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BACKGROUND: Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a validated assessment designed to screen cognitive functions and behavioral disorders in amyotrophic lateral sclerosis (ALS). Objective of this study is to determine the factors associated with ECAS impairment in a cohort of ALS patients without a co-morbid diagnosis of dementia, at the time of diagnosis. METHODS: We enrolled 71 non-demented ALS patient. We collected clinical and demographic data, ALS familiarity, analysis of the most commonly mutated genes in ALS, ALS Milano Torino Staging System and ALS Functional Rate Scale revised scores, progression rate; finally, we recorded whether symptoms onset involved spinal or bulbar area. The alteration of the ECAS was estimated based on age and education-adjusted-validated cut off for each of the items included in ECAS. A multivariable regression analysis was done. RESULTS: The significant determinants of ECAS alterations were: bulbar onset in both ALS-specific test and total ECAS score; bulbar onset and familiarity in ALS-non-specific test; finally, familiarity and diagnosis delay in ALS-behavioral test. All the subjects carrying C9orf72 mutations had alteration of both total ECAS score and ALS-specific tests. DISCUSSION: At diagnosis, bulbar-onset ALS, family history, diagnosis delay and C9orf72 hexanucleotide repeat expansion may contribute to impairment of ECAS.
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CONTEXT AND OBJECTIVE Acute coronary syndromes (ACS) are a common cause of intensive care unit (ICU) admission. Specific prognostic scores have been developed and validated for ACS patients and, among them, GRACE (Global Registry of Acute Coronary Events) has had the best performance. However, intensive care clinicians generally use prognostic scores developed from heterogeneous populations of critically ill patients, such as APACHE IV (Acute Physiologic and Chronic Health Evaluation IV) and SAPS 3 (Simplified Acute Physiology Score 3). The aim of this study was to evaluate and compare the performance of these three scores in a non-selected population of ACS cases. DESIGN AND SETTING Retrospective observational study to evaluate three prognostic scores in a population of ACS patients admitted to three general ICUs in private hospitals in São Paulo. METHODS All patients with ACS admitted from July 2008 to December 2009 were considered for inclusion in the study. Score calibration and discrimination were evaluated in relation to predicting hospital mortality. RESULTS A total of 1065 patients were included. The calibration was appropriate for APACHE IV and GRACE but not for SAPS 3. The discrimination was very good for all scores (area under curve of 0.862 for GRACE, 0.860 for APACHE IV and 0.804 for SAPS 3). CONCLUSIONS In this population of ACS patients admitted to ICUs, GRACE and APACHE IV were adequately calibrated, but SAPS 3 was not. All three scores had very good discrimination. GRACE and APACHE IV may be used for predicting mortality risk among ACS patients. .
CONTEXTO E OBJETIVO Síndromes coronarianas agudas (SCA) são causa comum de admissão à unidade de terapia intensiva (UTI). Escores prognósticos específicos foram desenvolvidos e validados para pacientes com SCA e, dentre esses, o GRACE (Registro Global de Eventos Coronarianos Agudos) tem tido a melhor performance. No entanto, os intensivistas normalmente usam escores desenvolvidos para populações heterogêneas de pacientes graves, como o APACHE IV (Avaliação de Saúde Crônica e Fisiologia Aguda IV) e o SAPS 3 (Escore Fisiológico Agudo Simplificado 3). O presente estudo objetiva avaliar e comparar a performance desses três escores em uma população não selecionada admitida com diagnóstico de SCA. TIPO DE ESTUDO E LOCAL Estudo retrospectivo observacional para a avaliação de três escores prognósticos em uma população admitida com SCA em três UTIs gerais de hospitais particulares em São Paulo. MÉTODOS Todos os pacientes admitidos com SCA de julho de 2008 a dezembro de 2009 foram avaliados para inclusão no estudo. Foram avaliadas a calibração e a discriminação dos escores em predizer a mortalidade hospitalar. RESULTADOS Um total de 1.065 pacientes foi incluído. A calibração foi adequada para o APACHE IV e para o GRACE, mas não para o SAPS 3. A discriminação foi muito boa para todos os escores (área sob a curva de 0,862; 0,860 e 0,804 para GRACE, APACHE IV e SAPS 3). CONCLUSÕES Nesta população de pacientes com SCA admitidos à UTI, os escores GRACE e APACHE IV apresentaram uma calibração adequada, mas o SAPS 3 não. Todos os escores tiveram uma discriminação muito boa. O GRACE e o APACHE IV podem ser usados para pre...