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INTRODUCTION: Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD) are frequent causes of dementia and, therefore, instruments for differential diagnosis between these two conditions are of great relevance. OBJECTIVE: To investigate the diagnostic accuracy of Addenbrooke's Cognitive Examination-Revised (ACE-R) for differentiating AD from bvFTD in a Brazilian sample. METHODS: The ACE-R was administered to 102 patients who had been diagnosed with mild dementia due to probable AD, 37 with mild bvFTD and 161 cognitively healthy controls, matched according to age and education. Additionally, all subjects were assessed using the Mattis Dementia Rating Scale and the Neuropsychiatric Inventory. The performance of patients and controls was compared by using univariate analysis, and ROC curves were calculated to investigate the accuracy of ACE-R for differentiating AD from bvFTD and for differentiating AD and bvFTD from controls. The verbal fluency plus language to orientation plus name and address delayed recall memory (VLOM) ratio was also calculated. RESULTS: The optimum cutoff scores for ACE-R were <80 for AD, <79 for bvFTD, and <80 for dementia (AD + bvFTD), with area under the receiver operating characteristic curves (ROC) (AUC) >0.85. For the differential diagnosis between AD and bvFTD, a VLOM ratio of 3.05 showed an AUC of 0.816 (Cohen's d = 1.151; p < .001), with 86.5% sensitivity, 71.4% specificity, 72.7% positive predictive value, and 85.7% negative predictive value. CONCLUSIONS: The Brazilian ACE-R achieved a good diagnostic accuracy for differentiating AD from bvFTD patients and for differentiating AD and bvFTD from the controls in the present sample.

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ABSTRACT Objective: To determine the diagnostic accuracy of the Addenbrooke's Cognitive Examination Revised (ACE-R) as a cognitive screening tool for older adults with low levels of schooling and healthy aging, MCI and dementia in Brazil. Methods: All participants underwent neurological and psychiatric examinations and were administered a validated version of ACE-R. Results: A total of 85 participants were evaluated; most were females (84.7%, n = 72). The post hoc analysis showed statistical differences in ACE-R total scores between older adults with mild cognitive impairment (MCI) and controls (p < 0.001) and in subitem scores including verbal fluency, language, visuospatial skills and attention (p < 0.001). The visual-spatial skills subitem was the most strongly correlated with schooling level (r = 0.509, p < 0.001), whereas late, immediate recall and recognition memory were not influenced by schooling. The ACE-R had the best diagnostic accuracy in discriminating between MCI and controls = 0.69 (<57.5; 80/66), MD and controls = 0.98 (<50; 100/96), MCI and MD = 0.86 (<49.5; 100/74). Conclusions: ACE-R and Mini-Mental State Examination (MMSE) scores for older adults with MCI and controls were significantly lower than those reported in similar studies. These preliminary findings support the need for establishing reliable cut-off scores for cognitive assessment of older Brazilian adults with low schooling at risk for dementia taking into consideration ecological and local variables.

RESUMO Objetivo: Determinar a precisão diagnóstica do Exame Cognitivo de Addenbrooke (ACE-R) como uma ferramenta de triagem cognitiva para adultos idosos com baixos níveis de educação e envelhecimento saudável, MCI e demência no Brasil. Métodos: Os indivíduos submeteram-se à avaliação clínica e psiquiátrica e foi administrada uma versão validada da versão revisada da bateria cognitiva ACE-R (ACE-R). Resultados: Oitenta e cinco indivíduos foram avaliados, predominando as mulheres (84,7%, n = 72). Na análise post hoc, controles e CCL exibiram diferenças estatísticas nos escores globais do ACE-R (p < 0,001) e seus subdomínios, incluindo fluência verbal, linguagem, habilidades visuoespaciais e atenção (p < 0,001). A habilidade visuoespacial foi o item mais correlacionado com a escolaridade (r = 0,509, p < 0,001), enquanto a memória tardia, de recordação e reconhecimento não foi influenciada pela educação. A precisão do ACE-R produziu melhores resultados para CCL versus controles = 0,69 (<57,5; 80/66), demência versus controles = 0,98 (<50; 100/96), CCL versus demência = 0,86 (<49,5; 100/74). Conclusões: Os escores de ACE-R e MMSE para controles e CCL foram consideravelmente inferiores aos encontrados em estudos semelhantes. Resultados preliminares confirmam a necessidade de estudos brasileiros estabelecerem pontos de corte confiáveis para baterias cognitivas em idosos com baixa escolaridade e em risco de demência, reconhecendo variáveis ecológicas e regionais.

