ABSTRACT Rowland Universal Dementia Assessment Scale (RUDAS) is a cognitive screening that evaluates older people with low educational levels. In Chile, there are no normative data to assess this population. Objective: To obtain normative data on RUDAS in older Chilean people with up to 12 years of schooling, and to determine whether age and schooling years influence a person’s performance on RUDAS and on the items that constitute it. Methods: A group of cognitively healthy people 60 years old or over, with up to 12 schooling years was evaluated (n=135). Multiple regression models were applied to obtain normative data on RUDAS, according to age and schooling years, and to measure the effects of schooling on different items. Results: Regression analysis showed that none of the items had schooling as a significant predictor, except for the visuoconstruction item. The variables age and schooling explained 12.6% (R^2=0.126) of the RUDAS total score variance. The item visuoconstruction was the most associated with the educational level (OR=1,147). Conclusion: This study showed that RUDAS is a recommended instrument for evaluating older people with low educational levels. However, more studies are needed to prove the validity of the RUDAS on Chilean older people.

RESUMO RUDAS é uma triagem cognitiva que avalia idosos com baixa escolaridade. No Chile não existem dados normativos para avaliar essa população. Objetivo: Obter dados normativos sobre RUDAS em idosos chilenos com até 12 anos de escolaridade. Além disso, determinar se a idade e a escolaridade influenciam o desempenho de uma pessoa no RUDAS e nos itens que o constituem. Métodos: Foi avaliado um grupo de pessoas cognitivamente saudáveis, com 60 anos ou mais e até 12 anos de escolaridade (n=135). Modelos de regressão múltipla foram utilizados para obter dados normativos do RUDAS, segundo idade e anos de escolaridade, e para mensurar os efeitos da escolaridade em diferentes itens. Resultados: A análise de regressão mostrou que nenhum dos itens teve a escolaridade como preditor significativo, exceto o item visuoconstrução. As variáveis idade e escolaridade explicam 12,6% (R^2=0,126) da variância do escore total do RUDAS. O item visuoconstrução é o mais associado ao nível de escolaridade (OR=1,147). Conclusão: Este estudo mostrou que o RUDAS é um instrumento recomendado para a avaliação de idosos com baixa escolaridade. No entanto, são necessários mais estudos para comprovar a validade do RUDAS em idosos chilenos.

RESUMO. A Bateria de Avaliação Frontal (FAB) é um teste de rastreio que mede as funções executivas. Embora esse instrumento tenha sido validado em vários países, sua utilidade diagnóstica em uma população chilena ainda não foi estudada. Objetivos: (1) Adaptar a FAB para uma população chilena; (2) estudar as propriedades psicométricas da FAB em uma população chilena; (3) avaliar a influência sociodemográfica no desempenho da FAB em uma amostra de controles saudáveis; e (4) desenvolver dados normativos para este último grupo. Métodos: Um grupo controle saudável (n=344) e um grupo de pacientes com demência (n=156) foram avaliados com a versão chilena da FAB. Resultados: A FAB apresentou boa consistência interna (alfa de Cronbach=0,79) e validade aceitável com base na relação com outras variáveis. A análise fatorial mostrou a unidimensionalidade do instrumento. Diferenças significativas foram encontradas no valor total da FAB entre os grupos controle saudável e demência. Com a amostra pareada, o ponto de corte estabelecido foi de 13,5, que apresentou sensibilidade de 80,8% e especificidade de 90,4%. A análise de regressão mostrou que a escolaridade e a idade predisseram significativamente o desempenho da FAB no grupo saudável. Finalmente, os dados normativos são fornecidos. Conclusões: O presente estudo mostrou que a FAB é uma ferramenta útil para discriminar entre pessoas saudáveis e aquelas com demência. No entanto, mais estudos são necessários para explorar a capacidade do instrumento para caracterizar a síndrome disexecutiva em pessoas com demência na população chilena.

