Specific cognitive deficits are common in children with Duchenne muscular dystrophy

A neuropsychological assessment was conducted to study cognition, with emphasis on memory, information processing/learning ability, and executive functions in boys with Duchenne muscular dystrophy (DMD). A group of 20 boys with DMD, aged 7 to 14 years (mean age 9 years 5 months, SD 2 years 2 months), was contrasted with 17 normally developing age-matched comparison individuals, using specific neuropsychological tests (Block Span, Digit Span, Story Recall, Rey Auditory Verbal Learning Test, Rey Complex Figure Test, Spatial Learning Test, Verbal Fluency, Trail Making Test, Tower of London, Memory for Faces, and Raven's Coloured Progressive Matrices). The DMD group performed significantly worse on all aspects of memory, learning, and executive functions. There was no significant difference in general intellectual ability between the two groups. Analyses of group differences indicate that problems in short-term memory are the most apparent, suggesting specific cognitive deficits. The differences between the groups were similar for both verbal-auditory and visuospatial tests, thus contradicting the idea that cognitive deficits are related to type of stimulus presented. It is concluded from this study that short-term memory deficits might play a critical role in the cognitive impairment and intellectual development seen in those with DMD.     

A neuropsychological investigation of male premutation carriers of fragile X syndrome

It is currently thought that fragile X syndrome (FraX; the most common inherited form of learning disability) results from having more than 200 cytosine–guanine–guanine (CGG) trinucleotide repeats, with consequent methylation of the fragile X mental retardation (FMR1) gene and loss of FMR1 protein (FMRP). It was also considered that premutation carriers (with 55–200 CGG repeats) are unaffected, although a tremor/ataxia syndrome has recently been described in older adult male carriers. We reported that premutation expansion of CGG trinucleotide repeats affects brain anatomy, which, together with other studies, indicates that the molecular model for FraX needs modification. However, there are few studies on the cognitive ability of adult male premutation carriers. Thus, we selected 20 male premutation carriers on the basis of their genetic phenotype, and compared them to 20 male controls matched on age, IQ and handedness. We investigated intellectual functioning, executive function, memory, attention, visual and spatial perception, and language and pragmatics. The premutation carriers had significant impairments on tests of executive function (Verbal Fluency, Trail Making Test and Tower of London) and memory (Names sub-test of the Doors and People, Verbal Paired Associates Immediate Recall and Visual Paired Associates Delayed Recall sub-tests of the WMS-R, and Category Fluency Test for natural kinds). We therefore suggest that CGG trinucleotide repeats in the premutation range affect specific neuronal circuits that are concordant with specific neuropsychological deficits; and that these deficits reflect an emerging neuropsychological phenotype of premutation FraX.     

Cognitive profiles in degenerative dementia without evidence of small vessel pathology and small vessel vascular dementia

Although a large number of studies have examined possible differences in cognitive performance between Alzheimer’s disease (AD) and vascular dementia (VaD), the data in the literature are conflicting. The aims of this study were to analyze the neuropsychological pattern of subjects affected by degenerative dementia without evidence of small vessel pathology (DD) and small vessel VaD subjects in the early stages and to investigate differences in the progression of cognitive impairment. Seventy-five patients with probable VaD and 75 patients with probable DD were included. All the subjects underwent a standard neuropsychological evaluation, including the following test: Visual Search, Attentional matrices, Story Recall, Raven’s Coloured Progressive Matrices, Phonological and Semantic Verbal Fluency, Token, and Copying Drawings. The severity of cognitive impairment was stratified according to the MMSE score. Fifteen subjects with probable DD and 10 subjects with probable VaD underwent a 12-month cognitive re-evaluation. No significant difference was found between DD and VaD subjects in any of the neuropsychological tests except Story Recall in the mild cognitive impairment (P < 0.001). The re-test value was significantly worse than the baseline value in the MMSE (P = 0.037), Corsi (P = 0.041), Story Recall (P = 0.032), Phonological Verbal Fluency (P = 0.02), and Copying Drawings (P = 0.043) in DD patients and in the Visual Search test (P = 0.036) in VaD subjects. These results suggest that a neuropsychological evaluation might help to differentiate degenerative dementia without evidence of small vessel pathology from small vessel VaD in the early stages of these diseases.     

Population-based dementia screening program in Kuopio: the effect of education, age, and sex on brief neuropsychological tests.