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INTRODUCCIÓN Las demencias son una de las patologías más frecuentes en neurología. El Addenbrooke's Cognitive Examination (ACE) es una batería breve de cribado en demencias. La validación en Argentina se ha realizado en una población de alto nivel educativo, lo que es una limitación para su aplicación. OBJETIVOS Analizar las propiedades psicométricas del ACE en una muestra de adultos mayores de bajo nivel socioeducativo (NSE) con y sin demencia. MÉTODOS Se revisaron en forma retrospectiva historias clínicas de pacientes evaluados en el Grupo de Apoyo contra el Mal de Alzheimer (GAMA) durante los últimos 4 años. A ellos se les realizó un examen neurocognitivo completo, que incluyó evaluación clínica/neurológica y estudio por imágenes. Se evaluó en forma prospectiva a 35 participantes sin patología neurológica, que conformaron el grupo normativo. Se les administró el ACE, el Cuestionario de Ansiedad de Hamilton y el Inventario de Depresión de Beck II. El NSE de los participantes se calculó utilizando el Índice de Hollingshead. La muestra quedó conformada por 82 participantes (31 con demencia, 26 con DCL, 25 del grupo normativo). RESULTADOS Se observó una aceptable consistencia interna (alfa de Cronbach 0,68). La validez concurrente con el CDR demostró ser alta (r=-0,66; p<0,001). Se realizó un análisis de curva ROC, con un área bajo la curva de 0,930 (IC95%: 0,869-0,992). Una puntuación de 70 del ACE como punto de corte presentó una sensibilidad del 84% y una especificidad del 80%. Discusión En este trabajo se determinaron las propiedades psicométricas del ACE en una población de bajo NSE. Se recomienda un punto de corte de 70, con una aceptable sensibilidad y especificidad. Los resultados obtenidos podrían ser utilizados en pacientes de los estratos sociales más bajos, lo cual es más frecuente en el subsector público de salud de Argentina.

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SPG11 mutations are the major cause of autosomal recessive Hereditary Spastic Paraplegia. The disease has a wide phenotypic variability indicating many regions of the nervous system besides the corticospinal tract are affected. Despite this, anatomical and phenotypic characterization is restricted. In the present study, we investigate the anatomical abnormalities related to SPG11 mutations and how they relate to clinical and cognitive measures. Moreover, we aim to depict how the disease course influences the regions affected, unraveling different susceptibility of specific neuronal populations. We performed clinical and paraclinical studies encompassing neuropsychological, neuroimaging, and neurophysiological tools in a cohort of twenty-five patients and age matched controls. We assessed cortical thickness (FreeSurfer software), deep grey matter volumes (T1-MultiAtlas tool), white matter microstructural damage (DTI-MultiAtlas) and spinal cord morphometry (Spineseg software) on a 3 T MRI scan. Mean age and disease duration were 29 and 13.2 years respectively. Sixty-four percent of the patients were wheelchair bound while 84% were demented. We were able to unfold a diffuse pattern of white matter integrity loss as well as basal ganglia and spinal cord atrophy. Such findings contrasted with a restricted pattern of cortical thinning (motor, limbic and parietal cortices). Electromyography revealed motor neuronopathy affecting 96% of the probands. Correlations with disease duration pointed towards a progressive degeneration of multiple grey matter structures and spinal cord, but not of the white matter. SPG11-related hereditary spastic paraplegia is characterized by selective neuronal vulnerability, in which a precocious and widespread white matter involvement is later followed by a restricted but clearly progressive grey matter degeneration.

