Validación argentino-chilena de la versión en español del test Addenbrooke's Cognitive Examination III para el diagnóstico de demencia / Argentinian/Chilean validation of the Spanish-language version of Addenbrooke's Cognitive Examination III for diagnosing dementia

INTRODUCCIÓN: El Addenbrooke's Cognitive Examination III (ACE-III) es una adaptación del test de cribado ACE, la cual ha demostrado tener una alta sensibilidad y especificidad para detectar disfunción cognitiva en pacientes con demencia y otras patologías neurológicas y psiquiátricas. Si bien el ACE-III ya ha sido validado en castellano (España), este no ha sido validado en Latinoamérica. El objetivo del presente estudio fue validar el ACE-III en una población argentina y chilena. MÉTODOS: Se evaluó un grupo de pacientes con enfermedad de Alzheimer (n = 70), un grupo de pacientes con la variante conductual de la demencia frontotemporal (n = 31) y un grupo control (n = 139) con la versión en español del ACE-III; reclutados en centros médicos de ambos países. RESULTADOS: La versión argentina-chilena del ACE-III presentó una buena consistencia interna (alfa de Cronbach = 0,87). Se hallaron diferencias significativas en los valores totales del ACE-III entre el grupo control y el grupo de demencias (p < 0,05) y entre ambos grupos de demencia (p < 0,05). Con un punto de corte de 86, el 98,6% de los pacientes con DTA, el 83,9% de pacientes con variante conductual de la demencia frontotemporal y el 84,2% de los controles fue correctamente clasificado. CONCLUSIONES: El presente estudio ha demostrado que el ACE-III continúa siendo una herramienta útil para la detección de la disfunción cognitiva en la demencia

BACKGROUND: The Addenbrooke's Cognitive Examination III (ACE-III), an adaptation of the ACE cognitive screening test, has been demonstrated to have high sensitivity and specificity in detecting cognitive impairment in patients with dementia and other neurological and psychiatric disorders. Although the Spanish-language version of the ACE-III has already been validated in Spain, it is yet to be validated in Latin America. The aim of this study was to validate the ACE-III test in an Argentinean and Chilean population. METHODS: ACE-III was administered to 70 patients with Alzheimer disease, 31 patients with behavioural variant frontotemporal dementia, and a control group of 139 healthy volunteers. Participants were recruited at centres in both countries. RESULTS: The Spanish-language version of ACE-III was found to have good internal consistency (Cronbach's alpha = 0.87). We found significant differences in total ACE-III scores between patients with Alzheimer disease and controls (p < .05) and between patients with Alzheimer disease and bvFTD (p < .05). With a cut-off point of 86, 98.6% of AD patients, 83.9% of behavioural variant frontotemporal dementia patients, and 84.2% of controls were correctly classified. CONCLUSIONS: This study shows that the Spanish-language version of ACE-III continues to be an effective tool for detecting cognitive dysfunction in patients with dementia

Argentinian/Chilean validation of the Spanish-language version of Addenbrooke's Cognitive Examination III for diagnosing dementia. / Validación argentino-chilena de la versión en español del test Addenbrooke's Cognitive Examination III para el diagnóstico de demencia.

BACKGROUND: The Addenbrooke's Cognitive Examination III (ACE-III), an adaptation of the ACE cognitive screening test, has been demonstrated to have high sensitivity and specificity in detecting cognitive impairment in patients with dementia and other neurological and psychiatric disorders. Although the Spanish-language version of the ACE-III has already been validated in Spain, it is yet to be validated in Latin America. The aim of this study was to validate the ACE-III test in an Argentinean and Chilean population. METHODS: ACE-III was administered to 70 patients with Alzheimer disease, 31 patients with behavioural variant frontotemporal dementia, and a control group of 139 healthy volunteers. Participants were recruited at centres in both countries. RESULTS: The Spanish-language version of ACE-III was found to have good internal consistency (Cronbach's alpha=0.87). We found significant differences in total ACE-III scores between patients with Alzheimer disease and controls (p< .05) and between patients with Alzheimer disease and bvFTD (p< .05). With a cut-off point of 86, 98.6% of AD patients, 83.9% of behavioural variant frontotemporal dementia patients, and 84.2% of controls were correctly classified. CONCLUSIONS: This study shows that the Spanish-language version of ACE-III continues to be an effective tool for detecting cognitive dysfunction in patients with dementia.

