challenges in the diagnosis of dementia: Insights from the United Kingdom-Brazil Dementia Workshop

In July 2019, a group of multidisciplinary dementia researchers from Brazil and the United Kingdom (UK) met in the city of Belo Horizonte, Minas Gerais, Brazil, to discuss and propose solutions to current challenges faced in the diagnosis, public perception and care of dementia. Here we summarize the outcomes from the workshop addressing challenges in diagnosis. Brazil faces a major problem in dementia underdiagnosis, particularly involving the population in an adverse socioeconomic context. There is poor availability of resources and specialists, and the knowledge of general practitioners and other healthcare professionals is far from satisfactory. Low education level is a further obstacle in diagnosing dementia, as the most commonly used screening tests are not designed to evaluate this population. Patients and their families must overcome the stigma of a diagnosis of dementia, which is still prevalent in Brazil and increases the burden of this condition. Whilst the UK has greater resources, dedicated memory services and a National Dementia Strategy plan, the National Health Service (NHS) has limited funding. Therefore, some challenges regarding diagnosis are common across both countries. The authors suggest possible solutions to confront these, with the goal of improving assessment and recognition of dementia and reducing misdiagnosis

Phenotypic and Positron Emission Tomography with [18F]fluordeoxyglucose (FDG PET) differences in corticobasal syndrome: comparison of two cases

ABSTRACT Corticobasal syndrome (CBS) is a rare cause of dementia and comprises varied combinations of subcortical signs (akinetic-rigid parkinsonism, dystonia, or myoclonus) with cortical signs (apraxia, alien hand or cortical sensory deficit), usually asymmetric. We aimed to report and compare the clinical and neuroimaging presentation of two patients diagnosed with CBS. While case 1 had severe non-fluent aphasia associated with mild apraxia and limb rigidity, case 2 had a more posterior cognitive impairment, with a different language pattern associated with marked visuospatial errors and hemineglect. FDG PET played a significant role in diagnosis, suggesting, in the first case, corticobasal degeneration and, in the second, Alzheimer's disease pattern. CBS has been widely studied with the advent of new in vivo methods such as brain FDG PET. Studies that deepen the phenotypic and biomarker heterogeneity of CBS will be of great importance for better classification, prognosis, and treatment of the condition

Evaluation of chronic stress markers in individuals from the Brazilian aging and memory study