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ABSTRACT. There is limited evidence about the progression of cognitive performance during the post-stroke stage. Objective: To assess the evolution of cognitive performance in stroke patients without vascular cognitive impairment (VCI), patients with vascular mild cognitive impairment (MCI), and patients with vascular dementia (VD). Methods: A prospective cohort of stroke outpatients from two secondary medical centers in Lima, Peru was studied. We performed standardized evaluations at definitive diagnosis (baseline evaluation), and control follow-ups at 6 and 12 months, including a battery of short cognitive tests: Clinical Dementia Rating (CDR), Addenbrooke's Cognitive Examination (ACE), and INECO Frontal Screening (IFS). Results: 152 outpatients completed the follow-up, showing progressive increase in mean score on the CDR(0.34 to 0.46), contrary to the pattern observed on the ACE and IFS (78.18 to 76.48 and 23.63 to 22.24). The box plot for the CDR test showed that VCI patients had progressive worsening (0.79 to 0.16). Conversely, this trend was not observed in subjects without VCI. The box plot for the ACE and IFS showed that, for the majority of the differentiated stroke types, both non-VCI and VCI patients had progressive worsening. Conclusion: According to both ACE and IFS results during a 1-year follow-up, the cognitive performance of stroke patients worsened, a trend which was particularly consistent in infarction-type stroke patients.

RESUMO. Há evidências limitadas sobre a progressão do desempenho cognitivo durante o estágio pós- acidente vascular cerebral (AVC). Objetivo: Avaliar a evolução do desempenho cognitivo em pacientes com AVC sem comprometimento cognitivo vascular (SCCV), pacientes com comprometimento cognitivo leve vascular (CCL-V) e pacientes com demência vascular (DV). Métodos: Coorte prospectivo de pacientes ambulatoriais com AVC de dois centros médicos secundários de Lima, Peru. Realizamos avaliações padronizadas no diagnóstico definitivo (avaliação inicial) e controles aos 6 e 12 meses depois, incluindo um conjunto de testes cognitivos breves: Clinical Dementia Rating (CDR), Addenbrooke's Cognitive Examination (ACE), and INECO Frontal Screening (IFS). Resultados: Completaram o estudo 152 pacientes ambulatoriais, mostrando que as médias de pontuação da CDR apresentaram aumento progressivo (0,34 a 0,46), contrariamente ao observado com ACE e IFS (78,18 a 76,48 e 23,63 a 22,24). A soma das caixas do teste CDR mostrou que os pacientes com comprometimento cognitivo vascular (CCL-V e DV) apresentaram piora progressiva (0,79 a 0,16). Por outro lado, em indivíduos SCCV, essa tendência não foi observada. O gráfico box-plot para ACE e IFS mostrou que, para a maioria dos tipos de AVC observados, tanto os pacientes SCCV como aqueles com CCV apresentaram piora progressiva. Conclusão: De acordo com os resultados de ACE e IFS durante o acompanhamento de 1 ano, o desempenho cognitivo em pacientes com AVC piora, o que é particularmente consistente em pacientes com AVC tipo infarto.

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BACKGROUND/AIMS: The diagnostic accuracy of the Cuban version of the revised Addenbrooke's Cognitive Examination (ACE-R) in identifying mild cognitive impairment (MCI) in comparison with the Mini-Mental State Examination (MMSE) was assessed. METHODS: The Cuban ACE-R was administered to a group of 129 elderly subjects (92 cognitively healthy and 37 subjects with MCI). The t tests for independent samples were used to compare scores of different psychometric scales between groups, and effect sizes (Cohen's d) were calculated. Cronbach's coefficient α was used to evaluate the reliability of psychometric scales. The validity of ACE-R to screen for MCI was assessed by receiver operating characteristic (ROC) curves. RESULTS: The Cuban ACE-R had reliable internal consistency (Cronbach's coefficient α = 0. 879). The optimal cut-off score for ACE-R for detecting MCI was 84/85. The sensitivity and specificity of ACE-R to screen for MCI was superior to those of MMSE. The area under the ROC curve of the Cuban ACE-R was much larger than that of MMSE (0.93 and 0.63) for detecting MCI. CONCLUSION: The Cuban ACE-R is a valid screening tool for detecting cognitive impairment. It is more sensitive and accurate in screening for MCI than MMSE.