ABSTRACT. The Frontal Assessment Battery (FAB) is a screening test that measures executive functions. Although this instrument has been validated in several countries, its diagnostic utility in a Chilean population has not been studied yet. Objectives: This study aimed to (1) adapt FAB in a Chilean population; (2) study the psychometric properties of the FAB in a Chilean population; (3) assess the sociodemographic influence in the performance of the FAB in a sample of healthy controls (HC); and (4) develop normative data for this healthy group. Methods: A HC (n=344) and a group of patients with dementia (n=156) were assessed with the Chilean version of FAB. Results: FAB showed good internal consistency (Cronbach's alpha=0.79) and acceptable validity based on the relationship with other variables. Factor analysis showed the unidimensionality of the instrument. Significant differences were found in the total FAB value between the HC and dementia groups. With the matched sample, the established cutoff point was 13.5, showing a sensitivity of 80.8% and a specificity of 90.4%. Regression analysis showed that education and age significantly predicted FAB performance in the healthy group. Finally, normative data are provided. Conclusions: This study shows that FAB is a useful tool to discriminate between healthy people and people with dementia. However, further studies are needed to explore the capacity of the instrument to characterize the dysexecutive syndrome in people with dementia in the Chilean population.

RESUMO O Questionário de Inventário Neuropsiquiátrico (NPI-Q) é um instrumento baseado em informantes que mede a presença e a gravidade de 12 Sintomas Neuropsiquiátricos (NPS) em pacientes com demência, bem como o sofrimento do informante. Objetivo: Avaliar as propriedades psicométricas do NPI-Q e a prevalência de NPS em pacientes com doença de Alzheimer (DA). Métodos: Foram avaliados 53 pacientes com DA. Eles foram divididos em dois grupos diferentes: AD leve (n=26) e AD moderado (n=27). A validade convergente foi estimada correlacionando os resultados do NPI-Q com os escores do Inventário Neuropsiquiátrico (NPI) e um teste de eficiência cognitiva global (Addenbrooke's Cognitive Examination - Revised - ACE-R). A confiabilidade do NPI-Q foi analisada pelo cálculo da sua consistência interna. A prevalência de NPS foi estimada com NPI e NPI-Q. Resultados: Foram observadas correlações positivas e significativas entre NPI-Q, NPI e ACE-R (r=0,730; p<0,01 e 0>315; p<0>05). O instrumento apresentou um nível adequado de confiabilidade (alfa de Cronbach=0J83). Os NPS mais prevalentes foram apatia/indiferença (62,3%) e disforia/depressão (58,5%). Conclusão: O NPI-Q apresenta indicadores de validade e confiabilidade aceitáveis em pacientes com DA, o que indica que é um instrumento adequado para a avaliação rotineira de NPS na prática clínica.

ABSTRACT The Neuropsychiatric Inventory Questionnaire (NPI-Q) is an informant-based instrument that measures the presence and severity of 12 Neuropsychiatric Symptoms (NPS) in patients with dementia, as well as informant distress. Objective: To measure the psychometric properties of the NPI-Q and the prevalence of NPS in patients with Alzheimer's disease (AD) in Chile. Methods: 53 patients with AD were assessed. Subjects were divided into two different groups: mild AD (n=26) and moderate AD (n=27). Convergent validity was estimated by correlating the outcomes of the NPI-Q with Neuropsychiatric Inventory (NPI) scores and with a global cognitive efficiency test (Addenbrooke's Cognitive Examination - Revised - ACE-R). Reliability of the NPI-Q was analysed by calculating its internal consistency. Prevalence of NPS was estimated with both the NPI and NPI-Q. Results: Positive and significant correlations were observed between the NPI-Q, the NPI, and the ACE-R (r=0.730; p<0.01 and 0.315; p<0.05 respectively). The instrument displayed an adequate level of reliability (Cronbach's alpha=0.783). The most prevalent NPS were apathy/indifference (62.3%) and dysphoria/depression (58.5%). Conclusion: The NPI-Q exhibited acceptable validity and reliability indicators for patients with AD in Chile, indicating that it is a suitable instrument for the routine assessment of NPS in clinical practice.

Background: The inability to carry out activities of daily living (ADL) is prevalent in elderly people and it is associated with hypertension and stroke. Aim: To evaluate ADLs using the T-ADLQ in hypertensive patients with minor stroke. Subjects and Methods: T-ADLQ, Cognitive tests (Minimental and Addenbrooke), and Hamilton depression test were applied to 100 hypertensive ambulatory patients (55 without symptomatic stroke and 45 with ischemic stroke, Rankin ≤ 2). Results: In stroke patients the ability to perform ADL was significantly reduced compared with hypertensive patients without stroke. Cognitive dysfunction and depressive symptoms were associated with a lower ADL performance. Conclusions: The T-ADLQ is useful to evaluate ADL in hypertensive ambulatory patients with ischemic stroke.

