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.

Systemic lupus erythematosus impairs memory cognitive tests not affected by depression.

OBJECTIVE: Our aim was to assess the contribution of depression to cognitive impairment in patients with systemic lupus erythematosus (SLE). METHODS: Clinical features, education, age, and Hospital Anxiety and Depression Scale (HADS) were evaluated in 82 patients with SLE and 22 healthy controls, all Chilean women. The Cambridge Neuropsychological Test Automated Battery (CANTAB eclipseTM) assessing attention, spatial memory, and learning and executive function domains was applied. Cognitive deficit definition: a cut-off for definite impairment was defined as a score below -2 standard deviations in at least one outcome measure in two or more domains. ANCOVA with stepwise selection evaluated influences of health status (SLE or control), age, education, and HADS depression and anxiety scores on cognitive outcomes. To avoid overfitting, a shrinkage method was performed. Also, adjusted p-values for multiple comparisons were obtained. RESULTS: Cognitive deficit affected 16 (20%) patients, and no controls (p=0.039). Median HADS depression score in SLE patients was 6 (range 0-19) and in controls was 0 (0-19), p<0.001). ANCOVA and shrinkage models showed that worse cognitive performance in sustained attention and spatial working memory tests was explained by the presence of SLE but not depression, whereas depression only affected a measure of executive function (I/ED Stages completed). CONCLUSION: Depression has a limited role in cognitive impairment in SLE. Impairments in sustained attention and spatial working memory are distinctly influenced by yet-unknown disease-intrinsic factors.

Testing the model of caudo-rostral organization of cognitive control in the human with frontal lesions.

The cascade model of cognitive control, mostly relying on functional neuroimaging studies, stipulates that the lateral frontal cortex (LFC) is organized as a cascade of executive processes involving three levels of cognitive control, implemented in distinct LFC areas from the premotor to the anterior prefrontal regions. The present experiment tested this model in patients with LFC lesions and studied the hierarchy of executive functions along the caudo-rostral axis, i.e. the respective roles of the different LFC areas in the control of behavior. Voxel-based lesion-symptom mapping and region of interest group analyses were conducted in 32 patients with focal LFC lesions who performed cognitive tasks assessing the cascade model. We first showed that three different LFC areas along the caudo-rostral axis subserved three distinct control levels, whose integrity is necessary for adaptive behavior. Second, we found that prefrontal cognitive control has an asymmetric organization: higher control processes involving more anterior prefrontal regions rely on the integrity of lower control processes in more posterior regions, while lower control processes can operate irrespective of the integrity of higher control processes. Altogether, these findings support a caudo-rostral cascade of executive processes from premotor to anterior prefrontal regions.

Affective style and eating disorders: a field study.

Eating disorders (ED) are a heterogeneous group of problems related to restraint and/or overeating. It is proposed that individual differences in affective reactivity and moods (affective style) might be related to these behaviours. Variations in affective style are expressed by differing levels of sensitivity to the motivation systems of approach and avoidance. The present study tested whether a relation exists between ED and variations in the sensitivity of motivational systems as well as mood dispositions. A total of 2020 undergraduate students completed the Eating Disorder Diagnostic Scale (EDDS), the Behavioural Inhibition System and Behavioural Activation System Scales (BIS/BAS), and the Positive and Negative Affect Schedule (PANAS). The results showed a significant within- subject interaction of Alimentary group x Motivation (F=4.056; p<0.007). It was also observed that the Overeating group had lower levels of motivation asymmetry than the Normal (p<0.01) and Restrictive (p<0.005) groups and marginally lower levels than the Purgative group (p<0.07). The study results suggest mainly that the avoidance/inhibition motivational system is related to eating problems connected with overeating, including chronic alimentary restraint (chronic dieters). The theoretical and clinical implications of these findings are discussed.

Causal role of prefrontal cortex in the threshold for access to consciousness.

