Longitudinal Study of Cognitive Functioning in Friedreich's Ataxia.

OBJECTIVE: Friedreich's ataxia (FRDA) is the most common hereditary ataxia. It is a neurodegenerative disorder, characterized by progressive ataxia. FRDA is also associated with cognitive impairments. To date, the evolution of cognitive functioning is unknown. Our aim was to investigate the changes in the cognitive functioning of FRDA patients over an average eight-year timeframe. In addition, we aimed to study the relationship between cognitive changes and clinical variables. METHODS: Twenty-nine FRDA patients who had been part of the sample of a previous study participated in the present study. The mean average time between the two assessments was 8.24 years. The participants completed an extensive battery of neuropsychological tests chosen to examine cognitive functioning in various cognitive domains: processing speed, attention, working memory, executive functions, verbal and visual memory, visuoperceptive and visuospatial skills, visuoconstructive functions and language. RESULTS: At follow-up, cerebellar symptoms had worsened, and patients presented greater disability. Differences between baseline and follow-up were observed in motor and cognitive reaction times, several trials of the Stroop test, semantic fluency, and block designs. No other cognitive changes were observed. Deterioration in simple cognitive reactions times and block designs performance correlated with the progression of cerebellar symptoms. CONCLUSIONS: Our study has demonstrated for the first time that patients with FRDA experience a significant decline over time in several cognitive domains. Specifically, after an eight-year period, FRDA patients worsened in processing speed, fluency, and visuoconstructive skills. This progression is unlikely to be due to greater motor or speech impairment.

Self and hetero-perception and discrimination in Attention Deficit Hyperactivity Disorder.

This study intends to show the external perception that Primary Education students have of their schoolmates with Attention Deficit Hyperactivity Disorder (ADHD) and the perception of the student who has being diagnosed with ADHD himself/herself in order to analyse the differences between both perceptions. For that purpose, a questionnaire was e elaborated ad hoc, and the main results shown point to the fact that the external perception of the Primary Education student is more positive than the perception of the diagnosed student. Moreveover, the perception of the schoolmates of students diagnosed with ADHD tends to worsen in superior courses. This discovery invites us to reflect on and understand the more frequent attitudes and behaviours that develop in interaction situations as well as investigate those conducts related to social prejudice associated to their clinical characteristics and prevalent syntoms (attention déficit, hyperactivity and impulsivity), which develop an increase of the negative perception towards the student with ADHD.

Health-related quality of life and depressive symptoms in Friedreich ataxia.

PURPOSE: Friedreich ataxia (FRDA) is a chronic, progressive and highly disabling cerebellar degenerative disease. Despite this, little attention has been paid to the health-related quality of life (HRQOL) in this disease. The aim of the present study was to assess FRDA patients' perception of HRQOL and to determine the influence of depression, and demographic and clinical variables. METHOD: The sample consisted of 62 patients with genetically confirmed FRDA. The SF-36 Health Survey was used to assess HRQOL. Depressive symptoms were evaluated with the Beck Depression Inventory-II. RESULTS: FRDA patients' mean scores were significantly lower than the values for the Spanish population in all SF36 dimensions. Average z scores ranged from - 5.5 in physical functioning to - 0.48 in mental health. Age and clinical variables were significant predictors of HRQOL in only several dimensions, whereas BDI scores were able to predict a significant percentage of variance in all SF36 dimensions, except physical functioning. CONCLUSIONS: Our study demonstrates the high impact of Friedreich ataxia on quality of life. This impact does not only occur in those aspects most related to motor disability but it is also present in non-motor dimensions. Depressive symptomatology is the most relevant variable for predicting quality of life.

Depressive symptoms in Friedreich ataxia.

Background/Objective: Almost no attention has been paid to depression in Friedreich ataxia (FRDA), a highly disabling cerebellar degenerative disease. Our aim was to study the presence and the profile of depressive symptoms in FRDA and their relationship with demographic-disease variables and cognitive processing speed. Method: The study groups consisted of 57 patients with a diagnosis of FRDA. The Beck Depression Inventory-II was used to assess symptoms of depression. Speed of information processing was measured with a Choice Reaction time task. Results: The mean BDI score for patients was significantly higher than the mean score in the general population. Twenty one percent of participants scored in the moderate/severe range. A Cognitive-Affective score and a Somatic-Motivational score was calculated for each patient. Patients' scores in both dimensions were significantly higher than the scores in the general population. Demographic and disease variables were not related with symptoms of depression, except for severity of ataxia. Depressive symptoms predict cognitive reaction times. The greater proportion of variance was explained by the Cognitive-Affective dimension. Conclusions: Our data show that both somatic-motivational and cognitive affective symptoms of depression are frequent in individuals with FRDA. In addition, depressive symptoms may influence cognition, especially, the cognitive and affective symptoms.