Recent genetic and neuropathologic advances support the concept that frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are overlapping multisystem disorders. While 10-15% of ALS patients fulfil criteria for FTD, features of motor neuron disease appear in approximately 15% of FTD patients, during the evolution of the disease. This overlap has been reinforced by the discovery of Transactive Response DNA Binding Protein 43 kDa (TDP43) inclusions as the main neuropathologic finding in the majority of ALS cases and almost a half of FTD cases. Also, an expansion in the intron of C9ORF72 (chromosome 9p21) has been identified in families affected by ALS, ALS-FTD and FTD. This review provides an update on the recent genetic and neuropathologic findings of ALS and FTD and a characterization of their clinical presentation forms, based on the current diagnostic criteria. Finally it underscores the importance of having a national registry of patients with ALS and FTD, to provide an earlier diagnosis and a multidisciplinary care.

RESUMO Objetivo: Analisar as propriedades psicométricas e utilitário de diagnóstico da versão chilena do rastreio frontal INECO (IFS-Ch) em uma amostra de pacientes com demência e controles. Métodos: Após a adaptação do instrumento para o contexto chileno e obtenção de evidências de validade de conteúdo, o IFS-Ch foi administrado a 31 pacientes com demência e 30 indivíduos do grupo controle, além de outros testes de eficiência cognitiva global e executiva. Cuidadores de pacientes com demência e informantes de indivíduos controles foram entrevistados com instrumentos de medidas de sintomas disexecutivos, gravidade da demência e estado funcional nas atividades da vida diária. Validade convergente e discriminante, consistência interna, pontos de corte, sensibilidade e especificidade para o IFS-Ch foram estimados. Resultados: A evidência de validade de conteúdo foi obtida através de consulta a um especialista. Evidências de validade convergente foram encontrados, bem como, descritas correlações significativas entre o IFS-Ch e outros instrumentos de medidas: de funções executivas (FAB, r=0,935; categorias alcançadas no MCST, r=0,791; erros perseverativos na MCST, r= -0,617; fluência verbal animais, r=0,728; "A" de fluência verbal, r=0,681; gravidade de demência e fluência verbal de "P", r=0,783), sintomas disexecutivos na vida diária (DEX, r= -0,494), (CDR, r= -0,75) e estado funcional nas atividades da vida diária (T-ADLQ, r= -0,745; AIVD, r=0,717). Quanto à confiabilidade, coeficiente alfa de Cronbach de 0,905 foi obtido. Quanto a utilidade de diagnóstico, um ponto de corte de 18 pontos (sensibilidade=0,903, especificidade=0,867) e uma área sob a curva de 0,951 foi estimada para distinguir entre pacientes com demência e sujeitos controles. Discussão: O IFS-Ch mostra propriedades psicométricas aceitáveis, apoiadas por evidências de validade e confiabilidade para sua utilização como medida de funções executivas em pacientes com demência. Sua utilidade diagnóstica para detectar pacientes com demência também é considerada aceitável.

ABSTRACT Objective: This study sought to analyze the psychometric properties and diagnostic accuracy of the Chilean version of the INECO Frontal Screening (IFS-Ch) in a sample of dementia patients and control Methods: After adapting the instrument to the Chilean context and obtaining content validity evidence through expert consultation, the IFS-Ch was administered to 31 dementia patients and 30 control subjects together with other executive assessments (Frontal Assessment Battery [FAB], Modified version of the Wisconsin Card Sorting Test [MCST], phonemic verbal fluencies [letters A and P] and semantic verbal fluency [animals]) and global cognitive efficiency tests (Mini mental State Examination [MMSE] and Addenbrooke's Cognitive Examination-Revised [ACE-R]). Caregivers of dementia patients and proxies of control subjects were interviewed with instruments measuring dysexecutive symptoms (Dysexecutive Questionnaire [DEX]), dementia severity (Clinical Dementia Rating Scale [CDR]) and functional status in activities of daily living (Activities of Daily Living Scale [IADL] and Technology-Activities of Daily Living Questionnaire [T-ADLQ]). Convergent and discriminant validity, internal consistency reliability, cut-off points, sensitivity and specificity for the IFS-Ch were estimated. Results: Evidence of content validity was obtained. Evidence of convergent validity was also found showing significant correlations (p<0.05) between the IFS-Ch and the other instruments measuring: executive functions (FAB, r=0.935; categories achieved in the MCST, r=0.791; perseverative errors in the MCST, r= -0.617; animal verbal fluency, r=0.728; A verbal fluency, r=0.681; and P verbal fluency, r=0.783), dysexecutive symptoms in daily living (DEX, r= -0.494), dementia severity (CDR, r= -0.75) and functional status in activities of daily living (T-ADLQ, r= -0.745; IADL, r=0.717). Regarding reliability, a Cronbach's alpha coefficient of 0.905 was obtained. For diagnostic accuracy, a cut-off point of 18 points (sensitivity=0.903; specificity=0.867) and an area under curve of 0.951 were estimated to distinguish between patients with dementia and control subjects. Discussion: The IFS-Ch showed acceptable psychometric properties, supported by evidence of validity and reliability for its use in the measurement of executive functions in patients with dementia. The diagnostic accuracy of the IFS-Ch for detecting dementia patients was also considered acceptable.

