Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Cognitive and olfactory deficits in Machado-Joseph disease: A dopamine transporter study

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Cross-cultural differences for three visual memory tasks in Brazilian children

Norms for three visual memory tasks, including Corsi's block tapping test and the BEM 144 complex figures and visual recognition, were developed for neuropsychological assessment in Brazilian children. The tasks were measured in 127 children ages 7 to 10 years from rural and urban areas of the States of São Paulo and Minas Gerais. Analysis indicated age-related but not sex-related differences. A cross-cultural effect was observed in relation to copying and recall of Complex pictures. Different performances between rural and urban children were noted. © Perceptual and Motor Skills 2005.

Depression severity levels and cognitive functions in institutionalized elderly patients

Depression is a highly prevalent illness among institutionalized aged and assumes peculiar characteristics such as the risk for progressing to dementia. The aims of this study was to assess the cognitive functions of institutionalized elderly with clinical diagnosis of depression and compare the severity of depressive symptoms with cognitive performance. From 120 residents at a nursing home in Rio Claro, Brazil, we study 23 individuals (mean age: 74.3 years; mean schooling: 4.0 years) with diagnosis of depression. At first, a clinical diagnosis of depression and measurement of its symptoms using the Geriatric Depression Scale were performed. The patient then underwent a neuropsychological assessment based on the following tests: Mini-Mental Examination, Verbal Fluency, Visual Perception, Immediate Memory, Recent Memory, Recognition, Clock Drawing Test. The patients were divided into two groups: those with less severe depression symptoms (Group 1: N=9) and more severe symptoms (Group 2: N=14). The significant difference between symptom severity of the two groups was p=0.0001. Patients with more severe symptoms revealed a slightly inferior cognitive performance in most of the tests when compared to those with less severe symptoms (p>0.05). In relation to Verbal Fluency, patients with more severe depression symptoms presented a significantly inferior cognitive performance when compared to those with less severe symptoms (p=0.0082). Verbal Fluency revealed to be a more sensitive test for measuring early cognitive alterations in institutionalized aged with depression, and appears to be a useful resource in monitoring the cognitive functions of patients faced with the risk of dementia. © Copyright Moreira Jr. Editora.

Mild cognitive impairment (part 1): Clinical characteristics and predictors of dementia

Objective: To critically review and evaluate existing knowledge on the conceptual limits and clinical usefulness of the diagnosis of mild cognitive impairment (MCI) and the neuropsychological assessment and short- and long-term prognosis thereof. Methods: We conducted a systematic search of the PubMed and Web of Science electronic databases, limited to articles published in English between 1999 and 2012. Based on the search terms mild cognitive impairment or MCI and epidemiology or diagnosis, we retrieved 1,698 articles, of which 248 were critically eligible (cross-sectional and longitudinal studies); the abstracts of the remaining 1,450 articles were also reviewed. Results: A critical review on the MCI construct is provided, including conceptual and diagnostic aspects; epidemiological relevance; clinical assessment; prognosis; and outcome. The distinct definitions of cognitive impairment, MCI included, yield clinically heterogeneous groups of individuals. Those who will eventually progress to dementia may present with symptoms consistent with the definition of MCI; conversely, individuals with MCI may remain stable or return to normal cognitive function. Conclusion: On clinical grounds, the cross-sectional diagnosis of MCI has limited prognostic relevance. The characterization of persistent and/or progressive cognitive deficits over time is a better approach for identification of cases at the pre-dementia stages, particularly if these cognitive abnormalities are consistent with the natural history of incipient Alzheimer's disease. © 2013 Associação Brasileira de Psiquiatria.

Funções neuropsicológicas executivas pós acidente vascular encefálico hemorrágico

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Does the acute cerebellitis play a role in the neurocognitive profile of a child after its onset?

This study aimed to present the neuropsychological assessment of MC, a seven-year-old girl, who was diagnosed with acute viral cerebellitis. The protocol included cognitive tests, stress and mood scales, behavioral observation, and interviews with family and school. In the neuropsychological assessment, difficulties in crystallized skills, especially in language and numerical cognition domains, were observed. The results of the neuropsychological assessment were discussed to detail her neurocognitive profile and verify to what extent her impairment could be attributed to the acute viral cerebellitis.