A neuropsychological screening battery including the Mini-Mental State Examination and four other brief cognitive tests (Russell's Adaptation of the Visual Reproduction Test, Trail Making Test, Verbal Fluency Tests on letters and category, and the Buschke Selective Reminding Test) was administered to a randomly selected population sample of 403 subjects aged 68 to 77 years to evaluate the effect of education, age, and sex on test scores. The difference in neuropsychological screening tests between various education groups (3 years or less, 4 to 6 years, 7 years or more) was statistically highly significant, even after the adjustment for the effect of age. The subscores and total scores were lowest in the minimal education group on every neuropsychological test. Education correlated more strongly than age with all neuropsychological test scores and subscores. The effect of sex on test results was seen only in some subscores of brief neuropsychological tests but not in a single item of the Mini-Mental State Examination. On the basis of our results, the effects of education, age, and sex have to be evaluated before using brief neuropsychological tests in population-based dementia screening.     

Free and cued selective reminding test: Moans norms

Abstract

Age-adjusted norms are presented for persons age 56–98 years on the Free and Cued Selective Reminding Test (FCSRT; Buschke, 1984; Grober & Buschke, 1987). These data were obtained via several research projects that are known collectively as Mayo's Older Americans Normative Studies (MOANS). These norms should be useful when the FCSRT is used as a “stand alone” test. They should also promote accuracy in the comparison of any person's FCSRT performance against his or her functioning on any other tests with MOANS norms. The unique features of the FCSRT are discussed. These norms will, hopefully, enhance the utility of the test when it is used in clinical and research investigations of various amnestic disorders.     

Normative Data for Neuropsychological Tests in a Rural Elderly Chinese Cohort

Normative information is important for appropriate interpretation of cognitive test scores as a critical component of dementia diagnosis in the elderly population. A cross-sectional evaluation of 1826 participants aged 65 years and older from four rural counties in China was conducted using six cognitive instruments including tests of global cognitive function (the Community Screening Instrument for Dementia), memory (Word List Learning and Recall tasks from the Consortium to Establish a Registry for Alzheimer's Disease, IU Story), language (Animal Fluency Test), and executive function (IU Token). Multiple regression models adjusting for demographic variables were used to provide standardized residuals z-scores and corresponding percentile ranking for each cognitive test. In all cognitive tests older age was associated with worse test performance while exposure to education was related to better cognitive test performance. We also detected a significant gender difference with men scoring better than women and a significant gender by education interaction on two tests. The interaction indicates that gender difference in test scores was much smaller in participants with more education than those who had less or no education. These demographically adjusted, regression-based norms can be a useful tool to clinicians involved with differential diagnosis of cognitive and memory disorders in older adults in rural China.     

On the nature of memory disturbance in multiple sclerosis

Abstract

Thirty-seven patients with multiple sclerosis (MS) were compared to 26 normal controls of equivalent age, education, and verbal intelligence on measures of verbal learning and memory (Digit Span and Supraspan, Brown-Peterson Distractor Task, Selective Reminding Test, Story Recall, and Free Verbal Recall) and verbal fluency (Letter and Animal Fluency). The MS patients exhibited deficits on measures of secondary (long-term) memory and verbal fluency, but performed normally on measures of primary (short-term) memory, recognition memory, and rate of forgetting from secondary memory. These results suggest that the memory disturbance in MS results primarily from an impaired ability to access information from secondary memory, while encoding and storage capacity is intact. Degree of memory impairment was unrelated to length of illness, severity of disability, or self-reported depression.     

Two-stage screening for early dementia in primary care

Objective: The objective was to compare two screening strategies for dementia in an urban primary care clinic, serving a low-education, minority community composed largely of Latino and African American patients. Method: Two hundred and fifty-seven patients underwent two-stage patient-based screening (PBS) and informant-based screening (IBS) followed by a diagnostic evaluation. In the first stage, PBS included brief tests of episodic memory (Memory Impairment Screen), semantic memory (Animal Fluency), and executive function (Reciting Months Backwards). For IBS, the first stage consisted of the short Informant Questionnaire on Cognitive Decline in the Elderly, administered to a family member or friend. Patients who screened positive in the first stage of either strategy underwent testing with the picture version of the Free and Cued Selective Reminding Test with Immediate Recall to identify memory impairment. Sensitivity, specificity, and positive and negative predictive values were computed for various cutoffs of each test and combination of tests. Dementia was diagnosed using Diagnostic and Statistical Manual of Mental Disorders–Fourth Edition (DSM–IV) criteria without access to the screening test results. Results: We identified 66 patients (25.7%) with previously undiagnosed dementia. Sensitivity was the same (77%) for both strategies but specificity was higher for IBS than for PBS (92% versus 83%). IBS’s higher specificity makes it the preferred strategy if a knowledgeable informant is available. Conclusion: Unrecognized dementia is common in primary care. Case-finding can be improved using either PBS or IBS two-stage screening strategies.     