Antecedentes/Objetivo: La depresión en la ataxia de Friedreich (FRDA), una enfermedad degenerativa cerebelosa altamente incapacitante, ha recibido poca atención. Nuestro objetivo es evaluar la presencia y el perfil de los síntomas depresivos en FRDA y su relación con variables clínico-demográficas y la velocidad de procesamiento cognitivo. Método: Se estudiaron 57 pacientes con diagnóstico de FRDA. Se usó el Inventario de Depresión de Beck-II para evaluar los síntomas de depresión. La velocidad de procesamiento se midió con una tarea de tiempos de reacción. Resultados: La puntuación media de los pacientes en el BDI fue significativamente mayor que en la población general. El 21% de los participantes obtuvo puntuaciones en el rango moderado/grave. Se calculó una puntuación cognitiva-afectiva y una puntuación somática-motivacional para cada paciente. Las puntuaciones en ambas dimensiones fueron significativamente mayores que en la población general. Las variables clínico-demográficas no estaban relacionadas con los síntomas de depresión, a excepción de la gravedad de la ataxia. Los síntomas depresivos predicen los tiempos de reacción cognitivos. Conclusiones: Nuestros datos muestran que en la FRDA son frecuentes los síntomas de depresión, tanto los síntomas somático-motivacionales como los cognitivo-afectivos. Además, los síntomas de depresión pueden influir en la cognición, especialmente, los de tipo cognitivo-afectivo.

Depressive symptoms in Friedreich ataxia / Síntomas depresivos en la ataxia de Friedreich

Background/Objective: Almost no attention has been paid to depression in Friedreich ataxia (FRDA), a highly disabling cerebellar degenerative disease. Our aim was to study the presence and the profile of depressive symptoms in FRDA and their relationship with demographic-disease variables and cognitive processing speed. Method: The study groups consisted of 57 patients with a diagnosis of FRDA. The Beck Depression Inventory-II was used to assess symptoms of depression. Speed of information processing was measured with a Choice Reaction time task. Results: The mean BDI score for patients was significantly higher than the mean score in the general population. Twenty one percent of participants scored in the moderate/ severe range. A Cognitive-Affective score and a Somatic-Motivational score was calculated for each patient. Patients' scores in both dimensions were significantly higher than the scores in the general population. Demographic and disease variables were not related with symptoms of depression, except for severity of ataxia. Depressive symptoms predict cognitive reaction times. The greater proportion of variance was explained by the Cognitive-Affective dimension. Conclusions: Our data show that both somatic-motivational and cognitive affective symptoms of depression are frequent in individuals with FRDA. In addition, depressive symptoms may influence cognition, especially, the cognitive and affective symptoms (AU)

Antecedentes/Objetivo: La depresión en la ataxia de Friedreich (FRDA), una enfermedad degenerativa cerebelosa altamente incapacitante, ha recibido poca atención. Nuestro objetivo es evaluar la presencia y el perfil de los síntomas depresivos en FRDA y su relación con variables clínico-demográficas y la velocidad de procesamiento cognitivo. Método: Se estudiaron 57 pacientes con diagnóstico de FRDA. Se usó el Inventario de Depresión de Beck-II para evaluar los síntomas de depresión. La velocidad de procesamiento se midió con una tarea de tiempos de reacción. Resultados: La puntuación media de los pacientes en el BDI fue significativamente mayor que en la población general. El 21% de los participantes obtuvo puntuaciones en el rango moderado/grave. Se calculó una puntuación cognitiva-afectiva y una puntuación somática-motivacional para cada paciente. Las puntuaciones en ambas dimensiones fueron significativamente mayores que en la población general. Las variables clínico-demográficas no estaban relacionadas con los síntomas de depresión, a excepción de la gravedad de la ataxia. Los síntomas depresivos predicen los tiempos de reacción cognitivos. Conclusiones: Nuestros datos muestran que en la FRDA son frecuentes los síntomas de depresión, tanto los síntomas somáticomotivacionales como los cognitivo-afectivos. Además, los síntomas de depresión pueden influir en la cognición, especialmente, los de tipo cognitivo-afectivo (AU)

Versión paralela del test de aprendizaje verbal España-Complutense (TAVEC). Réplica / Alternate form of the test de aprendizaje verbal España-Complutense (TAVEC). Reply

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Versión paralela del test de aprendizaje verbal España-Complutense (TAVEC) / Alternate form of the test de aprendizaje verbal España-Complutense (TAVEC)