Longitudinal evolution of cognitive functions in patients with multiple system atrophy: a prospective study

Introduction and Background: Multiple system atrophy (MSA) is a sporadic, adult-onset, progressive neurodegenerative disease characterized clinically by parkinsonism, cerebellar ataxia, and autonomic failure. We investigated cognitive functions longitudinally in a group of probable MSA patients, matching data with sleep parameters. Patients and Methods: 10 patients (7m/3f) underwent a detailed interview, a general and neurological examination, laboratory exams, MRI scans, a cardiovascular reflexes study, a battery of neuropsychological tests, and video-polysomnographic recording (VPSG). Patients were revaluated (T1) a mean of 16±5 (range: 12-28) months after the initial evaluation (T0). At T1, the neuropsychological assessment and VPSG were repeated. Results: The mean patient age was 57.8±6.4 years (range: 47-64) with a mean age at disease onset of 53.2±7.1 years (range: 43-61) and symptoms duration at T0 of 60±48 months (range: 12-144). At T0, 7 patients showed no cognitive deficits while 3 patients showed isolated cognitive deficits. At T1, 1 patient worsened developing multiple cognitive deficits from a normal condition. At T0 and T1, sleep efficiency was reduced, REM latency increased, NREM sleep stages 1-2 slightly increased. Comparisons between T1 and T0 showed a significant worsening in two tests of attention and no significant differences of VPSG parameters. No correlation was found between neuropsychological results and VPSG findings or RBD duration. Discussion and Conclusions: The majority of our patients do not show any cognitive deficits at T0 and T1, while isolated cognitive deficits are present in the remaining patients. Attention is the cognitive function which significantly worsened. Our data confirm the previous findings concerning the prevalence, type and the evolution of cognitive deficits in MSA. Regarding the developing of a condition of dementia, our data did not show a clear-cut diagnosis of dementia. We confirm a mild alteration of sleep structure. RBD duration does not correlate with neuropsychological findings.

Visuospatial working memory in very preterm and term born children - Impact of age and performance

Working memory is crucial for meeting the challenges of daily life and performing academic tasks, such as reading or arithmetic. Very preterm born children are at risk of low working memory capacity. The aim of this study was to examine the visuospatial working memory network of school-aged preterm children and to determine the effect of age and performance on the neural working memory network. Working memory was assessed in 41 very preterm born children and 36 term born controls (aged 7–12 years) using functional magnetic resonance imaging (fMRI) and neuropsychological assessment. While preterm children and controls showed equal working memory performance, preterm children showed less involvement of the right middle frontal gyrus, but higher fMRI activation in superior frontal regions than controls. The younger and low-performing preterm children presented an atypical working memory network whereas the older high-performing preterm children recruited a working memory network similar to the controls. Results suggest that younger and low-performing preterm children show signs of less neural efficiency in frontal brain areas. With increasing age and performance, compensational mechanisms seem to occur, so that in preterm children, the typical visuospatial working memory network is established by the age of 12 years.

Improving children’s cognitive performance – Effect of a memory strategy training in children born very preterm

Aims: To examine the effect of memory strategy training on different aspects of memory in children born very preterm and to determine whether there is a generalization of the training effect to non-trained functions. The influence of individual factors such as age and performance level on the training success will be determined. Methods: In a randomized, controlled and blinded clinical trial, 46 children born very preterm (aged 7-12 years) were allocated to a memory strategy training (MEMO-Training, n=23) or a control group (n=23). Neuropsychological assessment was performed before, immediately after the training and at a 6-month follow-up. In the MEMO-Training, five different memory strategies were introduced and practiced in a one-to-one setting (4 hour-long training sessions over 4 weeks, 20 homework sessions). Results: A significant training-related improvement occurred in trained aspects of memory (verbal and visual learning and recall, verbal working memory) and in non-trained functions (inhibition, mental arithmetic). No performance increase was observed in the control group. At six months follow-up, there was a significant training-related improvement of visual working memory. Age and performance level before the training predicted the training success significantly. Conclusion: Teaching memory strategies is an effective way to improve different aspects of memory but also non-trained functions such as inhibition and mental arithmetic in children born very preterm. Age and performance level influence the success of memory strategy training. These results highlight the importance of teaching children memory strategies to reduce scholastic problems.