Relationship between Social Cognition and traditional cognitive impairment in Progressive Multiple Sclerosis and possible implicated neuroanatomical regions.

BACKGROUND: Cognitive impairment is a relevant contributor of the medical and social burden in Progressive MS. Social Cognition, the neurocognitive processes underlying social interaction, has been explored mainly in European and North American cohorts, influencing social aspects of quality of life (QOL) of early MS patients and families. Few studies have studied Social Cognition in Progressive MS and the literature on its neuroanatomical bases or brain atrophy measurements is still scarce. OBJECTIVES: To explore the relationship between Social Cognition performance and its correlations with traditional cognitive domains, brain atrophy and QOL in primary and secondary Progressive MS patients. METHODS: Cross-sectional analysis including: mini-Social-Cognition-and-Emotional-Assessment (mini-SEA), neuropsychological battery, disability, depression, fatigue, QOL, and brain volume. RESULTS: Forty-three MS patients, 23 primary and 20 secondary Progressive, 65% women, mean age and disease duration of 57.2 and 15.7 years, respectively, with high levels of disability (median EDSS 6.0) and a widespread impairment in traditional domains (mostly episodic verbal/visual and working memories) were assessed. The Mini-SEA score was correlated with executive functions (cognitive shifts Rho:0.55; p = 0.001) analyzing the whole group, and with visual episodic memory (Rho:0.58, p = 0.009) in the primary Progressive MS group. Mini-SEA score was also correlated with total normalized grey matter volume (Rho:0.48; p = 0.004). Particularly, atrophy within bilateral cortical regions of orbitofrontal, insula and cerebellum, and right regions of fusiform gyrus and precuneus were significantly associated with higher Social Cognition impairment. In this cohort, QOL was not correlated with Social Cognition, but with EDSS, fatigue and depression. CONCLUSIONS: In Progressive MS, Social Cognition is directly correlated with traditional cognitive domains such as executive function and episodic memory. It is also associated with global grey matter atrophy and regional atrophy within associative visual and executive cortical areas, but no correlations with QOL were found in this cohort. These findings may contribute to the understanding of the pathological bases behind Social Cognition in Progressive MS.

[Effect of sports training in angular compartment of the lower limbs in children footballers aged 11 to 12 years old]. / Efecto de un entrenamiento deportivo en el compartimiento angular de la extremidad inferior en niños futbolistas de entre 11 y 12 años.

Anterior Cruciate Ligament (ACL) injury is an important cause of days lost in athletes. Most ACL injuries are non-contact and are associated with biomechanical risk factors that increase tension in the ACL: increased knee valgus (KV) and hip flexion (HF) and decreased flexion of knee (KF). Muscle around the knee contributes to knee stability, so fatigue produced by exercise could alter knee balance, increasing LCA tension. The aim of the study is to determine the angular behavior before and after a physical load for CF, RR and RV in children born in 2002-2003. A non-randomized clinical trial was conducted. The sample consisted of 50 students from soccer schools born between 2002 and 2003. The angular behavior of CF, RR and VR was compared, before and after performing standardized training. The angular behavior was measured by performing the DJ test with data obtained by inertial sensors. After exercise, the 3 variables increased, but only HF reached significant difference. Other important finding was the difference found in KV between the dominant leg and the support limb, at both times: rest and post exercise. It was concluded that the angular behavior of CF increases significantly in both limbs post-exercise and that preventive measures should be applied for the management of valgus in the supporting limb.

