Usability study and pilot validation of a computer-based emotion recognition test for older adults with Alzheimer's disease and amnestic mild cognitive impairment.

OBJECTIVES: This study aimed to carry out a pilot validation of Affect-GRADIOR, a computer-based emotion recognition test, with older adults. The study evaluated its usability, reliability and validity for the screening of people with Alzheimer´s disease (AD) and amnestic mild cognitive impairment (aMCI). METHODS: The test was administered to 212 participants (76.37 ± 6.20 years) classified into three groups (healthy controls, n = 69; AD, n = 84; and aMCI, n = 59) on the basis of detailed neurological, neuropsychological, laboratory and neuro-imaging evidence. Data on usability were collected by means of a questionnaire and automated evaluation. RESULTS: The validated test comprised 53 stimuli and 7 practice items (one per emotion). Participants reported that Affect-GRADIOR was accessible and user-friendly. It had high internal consistency (ordinal Cronbach's α = 0.96). Test-retest reliability correlations were significant and robust (r = 0.840, p < 0.001). Exploratory factor analysis supported a seven-factor model of the emotions assessed (neutral expression, happiness, surprise, disgust, sadness, anger and fear). Receiver operating characteristic curve analyses suggested that the test discriminated healthy older adults from AD and aMCI cases. Correct answer score improved MMSE predictive power from 0.547 to 0.560 (Cox & Snell R2, p = 0.012), and Affect-GRADIOR speed of processing score improved MMSE predictive power from 0.547 to 0.563 (Cox & Snell R2, p = 0.010). CONCLUSIONS: Affect-GRADIOR is a valid instrument for the assessment of the facial recognition of emotions in older adults with and without cognitive impairment.

ELECTRONIC DEVICES FOR COGNITIVE IMPAIRMENT SCREENING: A SYSTEMATIC LITERATURE REVIEW.

OBJECTIVES: The reduction in cognitive decline depends on timely diagnosis. The aim of this systematic review was to analyze the current available information and communication technologies-based instruments for cognitive decline early screening and detection in terms of usability, validity, and reliability. METHODS: Electronic searches identified 1,785 articles of which thirty-four met the inclusion criteria and were grouped according to their main purpose into test batteries, measures of isolated tasks, behavioral measures, and diagnostic tools. RESULTS: Thirty one instruments were analyzed. Fifty-two percent were personal computer based, 26 percent tablet, 13 percent laptop, and 1 was mobile phone based. The most common input method was touchscreen (48 percent). The instruments were validated with a total of 4,307 participants: 2,146 were healthy older adults (M = 73.59; SD = 5.12), 1,104 had dementia (M = 74.65; SD = 3.98) and 1,057 mild cognitive impairment (M = 74.84; SD = 4.46). Only 6 percent were administered at home, 19 percent reported outcomes about usability, and 22 percent about understandability. The methodological quality of the studies was good, the weakest methodological area being usability. Most of the instruments obtained acceptable values of specificity and sensitivity. CONCLUSIONS: It is necessary to create home delivered instruments and to include usability studies in their design. Involvement of people with cognitive decline in all phases of the development process is of great importance to obtain valuable and user-friendly products. It would be advisable for researchers to make an effort to provide cutoff points for their instruments.

Sporadic adult-onset leucodystrophy with axonal spheroids and pigmented glia with no mutations in the known targeted genes.