Introducción. La disponibilidad de formas paralelas de instrumentos de evaluación neuropsicológica es escasa. El uso repetido del material y el consiguiente efecto de la práctica dificultan la interpretación de los cambios observados en evaluaciones sucesivas. La memoria es una de las funciones más afectadas por este efecto. Objetivo. Obtener una versión paralela de uno de los instrumentos disponibles en español para la evaluación del aprendizaje y la memoria verbal, el test de aprendizaje verbal España-Complutense (TAVEC). Sujetos y métodos. Se realizó un estudio normativo con una muestra de 110 sujetos para la obtención de los ítems de la forma paralela, siguiendo los criterios utilizados en la versión original. La muestra para el estudio de la versión paralela estuvo formada por 70 sujetos neurológicamente sanos, de 18-89 años. Se aplicaron ambas versiones en un intervalo de 15-20 días. Resultados. Los análisis multivariados mostraron que no se producía efecto de la forma, del orden de administración ni de la sesión. Las correspondientes interacciones tampoco fueron significativas. Estos resultados se observaron tanto para la muestra total como para el grupo de jóvenes (18-29 años), edad intermedia (30-59 años) y envejecimiento (60-89 años). Los análisis correlacionales mostraron la validez y consistencia interna de la forma alternativa. Conclusiones. Los resultados muestran la equivalencia entre la versión original del TAVEC y la versión elaborada en esta investigación. Es, por tanto, una versión recomendable para su uso en el estudio de la evolución de los déficits de aprendizaje y memoria (AU)

Introduction. Parallel forms of neuropsychological tests are scarce. Practice effects associated to repeated testing with the same test confound the interpretation of observed changes in serial assessments. Practice effects are especially likely with memory testing. Aim. To develop an alternate form to the test de aprendizaje verbal España-Complutense (TAVEC), one of the most common memory tests used for Spanish speaking population. Subjects and methods. Participants in the normative study were 110 undergraduates. Participants in the study of the alternate vs original forms were 70 neurologically normal volunteers ranged in age from 18 to 89 years. Forms were administered in counterbalanced order, with a test-retest interval of 15-20 days. Results. Multivariate analyses showed that none of the effects for form, order of administration or session achieved significance. Interactions also failed to reach significance. Aforementioned results were observed in the total sample and the different age groups: young adults (18-29 years), middle-age (30-59 years) and older (60-89 years). Correlational analyses supported the validity and internal consistence of the alternate form. Conclusions. Results indicate the equivalence between the original TAVEC and the form elaborated in this study. This alternate form may be used in serial assessment of learning and memory deterioration (AU)

[Alternate form of the test de aprendizaje verbal España-Complutense (TAVEC)]. / Version paralela del test de aprendizaje verbal España-Complutense (TAVEC).

INTRODUCTION: Parallel forms of neuropsychological tests are scarce. Practice effects associated to repeated testing with the same test confound the interpretation of observed changes in serial assessments. Practice effects are especially likely with memory testing. AIM: To develop an alternate form to the test de aprendizaje verbal España-Complutense (TAVEC), one of the most common memory tests used for Spanish speaking population. SUBJECTS AND METHODS: Participants in the normative study were 110 undergraduates. Participants in the study of the alternate vs original forms were 70 neurologically normal volunteers ranged in age from 18 to 89 years. Forms were administered in counterbalanced order, with a test-retest interval of 15-20 days. RESULTS: Multivariate analyses showed that none of the effects for form, order of administration or session achieved significance. Interactions also failed to reach significance. Aforementioned results were observed in the total sample and the different age groups: young adults (18-29 years), middle-age (30-59 years) and older (60-89 years). Correlational analyses supported the validity and internal consistence of the alternate form. CONCLUSIONS: Results indicate the equivalence between the original TAVEC and the form elaborated in this study. This alternate form may be used in serial assessment of learning and memory deterioration.

TITLE: Version paralela del test de aprendizaje verbal España-Complutense (TAVEC).Introduccion. La disponibilidad de formas paralelas de instrumentos de evaluacion neuropsicologica es escasa. El uso repetido del material y el consiguiente efecto de la practica dificultan la interpretacion de los cambios observados en evaluaciones sucesivas. La memoria es una de las funciones mas afectadas por este efecto. Objetivo. Obtener una version paralela de uno de los instrumentos disponibles en español para la evaluacion del aprendizaje y la memoria verbal, el test de aprendizaje verbal España-Complutense (TAVEC). Sujetos y metodos. Se realizo un estudio normativo con una muestra de 110 sujetos para la obtencion de los items de la forma paralela, siguiendo los criterios utilizados en la version original. La muestra para el estudio de la version paralela estuvo formada por 70 sujetos neurologicamente sanos, de 18-89 años. Se aplicaron ambas versiones en un intervalo de 15-20 dias. Resultados. Los analisis multivariados mostraron que no se producia efecto de la forma, del orden de administracion ni de la sesion. Las correspondientes interacciones tampoco fueron significativas. Estos resultados se observaron tanto para la muestra total como para el grupo de jovenes (18-29 años), edad intermedia (30-59 años) y envejecimiento (60-89 años). Los analisis correlacionales mostraron la validez y consistencia interna de la forma alternativa. Conclusiones. Los resultados muestran la equivalencia entre la version original del TAVEC y la version elaborada en esta investigacion. Es, por tanto, una version recomendable para su uso en el estudio de la evolucion de los deficits de aprendizaje y memoria.