Introdução: Diversos estudos sugerem que o estresse crônico e níveis persistentemente elevados de cortisol estão relacionados a disfunção cognitiva e processos neurodegenerativos. O estudo brasileiro de memória e envelhecimento (Brazilian Memory and Aging Study BRAMS) é uma iniciativa de pesquisa para acompanhamento longitudinal de indivíduos em ambulatório especializado em cognição. Objetivos: investigar a relação entre marcadores de estresse crônico e status cognitivo no BRAMS, realizando comparações entre os grupos classificados como controle, declínio cognitivo subjetivo (DCS) e comprometimento cognitivo leve (CCL) (amnéstico, não amnéstico, único domínio ou múltiplos domínios). Secundariamente correlacionar os marcadores quanto ao status amiloide dos participantes. Metodologia: estudo observacional do tipo recorte transversal de subgrupo do BRAMS, em que indivíduos de idade > 60 anos que tiveram acesso à avaliação médica, neuropsicológica e PET amiloide. A presente análise busca aferir o índice de carga alostática (ICA), uma medida global de estresse crônico que engloba marcadores antropométricos, endocrinológicos, cardiovasculares, metabólicos e inflamatórios, correlacionando as variáveis com status cognitivo e status amiloide. Resultados: Entre os anos de 2018 e 2021, 77 participantes foram avaliados no presente protocolo, sendo que, destes, 65 preencheram critério de inclusão. A média de idade foi de 70,2 (+ 6,01) anos, sendo 72% do sexo feminino e escolaridade média de 11 anos (intervalo interquartil 7 15). Houve 42 indivíduos classificados como CCL, 15 como DCS e 8 como controles. Houve diferença significativa entre as medidas de HDL (high-density lipoprotein) (55,6 no grupo CCL vs. 66 no grupo DCS vs. 69 no grupo controle, p = 0,04), relação cintura/quadril (0,94 no grupo CCL vs. 0,88 no grupo DCS, p = 0.03) e também para a medida do ICA em valor percentual considerando os extremos de cortisol (ICA % cortisol p87,5) (36,9% no grupo CCL vs. 27,2% no grupo DCS, p = 0,04). Corrigindo-se para fatores de confusão através da análise multivariável, maior escolaridade foi associada a risco reduzido de CCL. Maior escolaridade e classe econômica (ABEP) também foram associadas de forma independente a menor chance de DCS. Conclusão: o presente trabalho representa provavelmente uma das primeiras iniciativas de pesquisa a abordar as medidas de estresse crônico para além do cortisol em uma população com os diagnósticos de DCS e CCL, com marcadores aumentados no grupo CCL. Pela natureza transversal do estudo, as associações encontradas não permitem inferir causalidade entre as medidas, mas poderão ser melhor exploradas em seguimento longitudinal dos participantes no médio e longo prazoIntroduction: Several studies suggest that chronic stress and persistently high levels of cortisol are related to cognitive dysfunction and neurodegeneration. The Brazilian Memory and Aging Study (BRAMS) is a research initiative for the longitudinal follow-up of individuals in a tertiary center specialized memory outpatient clinic. Objectives: to investigate the relationship between markers of chronic stress and cognitive status in BRAMS, making comparisons between participants classified as controls, subjective cognitive decline (SCD) and mild cognitive impairment (MCI) (amnestic, non-amnestic, single domain or multiple domains). Secondarily to correlate the markers with the amyloid status of the participants. Methodology: observational study of the cross-sectional type with a subgroup analysis of the BRAMS cohort, in which individuals aged > 60 years who had access to medical, neuropsychological and amyloid PET assessment. The present analysis seeks to measure the allostatic load index (ALI), a global measure of chronic stress through anthropometric, endocrinological, cardiovascular, metabolic and inflammatory markers, correlating the variables with cognitive status and amyloid status. Results: Between the years 2018 and 2021, 77 participants were evaluated in the present protocol, of which 65 met the inclusion criteria. The mean age was 70.2 (+ 6.01) years, with 72% being female and 11 years of schooling (interquartile range 7 15). There were 42 subjects classified as MCI, 15 as SCD and 8 as controls. There was a significant difference between HDL (high-density lipoprotein) measurements (55.6 in the MCI group vs. 66 in the SCD group vs. 69 in the control group, p = 0.04), waist/hip ratio (0.94 in the MCI group vs. 0, 88 in the SCD group, p = 0.03) and also for the measurement of the ALI in percentage value considering the extremes of cortisol (ALI % cortisol p87.5) (36.9% in the MCI group vs. 27.2% in the SCD group, p = 0.04). Correcting for confounders through multivariate analysis, higher education was associated with reduced risk of MCI. Higher education and economic class were also independently associated with a lower chance of SCD. Conclusion: The present work probably represents one of the first research initiatives to address measures of chronic stress beyond cortisol in elderly participants diagnosed with SCD and MCI, with increased markers in the MCI group. Due to the cross-sectional nature of the study, the associations found do not allow inferring causality between the measures, but they can be better explored in the longitudinal follow-up of the participant