AIMS: Adult-onset orthochromatic leucodystrophy, associated with pigmented macrophages and hereditary diffuse leucoencephalopathy with spheroids, are two disorders with similar clinical manifestations, radiological characteristics and neuropathological findings. Mutations in the colony-stimulating factor 1 receptor (CSF1R) gene are the hallmark of this spectrum of disease. Furthermore, polycystic membranous lipomembranous osteodysplasia with sclerosing leucoencephalopathy is caused by mutations in two genes, DAP12 and TREM2, which encode proteins involved in the same pathways as CSF1R. We describe a case of sporadic adult-onset orthochromatic leucodystrophy associated with pigmented macrophages diagnosed by biopsy without harbouring mutations in the known targeted genes. METHODS AND RESULTS: A 51-year-old woman, with no familial history of neurological diseases, developed a progressive neurological deterioration showing inappropriate behaviour, ataxia, spasticity, axial dystonia and agitation. Radiological images and a stereotaxic biopsy were conclusive with adult-onset orthochromatic leucodystrophy associated with pigmented macrophages. Genetic analysis did not show mutations in either CSF1R, DAP12 or TREM2. CONCLUSIONS: We add support to the idea that all these entities are closely related diseases linked to a convergent metabolic pathway, but caused by different genes or perhaps by the combination of individually non-pathogenic variations of selected genes. Genetic defects are still barely known in a substantial number of adult leucodystrophies.

The efficacy of nonpharmacological treatment for dementia-related apathy.

OBJECTIVE: To determine the usefulness of a formal, structured, nonpharmacological treatment for apathetic dementia patients. PATIENTS AND METHODS: A controlled, cross-over, randomized, simple-blind, multicentre clinical trial. A total of 146 institutionalized or day care dementia patients with a stage 1 or 2 Clinical Dementia Rating, with no significant motor or sensory problems, or with rapidly evolving dementia and in a clinically stable state were recruited. A cognitive mini examination, and functional, depression, and potential toxicity scales, Neuropsychiatric Inventory Questionnaire (NPI-Q), and Dementia Apathy Interview and Rating (DAIR) scales were applied at baseline. Patients were randomized to initial intervention (music and art therapy and psychomotor activity) or initial control (free activities in the day room) and changed over at 4 weeks. NPI-Q and DAIR scales were administered at the end of weeks 4 and 8. RESULTS: A significant difference between intervention and control periods, as measured using the DAIR scale, was observed, with a difference of 0.21 (95% confidence interval: 0.07-0.34, P<0.005). The difference was very important in the patients with moderated apathy. However, differences were not so important in the patients with severe apathy and there were no differences at all in the non-apathetic patients. No significant differences were found with the NPI-Q scale, although there was a clear improvement trend in the "apathy" question on this scale. This effect seems to extend beyond the therapeutic intervention period. CONCLUSIONS: A structured, nonpharmacological, short-term occupational therapy intervention is more useful than activities of the patients' own choice for improving apathy in patients with mild or moderate dementia.

Domain or not domain? That is the question: longitudinal semantic deterioration in Alzheimer's disease.

Category specific semantic impairment (e.g. living versus nonliving things) has been reported in association with various pathologies, including herpes simplex encephalitis and semantic dementia. However, evidence is inconsistent regarding whether this effect exists in diseases progressively impacting diverse cortical regions, such as Alzheimer's disease (AD). Ceiling effects producing non-Gaussian distributions and poor control for confounds such as nuisance variables (e.g. familiarity) may contribute to this discrepancy. Fourteen AD patients were longitudinally studied examining category effects on three semantic tasks (picture naming, naming to description and word to picture matching) matched across domain on all known nuisance variables (NV). To address non-Gaussian distributions, we run bootstrap analyses to determine whether NV, semantic domain or control performance best predicted AD patient performance. Multiple hierarchical regression analyses revealed that, whilst NV accounted for most of the explained variance in patients in the three tasks, the influence of semantic domain was substantially lower. Individual logistic regression demonstrated a significant category effect in only a few patients and healthy controls. No significant qualitative changes were observed in patients over time. Our results confirm the importance of NVs as predictors of AD patient performance, suggesting that the role of semantic domain is not a useful predictor of the progressive deterioration in AD.