The neuro-scientific study of moral actions and judgments is particularly relevant to medicine, especially when assessing behavior disorders secondary to brain diseases. In this paper, moral behavior is reviewed from an evolutionary and neuro-scientific perspective. We discuss the role of emotions in moral decisions, the role of brain development in moral development and the cerebral basis of moral behavior. Empirical evidence shows a relationship between brain and moral development: changes in cerebral architecture are related to changes in moral decision complexity. Moral development takes a long time, achieving its maturity during adulthood. It is suggested that moral cognition depends on cerebral regions and neural networks related to emotional and cognitive processing (i.e. prefrontal and temporal cortex) and that moral judgments are complex affective and cognitive phenomena. This paper concludes with the suggestion that a satisfactory clinical/legal evaluation of a patient requires that the neural basis of moral behavior should be taken into account.

The role of the frontal lobe in control of behavioral and cognitive abilities is explored in a group of 34 patients with brain lesions restricted to the prefrontal cortex. The scores in both structured behavioral questionnaires and standard neuropsychological tests were analyzed using the injured area of the frontal lobe as the independent variable. Our results show that patients with simultaneous lesions in supero_ and inferomedial areas of the prefrontal cortex exhibit higher behavioral disturbances. Bilateral lesions also are associated with greater behavioral troubles. On the contrary, cognitive abilities are globally impaired in prefrontal patients. Results are discussed in relation to current models of the organization of the prefrontal cortex and its role on behavior control

A 62 year-old patient is presented, without family antecedents of dementia who begins with 45 years of age with sudden crisis of dyspnea, blurred vision, fall to the floor and repetitive jerks of arms. Tried by epilepsy with phenytoin and valproate repeats similar crisis sporadically. Four years ago their relatives began to notice changes of personality, irritability and obsessive behaviors. Later on, are added episodes of disorientation of days of duration, some with auditory hallucinations and also convulsive manifestations. Finally appear polymorphic crisis, some with continue alteration of consciousness, catatonic states and sphincteric incontinence. Physical and neurological examination without abnormalities. Neuropsychological evaluation evidenced consistent defects in frontal functions. EEG showed widespread slowness and sporadic irritative activity in frontotemporal regions. Anterior cortical atrophy in CTscan and bilateral frontotemporal hypoperfusion in SPECT. Labs exams and CSF were normal. CONCLUSION: The association of FTD and epilepsy, in non-family form, suggests a different neurodegenerative cortical syndrome.

Se presenta un paciente de 62 años, sin antecedentes familiares de demencia, que a los 45 años debuta con crisis súbitas de disnea, visión borrosa, caída al suelo y movimientos repetitivos de brazos. Tratado por epilepsia con fenitoína y ácido valproico, repite esporádicamente crisis semejantes. Hace 4 años sus familiares notan cambios de personalidad, irritabilidad y conductas obsesivas. Hace 2 años aparecen episodios de desorientación de días de duración, algunos con alucinaciones auditivas y también fenómenos convulsivos. Ultimamente presenta crisis polimorfas, algunas con prolongada alteración de conciencia, estados catatoniformes y relajación esfinteriana. Examen físico y neurológico sin anormalidades. Evaluación neuropsicológica evidenció consistentes defectos en funciones frontales. EEG mostraron lentitud generalizada y actividad irritativa esporádica en regiones frontotemporales. Atrofia cortical de predominio anterior en CT scan e hipoperfusión fronto-temporal bilateral en SPECT. Exámenes de laboratorio y LCR normales. CONCLUSIÓN: La asociación de DFT con epilepsia, en forma no familiar, sugiere un síndrome neurodegenerativo cortical diferente.