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There is limited evidence about the progression of cognitive performance during the post-stroke stage. OBJECTIVE: To assess the evolution of cognitive performance in stroke patients without vascular cognitive impairment (VCI), patients with vascular mild cognitive impairment (MCI), and patients with vascular dementia (VD). METHODS: A prospective cohort of stroke outpatients from two secondary medical centers in Lima, Peru was studied. We performed standardized evaluations at definitive diagnosis (baseline evaluation), and control follow-ups at 6 and 12 months, including a battery of short cognitive tests: Clinical Dementia Rating (CDR), Addenbrooke's Cognitive Examination (ACE), and INECO Frontal Screening (IFS). RESULTS: 152 outpatients completed the follow-up, showing progressive increase in mean score on the CDR(0.34 to 0.46), contrary to the pattern observed on the ACE and IFS (78.18 to 76.48 and 23.63 to 22.24). The box plot for the CDR test showed that VCI patients had progressive worsening (0.79 to 0.16). Conversely, this trend was not observed in subjects without VCI. The box plot for the ACE and IFS showed that, for the majority of the differentiated stroke types, both non-VCI and VCI patients had progressive worsening. CONCLUSION: According to both ACE and IFS results during a 1-year follow-up, the cognitive performance of stroke patients worsened, a trend which was particularly consistent in infarction-type stroke patients.

Há evidências limitadas sobre a progressão do desempenho cognitivo durante o estágio pós- acidente vascular cerebral (AVC). OBJETIVO: Avaliar a evolução do desempenho cognitivo em pacientes com AVC sem comprometimento cognitivo vascular (SCCV), pacientes com comprometimento cognitivo leve vascular (CCL-V) e pacientes com demência vascular (DV). MÉTODOS: Coorte prospectivo de pacientes ambulatoriais com AVC de dois centros médicos secundários de Lima, Peru. Realizamos avaliações padronizadas no diagnóstico definitivo (avaliação inicial) e controles aos 6 e 12 meses depois, incluindo um conjunto de testes cognitivos breves: Clinical Dementia Rating (CDR), Addenbrooke's Cognitive Examination (ACE), and INECO Frontal Screening (IFS). RESULTADOS: Completaram o estudo 152 pacientes ambulatoriais, mostrando que as médias de pontuação da CDR apresentaram aumento progressivo (0,34 a 0,46), contrariamente ao observado com ACE e IFS (78,18 a 76,48 e 23,63 a 22,24). A soma das caixas do teste CDR mostrou que os pacientes com comprometimento cognitivo vascular (CCL-V e DV) apresentaram piora progressiva (0,79 a 0,16). Por outro lado, em indivíduos SCCV, essa tendência não foi observada. O gráfico box-plot para ACE e IFS mostrou que, para a maioria dos tipos de AVC observados, tanto os pacientes SCCV como aqueles com CCV apresentaram piora progressiva. CONCLUSÃO: De acordo com os resultados de ACE e IFS durante o acompanhamento de 1 ano, o desempenho cognitivo em pacientes com AVC piora, o que é particularmente consistente em pacientes com AVC tipo infarto.

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BACKGROUND/AIMS: Dementia and depression are different clinical conditions, but share common features, and can be indistinguishable in the initial disease stages. We aimed to establish whether the Peruvian version of the Addenbrooke's Cognitive Examination (ACE-Peru) can distinguish between the cognitive profile in patients with incipient dementia and that in patients with depression. METHODS: This was a cross-sectional study to assess the performance on the ACE-Peru of 193 elderly subjects (102 with dementia, 21 with depression, and 70 healthy controls). Depending on the diagnosis, there were two groups of cognitive impairment (CI) - the primary neurodegenerative (PN-CI) subtype and the secondary to depression (SD-CI) subtype - as well as a non-CI group. The area under the curve (AUC) of the receiver-operating characteristic curve was determined to compare the diagnostic performance, using the diagnosis of CI as the gold standard. RESULTS: In our sample of elderly subjects aged 59-82 years with at least 7 years of education, the ACE-Peru showed a significantly better performance than the MMSE (AUC = 0.997 vs. AUC = 0.887; p < 0.05) for the discrimination between PN-CI and SD-CI. CONCLUSIONS: The ACE-Peru is able to distinguish between the cognitive profile in patients with incipient dementia and that in patients with depression.

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Detecting cognitive impairment in patients with Parkinson's disease is crucial for good clinical practice given the new therapeutic possibilities available. When full neuropsychological evaluations are not available, screening tools capable of detecting cognitive difficulties become crucial. OBJECTIVE: The goal of this study was to investigate whether the Spanish version of the Addenbrooke's Cognitive Examination (ACE) is capable of detecting cognitive difficulties in patients with Parkinson's disease and discriminating their cognitive profile from patients with dementia. METHODS: 77 early dementia patients (53 with Alzheimer's Disease and 24 with Frontotemporal Dementia), 22 patients with Parkinson's disease, and 53 healthy controls were evaluated with the ACE. RESULTS: Parkinson's disease patients significantly differed from both healthy controls and dementia patients on ACE total score. CONCLUSIONS: This study shows that the Spanish version of the ACE is capable of detecting patients with cognitive impairment in Parkinson's disease and is able to differentiate them from patients with dementia based on their general cognitive status.