Intelligence impairment, personality features and psychopathology disturbances in a family affected with CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a small-vessel disease of the brain that is characterized by headache, recurring lacunar strokes, mood changes and progressive cognitive deterioration. The disease is transmitted with an autosomal dominant pattern and usually starts during midadulthood (at 30-50 years of age). Cognitive deficits in patients with CADASIL develop slowly. The dementia causes frontal-like symptoms and it typically develops after a history of recurrent stroke. We describe three patients from one Spanish family affected by this disease. All three cases underwent comprehensive clinical and neuropsychological examination, and were monitored for seven years. The results obtained in this study describe a) a significant loss of the intelligence quotient (IQ) and noticeable damage to abstract ability (g factor), b) mood and psychopathological disturbances (major depression and dysthymia), and c) a personality with neurotic features.

Hemiballism due to hyperglycemic hyperosmolar syndrome.

Non-ketotic hyperglycemia has occasionally been associated with various neurological abnormalities including movement disorders. Hyperglycemic hyperosmolar syndrome (HHS) is the second most common cause of hemiballism-hemichorea, which is due to a contralateral putaminal lesion. We describe a 95-year-old woman with HHS who developed hemichorea-hemiballism syndrome due to a putaminal lesion.

The Efficacy of Emotion Recognition Rehabilitation for People with Alzheimer's Disease.

BACKGROUND: The ability to recognize emotional expression is essential for social interactions, adapting to the environment, and quality of life. Emotion recognition is impaired in people with Alzheimer's disease (AD), thus rehabilitation of these skills has the potential to elicit significant benefits. OBJECTIVE: This study sought to establish whether emotion recognition capacity could be rehabilitated in people with AD. METHODS: Thirty-six participants with AD were assigned to one of three conditions: an experimental group (EG) that received 20 sessions of rehabilitation of emotion recognition and 20 sessions of cognitive stimulation therapy (CST), a control group (CG) that received 40 sessions of CST, and a treatment as usual group (TAU). RESULTS: A positive treatment effect favoring the EG was found; participants were better able to correctly identify emotions (p = 0.021), made fewer errors of commission (p = 0.002), had greater precision of processing (p = 0.021), and faster processing speed (p = 0.001). Specifically, the EG were better able to identify sadness (p = 0.016), disgust (p = 0.005), and the neutral expression (p = 0.014), with quicker processing speed for disgust (p = 0.002). These gains were maintained at one month follow-up with the exception of processing speed for surprise, which improved. CONCLUSION: Capacity to recognize facial expressions of emotions can be improved through specific rehabilitation in people with AD, and gains are still present at a one month follow up. These findings have implications for the design of rehabilitation techniques for people with AD that may lead to improved quality of life and social interactions for this population.

Rehabilitación del reconocimiento de emociones combinada con estimulación cognitiva para personas con enfermedad de Alzheimer. Eficacia sobre aspectos cognitivos y funcionales / Emotion recognition rehabilitation combined with cognitive stimulation for people with Alzheimer’s disease. Efficacy for cognition and functional aspects

Introducción. Se han encontrado déficits en el reconocimiento de emociones en personas con enfermedad de Alzheimer (EA), pero no se ha informado de ningún tratamiento rehabilitador de esta capacidad en esta población. Objetivo. Evaluar la eficacia de un tratamiento combinado de rehabilitación del reconocimiento de emociones (RRE) y estimulación cognitiva (EsC) sobre las funciones cognitivas, el estado de ánimo, la anosognosia, la capacidad funcional y el reconocimiento de emociones de personas con EA. Sujetos y métodos. En un diseño cuasi experimental, 36 pacientes con EA fueron asignados a tres condiciones: un grupo experimental, que recibió 20 sesiones de RRE y 20 sesiones de EsC; un grupo control, que recibió 40 sesiones de EsC, y un grupo que recibió tratamiento usual (GTU). Resultados. Completaron el estudio 32 pacientes, con una edad media de 77,53 ± 5,43 años. Se observó mejoría en el Minimental State Examination en su versión de 30 ítems (F = 5,10; p = 0,013) y en la de 35 (F = 4,16; p = 0,026), reconocimiento de afectos (Z = -2,81; p = 0,005) y actividades básicas de la vida diaria (Z = -2,27; p = 0,018) a favor del grupo experimental. El grupo que recibió tratamiento usual empeoró en depresión (Z = -1,99; p = 0,048), apatía (Z = -2,30; p = 0,022) y anosognosia (Z = -2,19; p = 0,028). Conclusiones. El tratamiento combinado de RRE y EsC fue más eficaz que la EsC y que el tratamiento usual. Es la primera vez que se informa de un tratamiento rehabilitador del reconocimiento de emociones en personas con EA (AU)