What neural mechanisms support our conscious perception of briefly presented stimuli? Some theories of conscious access postulate a key role of top-down amplification loops involving prefrontal cortex (PFC). To test this issue, we measured the visual backward masking threshold in patients with focal prefrontal lesions, using both objective and subjective measures while controlling for putative attention deficits. In all conditions of temporal or spatial attention cueing, the threshold for access to consciousness was systematically shifted in patients, particular after a lesion of the left anterior PFC. The deficit affected subjective reports more than objective performance, and objective performance conditioned on subjective visibility was essentially normal. We conclude that PFC makes a causal contribution to conscious visual perception of masked stimuli, and outline a dual-route signal detection theory of objective and subjective decision making.

"Applause sign" helps to discriminate PSP from FTD and PD.

The "applause sign" is a simple test of motor control that helps to differentiate PSP from frontal or striatofrontal degenerative diseases. It was found in 0/39 controls, 0 of 24 patients with frontotemporal dementia (FTD), 0 of 17 patients with Parkinson disease (PD), and 30/42 patients with progressive supranuclear palsy (PSP). It discriminated PSP from FTD (p < 0.001) and PD (p < 0.00). The "three clap test" correctly identified 81.8% of the patients in the comparison PSP and FTD and 75% of the patients in the comparison of PSP and PD.

Visual word recognition in the left and right hemispheres: anatomical and functional correlates of peripheral alexias.

According to a simple anatomical and functional model of word reading, letters displayed in one hemifield are first analysed through a cascade of contralateral retinotopic areas, which compute increasingly abstract representations. Eventually, an invariant representation of letter identities is created in the visual word form area (VWFA), reproducibly located within the left occipito-temporal sulcus. The VWFA then projects to structures involved in phonological or lexico-semantic processing. This model yields detailed predictions on the reading impairments that may follow left occipitotemporal lesions. Those predictions were confronted to behavioural, anatomical and functional MRI data gathered in normals and in patients suffering from left posterior cerebral artery infarcts. In normal subjects, alphabetic stimuli activated both the VWFA and the right-hemispheric symmetrical region (R-VWFA) relative to fixation, but only the VWFA showed a preference for alphabetic strings over simple chequerboards. The comparison of normalized brain lesions with reading-induced activations showed that the critical lesion site for the classical syndrome of pure alexia can be tightly localized to the VWFA. Reading impairments resulting from deafferentation of an intact VWFA from right- or left-hemispheric input were dissected using the same methods, shedding light on the connectivity of the VWFA. Finally, the putative role of right-hemispheric processing in the letter-by-letter reading strategy was clarified. In a letter-by-letter reader, the R-VWFA assumed some of the functional properties normally specific to the VWFA. These data corroborate our initial model of normal word perception and underline that an alternative right-hemispheric pathway can underlie functional recovery from alexia.

The prefrontal cortex and conscious monitoring of action: an experimental study.

To investigate the role of the prefrontal cortex in conscious monitoring, we used an experimental paradigm generating a conflict between the action planned and the sensory-motor feedback. We analyzed the acquisition of explicit knowledge of the strategy for resolving the conflict and its influence on motor adaptation. Twenty patients with frontal lobe lesions and 18 controls had to trace a sagittal line with a stylus on a graphics tablet. A mirror on which the traced line, processed by a computer, was projected hid the hand. A mask limited visual feedback to the last third of the trajectory. Without informing the subjects, the line traced was modified by introducing a bias of 24 degrees to the right. To succeed in the task, subjects had to modify their motor program and to deviate their trajectory in the opposite direction. Conscious elaboration of the strategy was evaluated by the number of trials needed to explicitly report the required deviation. Three groups of patients were distinguished: (1). with normal explicit strategy; (2). with delayed explicit strategy, and (3). without explicit strategy at the last trial. They significantly differed by the severity of the dysexecutive syndrome, particularly of environmental adherence. Motor adaptation was evaluated by the area between the line traced and the ideal line to compensate for the deviation. In patients with normal elaboration of the strategy, motor control was similar to that of controls, but it was severely disturbed in the other two groups. These results suggest the involvement of the prefrontal cortex in conscious motor monitoring.