Efficacy of assistive technologies in the surgical treatment of insular gliomas

Introdução: No campo do tratamento cirúrgico de Gliomas, tem se observado um interesse crescente no uso de novas tecnologias de suporte como métodos auxiliares na obtenção do equilíbrio entre radicalidade cirúrgica e preservação da funcionalidade cerebral. Na maior parte dos estudos, a localização tumoral tem sido pouco considerada e a real eficácia das tecnologias de suporte ainda está pouco investigada nos gliomas insulares. Objetivos: avaliar a eficácia da fluorescência intraoperatória com 5-ALA, monitorização neurofisiológica, neuronavegação e tractografia no grau de ressecção tumoral (GRT), escores de funcionalidade, sobrevida global e sobrevida livre de progressão em uma coorte retrospectiva de gliomas insulares. Métodos: revisamos todos os casos de tumores insulares operados no Departamento de Neurocirurgia da Universidade de Tübingen - Alemanha, entre maio de 2008 e novembro de 2013. O grau de ressecção foi avaliado por volumetria. Foram utilizados os testes de Mann Whitney, Qui-quadrado e funções de Kaplan Meier para análise do efeito de cada tecnologia nos desfechos primários e secundários. Resultados: 28 casos - 18 homens (64%) e 10 mulheres (36%); idade média 52,5 anos (12 - 59) - foram inclusos para análise. Gliomas de alto grau corresponderam a 20 casos (71%), com 8 lesões de baixo grau (29%). As tecnologias mais utilizadas foram monitorização neurofisiológica (64%) e neuronavegação (68%). 5-ALA foi a única modalidade associada a taxas de ressecção > 90% (p = 0,05). O uso de tractografia determinou melhora no KPS (50% vs 5%, p = 0,02). Houve associação positiva entre o uso de neuronavegação e sobrevida global (23 vs. 27,4 meses, p = 0,03), mas o uso de 5-ALA se associou a piora na sobrevida global (34,8 vs 21,1 meses, p = 0,01) e sobrevida livre de progressão (24,4 vs. 11,8 meses, p = 0,01). Conclusões: Considerando os gliomas insulares, o presente trabalho demonstra pioneiramente que o uso de 5-ALA tem papel na obtenção de maiores taxas de ressecção, ainda que este achado possa estar associado a piora nas sobrevidas global e livre de progressão. Tractografia e neuronavegação parecem desempenhar papel importante no tratamento dos gliomas insulares, na medida em que determinaram melhor sobrevida global e funcionalidade, respectivamente. Estudos prospectivos com uma amostra mais proeminente e análise multivariada permitirão a avaliação do benefício real destas tecnologias de suporte no tratamento dos gliomas insularesIntroduction: In the field of Glioma surgery, there has been an increasing interest in the use of assistive technologies to overcome the difficulty of preserving brain function while improving surgical radicality. In most reports, tumor localization has seldom been considered a variable and the role of intraoperative adjuncts is yet to be determined for gliomas of the insula. Objectives: to evaluate the efficacy of fluorescence-guided resection with 5-ALA, intraoperative neurophysiological monitoring (IOM), neuronavigation, and tractography in the Extent of Resection (EOR), functionality scores, overall survival (OS) and progression-free survival (PFS) in a retrospective cohort of insular gliomas. Methods: we reviewed all cases of insular tumors operated on at the Department of Neurosurgery, University Hospital of Tübingen - Germany, between May 2008 and November 2013. EOR was determined by volumetric analysis. Mann Whitney, Chi-square and Kaplan Meier functions were used for assessment of each technology\'s effect on primary and secondary outcomes. Results: 28 cases (18 men (64%) and 10 women (36%); median age at diagnosis: 52.5 years, range 12 - 59) were considered eligible for analysis. High grade and low grade gliomas accounted for 20 (71%) and 8 (29%) cases, respectively. The most used technologies were IOM (64%) and Neuronavigation (68%). 5-ALA was the only technique associated with EOR > 90% (p = 0.05). Tractography determined improvement in the Karnofsky Performance Scale (50% vs 5% cases improved, p = 0.02). There was a positive association between the use of neuronavigation and overall survival (23 vs. 27.4 months, p = 0.03), but the use of 5-ALA was associated with shorter OS (34.8 vs. 21.1 months, p = 0.01) and PFS (24.4 vs. 11.8, p = 0.01). Conclusions: we demonstrate for the first time that for insular gliomas 5-ALA plays a role in achieving higher EOR, although this technology was associated with poor OS and PFS; Also tractography and neuronavigation can be of great importance in the treatment of insular gliomas as they determined better functionality and overall survival in this study, respectively. Prospective studies with a more prominent sample and proper multivariate analysis will help determine the real benefit of these adjuncts in the setting of insular glioma

Gerstmann's syndrome and unilateral optic ataxia in the emergency department

ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke

Adult Neurogenesis and Glial Oncogenesis: When the Process Fails

Malignant brain tumors, including glioblastoma multiforme (GBM), are known for their high degree of invasiveness, aggressiveness, and lethality. These tumors are made up of heterogeneous cell populations and only a small part of these cells (known as cancer stem cells) is responsible for the initiation and recurrence of the tumor. The biology of cancer stem cells and their role in brain tumor growth and therapeutic resistance has been extensively investigated. Recent work suggests that glial tumors arise from neural stem cells that undergo a defective process of differentiation. The understanding of this process might permit the development of novel treatment strategies targeting cancer stem cells. In the present review, we address the mechanisms underlying glial tumor formation, paying special attention to cancer stem cells and the role of the microenvironment in preserving them and promoting tumor growth. Recent advancements in cancer stem cell biology, especially regarding tumor initiation and resistance to chemo- or radiotherapy, have led to the development of novel treatment strategies that focus on the niche of the stem cells that make up the tumor. Encouraging results from preclinical studies predict that these findings will be translated into the clinical field in the near future

Gerstmann's syndrome and unilateral optic ataxia in the emergency department

<div><p>ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke.</p></div