Introduction. The ability to recognize facial emotional expression is essential for social interactions and adapting to the environment. Emotion recognition is impaired in people with Alzheimer’s disease (AD), thus rehabilitation of these skills has the potential to elicit significant benefits. Aim. To assess the efficacy of a combined treatment of rehabilitation of emotion recognition (RER) and cognitive stimulation (CS) for people with AD, due to its potential implications for more effective psychosocial interventions. Subjects and methods. 36 patients were assigned to one of three experimental conditions: an experimental group (EG) that received 20 sessions of RER and 20 sessions of CS; a control group (CG) that received 40 sessions of CS, and a treatment as usual group (TAU). Results. 32 patients completed the treatment (77.53 ± 5.43 years). Significant differences were found in MMSE30 (F = 5.10; p = 0.013), MMSE35 (F = 4.16; p = 0.026), affect recognition (Z = -2.81; p = 0.005) and basic activities of daily living (Z = -2.27; p = 0.018) favouring the efficacy of the combined treatment. The TAU group showed a decline in depression (Z = -1.99; p = 0.048), apathy (Z = -2.30; p = 0.022) and anosognosia (Z = -2.19; p = 0.028). Conclusions. The combined treatment of RER + CS was more effective than TAU and CS alone for the treatment of patients with AD. This is the first study about the rehabilitation of affect recognition in AD (AU)

Intelligence impairment, personality features and psychopathology disturbances in a family affected with CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a small-vessel disease of the brain that is characterized by headache, recurring lacunar strokes, mood changes and progressive cognitive deterioration. The disease is transmitted with an autosomal dominant pattern and usually starts during midadulthood (at 30–50 years of age). Cognitive deficits in patients with CADASIL develop slowly. The dementia causes frontal-like symptoms and it typically develops after a history of recurrent stroke. We describe three patients from one Spanish family affected by this disease. All three cases underwent comprehensive clinical and neuropsychological examination, and were monitored for seven years. The results obtained in this study describe a) a significant loss of the intelligence quotient (IQ) and noticeable damage to abstract ability (g factor), b) mood and psychopathological disturbances (major depression and dysthymia), and c) a personality with neurotic features (AU)

La arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía (CADASIL) se caracteriza por una alteración de las arterias cerebrales de pequeño y mediano calibre. La presentación clínica incluye migraña, infartos cerebrales recurrentes, cambios del humor y deterioro cognitivo. La enfermedad se transmite siguiendo un patrón autosómico dominante e inicia su desarrollo entre los 30- 50 años de edad. El deterioro cognitivo evoluciona lentamente hasta un cuadro similar al de la demencia frontal, y con frecuencia se desarrolla tras un periodo previo de episodios isquémicos recurrentes. Este trabajo describe a tres pacientes de una familia española afectada por la enfermedad. Se les siguió durante un período de siete años. Con el fin de estudiar la evolución del cuadro, se efectuaron diversas pruebas clínicas y neuropsicológicas. Los resultados obtenidos muestran a) una disminución significativa del cociente de inteligencia (CI) y un notorio deterioro del factor de inteligencia general (factor g), b) alteraciones psicopatológicas (depresión mayor y distimia), y c) un perfil de personalidad de rasgos neuróticos (AU)