Self-monitoring in schizophrenia revisited.

According to a widespread theory, the first-rank symptoms such as delusions of control or thought insertion met in schizophrenia result from a failure in predicting the consequences of an action on the basis of a forward model based of the intended motor commands (efference copy). This assumption of an impairment in the central monitoring of their own actions is inferred from experiments showing that it is more difficult for schizophrenic patients than for controls to correct erroneous movements in the absence of visual feedback. In our study, 19 schizophrenic patients (10 with Schneiderian symptoms and nine without) and 19 paired control subjects were subjected to a sensorimotor adjustment task to reassess this hypothesis. We show that the patients who succeeded the task not differently from the control subjects were those who were aware of the manual correction (n = 9). Surprisingly, most of them presented Schneiderian symptoms. This suggests that the experience of alien control observed in certain schizophrenic patients cannot be directly related to an underlying cognitive deficit in the conscious monitoring of their own actions.

Preserved adjustment but impaired awareness in a sensory-motor conflict following prefrontal lesions.

Control of action occurs at different stages of the executive process, in particular at those of sensory-motor integration and conscious monitoring. The aim of this study was to determine the implication of the prefrontal cortex in the control of action. For that purpose, we compared the performance of 15 patients with frontal lobe lesions and 15 matched controls on an experimental paradigm generating a conflict between the action planned and the sensory-motor feedback. Subjects had to trace a sagittal line with a stylus on a graphic tablet. The hand was hidden by a mirror on which the traced line, processed by a computer, was projected. Without informing the subjects, the line traced was modified by introducing a bias to the right, which increased progressively from 2 degrees to 42 degrees. To succeed the task, subjects had to modify their motor program and deviate their hand in the opposite direction. The sensory-motor adjustment to the bias was evaluated by the surface between the line traced and the ideal line to compensate for the deviation. The awareness of the conflict was measured by the angle of the bias at which subjects expressed the feeling that the line they traced was not the same as the line they saw. The deviation was similarly compensated for by patients and controls until 24 degrees. Then 14 controls but only 3 patients were aware of a conflict. After that, the variability of performance increased significantly for the unaware patients. These results suggest that the prefrontal cortex is required at the level of conscious monitoring of actions, but not at the level of sensory-motor integration.

The FAB: a Frontal Assessment Battery at bedside.

OBJECTIVE: To devise a short bedside cognitive and behavioral battery to assess frontal lobe functions. METHODS: The designed battery consists of six subtests exploring the following: conceptualization, mental flexibility, motor programming, sensitivity to interference, inhibitory control, and environmental autonomy. It takes approximately 10 minutes to administer. The authors studied 42 normal subjects and 121 patients with various degrees of frontal lobe dysfunction (PD, n = 24; multiple system atrophy, n = 6; corticobasal degeneration, n = 21; progressive supranuclear palsy, n = 47; frontotemporal dementia, n = 23). RESULTS: The Frontal Assessment Battery scores correlated with the Mattis Dementia Rating Scale scores (rho = 0.82, p < 0.01) and with the number of criteria (rho = 0.77, p < 0.01) and perseverative errors (rho = 0.68, p < 0.01) of the Wisconsin Card Sorting Test. These variables accounted for 79% of the variance in a stepwise multiple regression, whereas age or Mini-Mental State Examination scores had no significant influence. There was good interrater reliability (kappa = 0.87, p < 0.001), internal consistency (Cronbach's coefficient alpha = 0.78), and discriminant validity (89.1% of cases correctly identified in a discriminant analysis of patients and controls). CONCLUSION: The Frontal Assessment Battery is easy to administer at bedside and is sensitive to frontal lobe dysfunction.