Factors affecting cognitive outcome in early pediatric stroke

OBJECTIVE We examined cognitive performance in children after stroke to study the influence of age at stroke, seizures, lesion characteristics, neurologic impairment (NI), and functional outcome on cognitive outcome. METHODS This was a prospectively designed study conducted in 99 children who sustained an arterial ischemic stroke (AIS) between the age of 1 month and 16 years. All children underwent cognitive and neurologic follow-up examination sessions 2 years after the insult. Cognitive development was assessed with age-appropriate instruments. RESULTS Although mean cognitive performance was in the lower normative range, we found poorer results in subtests measuring visuoconstructive skills, short-term memory, and processing speed. Risk factors for negative cognitive outcome were young age at stroke, seizures, combined lesion location (cortical and subcortical), as well as marked NI. CONCLUSIONS We recommend that all children with a history of AIS undergo regularly scheduled neuropsychological assessment to ensure implementation of appropriate interventions and environmental adjustments as early as possible.

Effects of two different memory training approaches in very preterm-born children

Background: Little research has been conducted to assess the effect of using memory training with school-aged children who were born very preterm. This study aimed to determine whether two types of memory training approaches resulted in an improvement of trained functions and/or a generalization of the training effect to non-trained cognitive domains. Methods: Sixty-eight children born very preterm (7¬-12 years) were randomly allocated to a group undertaking memory strategy training (n=23), working memory training (n=22), or a waiting control group (n=23). Neuropsychological assessment was performed before and immediately after the training or waiting period, and at a six-month follow-up. Results: In both training groups, significant improvement of different memory domains occurred immediately after training (near transfer). Improvement of non-trained arithmetic performance was observed after strategy training (far transfer). At a six-month follow-up assessment, children in both training groups demonstrated better working memory, and their parents rated their memory functions to be better than controls. Performance level before the training was negatively associated with the training gain. Conclusions: These results highlight the importance of cognitive interventions, in particular the teaching of memory strategies, in very preterm-born children at early school age to strengthen cognitive performance and prevent problems at school.

Effects of two different memory training approaches in very pretermborn children

Background: Little research has been conducted to assess the effect of using memory training with school aged children who were born very preterm. This study aimed to determine whether two types of memory training approaches resulted in an improvement of trained functions and/or a generalization of the training effect to non-trained cognitive domains. Methods: Sixty-eight children born very preterm (7-12 years) were randomly allocated to a group undertaking memory strategy training (n=23), working memory training (n=22), or a waiting control group (n=23). Neuropsychological assessment was performed before and immediately after the training or waiting period, and at a six-month follow-up. Results: In both training groups, significant improvement of different memory domains occurred immediately after training (near transfer). Improvement of non-trained arithmetic performance was observed after strategy training (far transfer). At a six-month follow-up assessment, children in both training groups demonstrated better working memory, and their parents rated their memory functions to be better than controls. Performance level before the training was negatively associated with the training gain. Conclusions: These results highlight the importance of cognitive interventions, in particular the teaching of memory strategies, in very preterm-born children at early school age to strengthen cognitive performance and prevent problems at school.

Effects of subthalamic nucleus stimulation on cognition and mood in Parkinson’s disease (PD)

Objective: To assess the neuropsychological outcome as a safety measure and quality control in patients with subthalamic nucleus (STN) stimulation for PD. Background: Deep brain stimulation (DBS) is considered a relatively safe treatment used in patients with movement disorders. However, neuropsychological alterations have been reported in patients with STN DBS for PD. Cognition and mood are important determinants of quality of life in PD patients and must be assessed for safety control. Methods: Seventeen consecutive patients (8 women) who underwent STN DBS for PD have been assessed before and 4 months after surgery. Besides motor symptoms (UPDRS-III), mood (Beck Depression Inventory, Hamilton Depression Rating Scale) and neuropsychological aspects, mainly executive functions, have been assessed (mini mental state examination, semantic and phonematic verbal fluency, go-no go test, stroop test, trail making test, tests of alertness and attention, digit span, wordlist learning, praxia, Boston naming test, figure drawing, visual perception). Paired t-tests were used for comparisons before and after surgery. Results: Patients were 61.6±7.8 years old at baseline assessment. All surgeries were performed without major adverse events. Motor symptoms ‘‘on’’ medication remained stable whereas they improved in the ‘‘off’’ condition (p<0.001). Mood was not depressed before surgery and remained unchanged at follow-up. All neuropsychological assessment outcome measures remained stable at follow-up with the exception of semantic verbal fluency and wordlist learning. Semantic verbal fluency decreased by 21±16% (p<0.001) and there was a trend to worse phonematic verbal fluency after surgery (p=0.06). Recall of a list of 10 words was worse after surgery only for the third attempt of recall (13%, p<0.005). Conclusions: Verbal fluency decreased in our patients after STN DBS, as previously reported. The procedure was otherwise safe and did not lead to deterioration of mood.