La lesión del ligamento cruzado anterior (LCA) constituye una causa importante de reposo en deportistas. En su mayoría, las lesiones del LCA no requieren contacto externo y se asocian con factores de riesgo biomecánicos que aumentan la tensión en el LCA: el aumento del ángulo de valgo de rodilla (VR), flexión de cadera (FC) y una menor flexión de rodilla (FR). El LCA requiere cooperación de los grupos musculares perirrodilla, por lo que la fatiga producida por el ejercicio alteraría el balance y pondría en riesgo a este ligamento. El objetivo del estudio es determinar el comportamiento angular antes y después de una carga física para FC, FR y VR en niños nacidos en los años 2002-2003. Se realizó un estudio clínico no aleatorizado. La muestra consistió en 50 alumnos de escuelas de fútbol nacidos en los años 2002 y 2003. Se comparó el comportamiento angular de FC, FR y VR antes y después de realizar un entrenamiento estandarizado. El comportamiento angular fue medido a través de la realización de la prueba DJ, con datos obtenidos por sensores inerciales. Posterior al ejercicio, aumentaron las tres variables; sólo fue significativo el incremento en la flexión de cadera. Por otra parte, destaca la diferencia encontrada tanto en reposo como postejercicios entre la extremidad de apoyo y la hábil en el peak de valgo angular. Se concluyó que el comportamiento angular de FC aumenta significativamente en ambas extremidades postentrenamiento y que se deben aplicar medidas preventivas para el manejo del valgo en la extremidad de apoyo.

Efecto de un entrenamiento deportivo en el compartimiento angular de la extremidad inferior en niños futbolistas de entre 11 y 12 años / Effect of sports training in angular compartment of the lower limbs in children footballers aged 11 to 12 years old

Resumen: La lesión del ligamento cruzado anterior (LCA) constituye una causa importante de reposo en deportistas. En su mayoría, las lesiones del LCA no requieren contacto externo y se asocian con factores de riesgo biomecánicos que aumentan la tensión en el LCA: el aumento del ángulo de valgo de rodilla (VR), flexión de cadera (FC) y una menor flexión de rodilla (FR). El LCA requiere cooperación de los grupos musculares perirrodilla, por lo que la fatiga producida por el ejercicio alteraría el balance y pondría en riesgo a este ligamento. El objetivo del estudio es determinar el comportamiento angular antes y después de una carga física para FC, FR y VR en niños nacidos en los años 2002-2003. Se realizó un estudio clínico no aleatorizado. La muestra consistió en 50 alumnos de escuelas de fútbol nacidos en los años 2002 y 2003. Se comparó el comportamiento angular de FC, FR y VR antes y después de realizar un entrenamiento estandarizado. El comportamiento angular fue medido a través de la realización de la prueba DJ, con datos obtenidos por sensores inerciales. Posterior al ejercicio, aumentaron las tres variables; sólo fue significativo el incremento en la flexión de cadera. Por otra parte, destaca la diferencia encontrada tanto en reposo como postejercicios entre la extremidad de apoyo y la hábil en el peak de valgo angular. Se concluyó que el comportamiento angular de FC aumenta significativamente en ambas extremidades postentrenamiento y que se deben aplicar medidas preventivas para el manejo del valgo en la extremidad de apoyo.

Abstract: Anterior Cruciate Ligament (ACL) injury is an important cause of days lost in athletes. Most ACL injuries are non-contact and are associated with biomechanical risk factors that increase tension in the ACL: increased knee valgus (KV) and hip flexion (HF) and decreased flexion of knee (KF). Muscle around the knee contributes to knee stability, so fatigue produced by exercise could alter knee balance, increasing LCA tension. The aim of the study is to determine the angular behavior before and after a physical load for CF, RR and RV in children born in 2002-2003. A non-randomized clinical trial was conducted. The sample consisted of 50 students from soccer schools born between 2002 and 2003. The angular behavior of CF, RR and VR was compared, before and after performing standardized training. The angular behavior was measured by performing the DJ test with data obtained by inertial sensors. After exercise, the 3 variables increased, but only HF reached significant difference. Other important finding was the difference found in KV between the dominant leg and the support limb, at both times: rest and post exercise. It was concluded that the angular behavior of CF increases significantly in both limbs post-exercise and that preventive measures should be applied for the management of valgus in the supporting limb.