A detecção de comprometimento cognitivo em pacientes com doença de Parkinson é crucial para uma boa prática clínica devido às novas possibilidades terapêuticas disponíveis. Quando uma avaliação neuropsicológica completa não está disponível, instrumentos de rastreio capazes de detectar dificuldades cognitivas tornam-se cruciais. OBJETIVO: Investigar se a versão espanhola do Addenbrooke's Cognitive Examination (ACE) é capaz de detectar dificuldades cognitivas em pacientes com doença de Parkinson e discriminar seu perfil cognitivo de pacientes com demência. MÉTODOS: 77 pacientes com demência leve (53 com doença de Alzheimer e 24 com demência frontotemporal), 22 pacientes com doença de Parkinson e 53 controles saudáveis foram avaliados com a ACE. RESULTADOS: Os pacientes com doença de Parkinson significativamente diferiram de controles saudáveis e pacientes com demência no escore total do ACE. CONCLUSÕES: Este estudo mostra que a versão espanhola do ACE é capaz de detectar pacientes com comprometimento cognitivo na doença de Parkinson e de diferenciá-los de pacientes com demência baseados no seu estado cognitivo geral.

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The Addenbrooke's Cognitive Examination-Revised (ACE-R) is a highly sensitive and specific tool for the detection of mild dementia. It is particularly useful in differentiating Alzheimer's disease from frontotemporal dementia. While the first version of the test battery has been adapted in many countries, its revised version has not, probably because it was published very recently. OBJECTIVE: To translate and adapt the ACE-R for use in the Brazilian population. METHODS: Two independent translations were made from English into Portuguese, followed by two independent back-translations. Few adaptations in accordance to the Brazilian culture and language were made and a first version of the instrument produced. This former version of the ACE-R was administered to 21 cognitively healthy subjects aged 60 years or more, with different educational levels. RESULTS: The mean age of the studied sample of healthy elderly was 75.4 years (ranging from 60 to 89 years). Small additional modifications were necessary after the evaluation of the first ten subjects in order to improve comprehension of the test. The final Portuguese version of the ACE-R was produced and was found to be well understood by the remaining 11 subjects, taking an average of 15 minutes to be administered. CONCLUSIONS: The Brazilian version of the ACE-R proved to be a promising cognitive instrument for testing both in research and clinical settings. With this regard, additional studies are currently being carried out in our unit in order to investigate the diagnostic properties of the ACE-R in our milieu.

A Addenbrooke's Cognitive Examination - Versão Revisada (ACE-R) é um instrumento com elevada sensibilidade e especificidade para detectar demência em estágio leve. Ela é particularmente útil para diferenciar a doença de Alzheimer da demência frontotemporal. Enquanto a primeira versão desta bateria foi adaptada em vários países, a versão revisada ainda não, provavelmente por ter sido publicada muito recentemente. OBJETIVO: Traduzir e adaptar a ACE-R para uso na população brasileira. MÉTODOS: Foram feitas duas traduções independentes do Inglês para o Português, seguidas de duas retro-traduções também independentes. Algumas adaptações de acordo com a cultura brasileira e a língua portuguesa foram realizadas e a primeira versão brasileira do instrumento produzida. Esta foi administrada a 21 sujeitos saudáveis com idades de 60 anos ou mais, com diferentes níveis de escolaridade. RESULTADOS: A média de idade da amostra de idosos estudada foi de 75,4 anos (variando de 60 a 89 anos de idade). Após a avaliação dos dez primeiros indivíduos foi necessário realizar pequenas alterações com o objetivo de melhorar a compreensão do teste. A versão final em Português da ACE-R foi produzida e se mostrou de fácil entendimento pelos onze participantes restantes, tendo tempo médio de aplicação de 15 minutos. CONCLUSÕES: A versão brasileira da ACE-R provou ser um instrumento promissor de avaliação cognitiva promissor para a pesquisa e para a prática clínica. Estudos adicionais estão sendo realizados em nossa unidade para investigar as propriedades diagnósticas da ACE-R em nosso meio.