[Four "alien" hands for two hands after a lesion in corpus callosum]. / Quatre mains "étranges" pour deux mains après une lésion calleuse.

Callosal lesions, associated or not to internal frontal lesions, may produce different types of complex gestural behaviors. Four signs can be identified, each of which has been generally reported separately: the "alien hand" sign, the "diagnostic apraxia", the "wayward hand" and the "callosal apraxia". Some authors justify considering these signs as different entities, while others propose regrouping them either in an unique syndrome--the "alien hand"--or as two syndromes--the "frontal alien hand" and the "callosal alien hand". We present the observation of a patient who presented with the four mentioned syndromes in association. In this context, we review the clinical features of each of the four signs and the arguments supporting their individualization.

["Boxer's dementia" without motor signs]. / "Démence pugilistique" sans signes moteurs.

BACKGROUND: The neurological complications related to boxing include dementia. Boxer's dementia is generally associated with severe motor impairment. CASE REPORT: A former professional boxer presented dementia with no motor signs. The diagnostic discussion was based on clinical observations, and neuropsychological and supplementary explorations, and eliminated all other etiological hypotheses. DISCUSSION: This case draws attention to the possibility of cognitive disorders without motor impairment in the neurological complications of boxing.

[Myopathy caused by acid maltase deficiency in an adult]. / Miopatía por déficit de maltasa ácida en el adulto.

We report a 46 years old male presenting with tetraparesis and severe respiratory involvement. He had moderately elevated serum creatine phosphokinase values and the electromyography showed myopathic alterations and irritative signs. In the muscle biopsy, a vacuolar myopathy with increased collagen deposits was found. Circulating lymphocytes presented abnormal PAS positive granules in their cytoplasm.

[Borderline personality disorder: a comparative study between the clinical diagnosis and the Rorschach test]. / Trastorno limítrofe de personalidad: estudio comparativo del diagnóstico clínico y la prueba de Rorschach.

Diagnostic concordance among Rorschach Test, clinical approach and DSM-III-R criteria for BPD is studied in an inpatient sample. A low degree of concordance among the three diagnostic systems was found. Two among 8 DSM-III-R criteria (physically self-damaging acts, and unstable-intense relationships) were the most discriminating symptoms for diagnosing BPD. The importance of considering the diagnostic efficiency of each DSM-III-R criterion is discussed. The most frequent concurrent diagnosis along with BPD were: Affective, and psychotic disorders. Their relationship is analyzed.

Trastorno limítrofe de personalidad: estudio comparativo del diagnóstico clínico y la prueba de Rorschach. / [Borderline personality disorder: a comparative study between the clinical diagnosis and the Rorschach test]

Diagnostic concordance among Rorschach Test, clinical approach and DSM-III-R criteria for BPD is studied in an inpatient sample. A low degree of concordance among the three diagnostic systems was found. Two among 8 DSM-III-R criteria (physically self-damaging acts, and unstable-intense relationships) were the most discriminating symptoms for diagnosing BPD. The importance of considering the diagnostic efficiency of each DSM-III-R criterion is discussed. The most frequent concurrent diagnosis along with BPD were: Affective, and psychotic disorders. Their relationship is analyzed.

Trastorno limítrofe de personalidad: estudio comparativo del diagnóstico clínico y la prueba de Rorschach. / [Borderline personality disorder: a comparative study between the clinical diagnosis and the Rorschach test]

Diagnostic concordance among Rorschach Test, clinical approach and DSM-III-R criteria for BPD is studied in an inpatient sample. A low degree of concordance among the three diagnostic systems was found. Two among 8 DSM-III-R criteria (physically self-damaging acts, and unstable-intense relationships) were the most discriminating symptoms for diagnosing BPD. The importance of considering the diagnostic efficiency of each DSM-III-R criterion is discussed. The most frequent concurrent diagnosis along with BPD were: Affective, and psychotic disorders. Their relationship is analyzed.