Indicadores clínicos precoces de los trastornos generalizados del desarrollo / Early clinical indicators of pervasive development disorders

Introducción: Los trastornos generalizados del desarrollo (TGD) son procesos caracterizados por presentar déficits en múltiples áreas de la conducta. El resultado son alteraciones en la interacción social, la comunicación verbal y no verbal, y la presencia de intereses restrictivos y repetitivos. La importancia de su diagnóstico precoz radica en la posibilidad de la intervención temprana y su efecto en el pronóstico del paciente. Como el diagnóstico de TGD se ha incrementado en los últimos años, se plantea la detección de situaciones clínicas de fácil identificación en el primer año de vida que permitan mejorar el diagnóstico y facilitar la intervención temprana. Pacientes e intervenciones: Se estudia a 37 pacientes con TGD y 69 controles sanos de forma ambispectiva, a los que se les practica somatometría básica, exploraciones neurológicas y recogida de datos de variables del cuestionario modificado para la detección de riesgo de autismo(MCHAT). Resultados: La proporción de varones (OR: 3,87; IC del 95%, 1,23-12,96), el retraso en los primeros bisílabos (TGD = 20,1±23 meses vs control = 10,8±10,3 meses; p < 0,01), la ausencia de contacto visual (OR: 0,05; IC del 95%, 0,01-0,29), la falta de respuesta a la llamada de atención (OR: 0,12; IC del 95%, 0,02-0,67) y el aumento de «berrinches» (OR: 6,37; IC del 95%,2,39-17,34) fueron significativamente superiores en el grupo con TGD. Conclusiones: Las diferencias que hemos detectado entre ambos grupos no pueden constituir un sistema diagnóstico de certeza en este periodo; sin embargo, creemos que deben ser considerados signos de alarma en el contexto de retrasos madurativos de lactantes y preescolares, a la vez que sugieren la existencia de un fenotipo precoz de TGD(AU)

Introduction: Pervasive development disorders (PDD) conditions characterised by deficits in many areas of behaviour, such as delay in social interactions, abnormalities in verbal and non-verbal communication, and the presence of the restrictive and repetitive interests. The relevance of early diagnosis is based on the fact that early intervention could have a beneficial effect on the long term outcome. Due to the increase of the PDD diagnosis in the recent years, we aimed to study easily detectable clinical traits during the first year of life, leading to an improvement in the diagnosis. Patients and interventions: A prospective and retrospective study was conducted on 37 PDD patients and 69 healthy controls. Somatometric and neurological examinations were performed and a questionnaire with several variables from the Modified Checklist for Autism in Toddlers (M-CHAT) completed by the parents. Results: The male to female ratio (OR: 3.87; 95% CI: 1.23-12.96), delay in the first disyllabic words (PDD = 20.1±23 months vs Control = 10.8±10.3 months; P < 0.01), absence of visual contact (OR: 0.05; 95% CI: 0.01-0.29), the lack of response to attention call (OR: 0.12; 95%CI: 0.02-0.67), and the increase in tantrums (OR: 6.37; 95% CI: 2.39-17.34), were significantly higher in the PDD group. Conclusions: The differences detected between groups can not been considered as a diagnostic tool of certainty in this time period, however, we believe that they should be considered in the context of maturational delay, as alarm signs in infants and toddlers, as well as suggesting the existence of an early pervasive development phenotype(AU)

[Early clinical indicators of pervasive development disorders]. / Indicadores clínicos precoces de los trastornos generalizados del desarrollo.

INTRODUCTION: Pervasive development disorders (PDD) conditions characterised by deficits in many areas of behaviour, such as delay in social interactions, abnormalities in verbal and non-verbal communication, and the presence of the restrictive and repetitive interests. The relevance of early diagnosis is based on the fact that early intervention could have a beneficial effect on the long term outcome. Due to the increase of the PDD diagnosis in the recent years, we aimed to study easily detectable clinical traits during the first year of life, leading to an improvement in the diagnosis. PATIENTS AND INTERVENTIONS: A prospective and retrospective study was conducted on 37 PDD patients and 69 healthy controls. Somatometric and neurological examinations were performed and a questionnaire with several variables from the Modified Checklist for Autism in Toddlers (M-CHAT) completed by the parents. RESULTS: The male to female ratio (OR: 3.87; 95% CI: 1.23-12.96), delay in the first disyllabic words (PDD=20.1±23 months vs Control=10.8±10.3 months; P<.01), absence of visual contact (OR: 0.05; 95% CI: 0.01-0.29), the lack of response to attention call (OR: 0.12; 95% CI: 0.02-0.67), and the increase in tantrums (OR: 6.37; 95% CI: 2.39-17.34), were significantly higher in the PDD group. CONCLUSIONS: The differences detected between groups can not been considered as a diagnostic tool of certainty in this time period, however, we believe that they should be considered in the context of maturational delay, as alarm signs in infants and toddlers, as well as suggesting the existence of an early pervasive development phenotype.

Activities of daily living in motor neuron disease: role of behavioural and motor changes.

Impairment in the activities of daily living (ADL) in motor neuron disease (MND) has been little investigated. The contributions of both behavioural and motor changes on functional performance have not been explored. A postal survey in New South Wales, Australia, included assessments of ADL, behavioural change (carer-based) and MND severity. Eighty-two patients were subdivided into groups according to onset presentation: bulbar (n=23) and limb (n=59). There were significant differences in ADL performance between limb and bulbar onset groups depending on ADL task. Disability was also dependent on disease severity as measured by the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS - R) score. Importantly, variance in ADL scores was dependent on both motor and behavioural factors. This study confirms the progressive disabling nature of MND, which is dependent on disease severity and shows qualitative differences depending on onset presentation. A model combining motor and behavioural changes explained 57% of variance on ADL performance, with important implications for clinical intervention.

Determinants of survival in behavioral variant frontotemporal dementia.

BACKGROUND: Behavioral variant frontotemporal dementia (bvFTD) is a common cause of younger onset dementia. Little is known about its rate of progression but a recently identified subgroup seems to have an excellent prognosis. Other determinants of survival are unclear. METHODS: We analyzed survival in a large group of clinically diagnosed bvFTD patients (n = 91) with particular attention to demographic and clinical features at presentation. Of the 91 cases, 50 have died, with pathologic confirmation in 28. RESULTS: Median survival in the whole group was 9.0 years from symptom onset, and 5.4 years from diagnosis. After the exclusion of 24 "phenocopy" cases, the analysis was repeated in a subgroup of 67 patients. The mean age at symptom onset of the pathologic group was 58.5 years and 16% had a positive family history. Their median survival was 7.6 years (95% confidence interval [CI] 6.6-8.6) from symptom onset and 4.2 years (95% CI 3.4-5.0) from diagnosis. The only factor associated with shorter survival was the presence of language impairment at diagnosis. CONCLUSIONS: Patients with definite frontotemporal dementia have a poor prognosis which is worse if language deficits are also present. This contrasts with the extremely good outcome in those with the phenocopy syndrome: of our 24 patients only 1 has died (of coincident pathology) despite, in some cases, many years of follow-up.

Tryptophan fluorescence of mitochondrial uncoupling protein.

Mitochondrial uncoupling protein (UcP) contains two tryptophans buried in transmembrane alpha-helices: Trp-173 at the matrix end of fourth alpha-helix and Trp-280 on the sixth alpha-helix. However, the steady-state emission of isolated UcP exhibited properties unusual for alpha-helices: maximum close to that of free tryptophan emission and low quantum yield of 0.04. The former suggests prevailing tryptophan contacts with hydrophilic residues and confirms Trp-173 proximity to the water/membrane interface and Trp-280 location near a water-filled nucleotide-binding-site cavity. The latter might indicate that transmembrane segments are not true alpha-helices. Measured depolarization factor of 0.6 suggests also their "breathing". Analysis of UcP emission decays, measured by time-correlated-single-photon-counting, yielded components 0.4-0.6 ns, 2.2-3 ns and 9-10 ns (or alternatively 0.1, 1.5, 4.3 and 12.2 ns; or 0.1-0.3, 1.2, 3.7 and 10.5 ns), very similar to those of free tryptophan in water, where the longest component belongs to anionic form. Hence, such an "anionic" conformation must exist in UcP, perhaps as a consequence of charge-transfer complexes between Trp-173 & Lys-174 and Trp-280 & Arg-276. Moreover, N-ethylmaleimide modification, known to induce conformational changes, prolonged the "10 ns" component, decreased quantum yield to 0.03 without changes in emission spectra, while slightly shifting absorption to red and increasing tyrosine exposure to water.