Cognitive test scores in community-based older adults with and without dementia

In an epidemiological survey of a rural, largely blue-collar, community, 1,363 randomly selected adults, aged 65 years, were administered a cognitive screening battery (including in part the CERAD neuropsychological tests): Mini-Mental State Examination; Word List Learning, Recall, and Recognition; Story, Immediate and Delayed Recall; Boston Naming Test; Verbal Fluency; Temporal Orientation; Constructional Praxis; Draw a Clock; and Trailmaking. Cognitively impaired subjects and cognitively intact controls underwent independent standardized diagnostic assessments and were rated on Clinical Dementia Rating (CDR) scale. Overall, subjects at higher CDR levels (more severe dementia) had worse scores on all tests; showing that standard neuropsychological tests are valid for characterizing the cognitive impairments seen in dementia, even in community settings. However, non-demented scores on the CERAD tests in this community-based sample were lower than reported from CERAD's pooled healthy controls from Alzheimer's Disease Centers (ADCs) nationwide. Thus, 'normal' scores from specialty dementia clinics, where there may be a selection bias, may differ from normative scores from rural and/or less-educated populations. Patients from such populations may be functionally intact despite low test scores. Community-based studies are required to complement specialty clinic-based studies of dementia and cognitive functioning.     

Cognitive profile of patients with manganese-methcathinone encephalopathy

Manganese-methcathinone encephalopathy (MME) is a rare parkinsonian syndrome described in drug addicts who have self-injected a home-made mixture containing methcathinone and manganese. We assessed 14 patients with MME and compared their results with 14 matched control subjects. The patients had a parkinsonian syndrome with symmetrical bradykinesia, dystonias, and postural, gait and speech impairment, with moderate restrictions in activities of daily living. Their cognitive status was assessed with the Russian version of the Wechsler Adult Intelligence Scale (WAIS) and with tests of attention (Trail Making Test, Bourdon-Wiersma Dot Cancellation Test), memory (Auditory Verbal Learning Test, Rey-Osterrieth Complex Figure), motor skills (Grooved Pegboard), visuospatial skills (Money Road Map Test, Benton Judgment of Line Orientation), and executive abilities (Verbal Fluency, 5-Point Test, Wisconsin Card Sorting Test). Only a few significant differences emerged. After controlling for multiple comparisons, the results in the WAIS Object Assembly subtest, the Grooved Pegboard test (dominant and nondominant hand) and the Verbal Fluency test remained significant.     

Subcortical aphasia: three different language disorder syndromes?

The study analyses clinical presentation of language functions of 32 patients with subcortical aphasia induced by stroke. The patients have been divided into three groups according to neuroanatomic localization of the lesion, defined by CT and MRI examination (striato-capsular aphasia, aphasia associated with white matter paraventricular lesions and thalamic aphasia). The following batteries and tests were used: the neurologic examination, CT scan, MRI, Doppler ultrasound, Mini Mental State Examination, Boston Diagnostic Aphasia Examination (BDAE), Boston Naming Test (BNT), Token Test and Verbal Fluency Test. Clinical presentation of subcortical aphasias is characterized with preserved repetition, however, some groups differ by certain specific features of language impairment. Striato-capsular aphasia and aphasia associated with white matter paraventricular lesions are characterized with lack of speech fluency, occurrence of literary paraphasias, mainly preserved comprehension and naming. Thalamic aphasia, however, is characterized with fluent output, impaired comprehension and naming with predominant verbal paraphasias. The specific features of language impairment suggest that subcortical structures contribute to language organization. Considering the results of language tests we presume that the most prominent feature in striato-capsular aphasia is phonetic impairment of language, opposite to thalamic aphasia where lexical-sematic processing seems to be affected.     

Multistrategic memory training with the metamemory concept in healthy older adults

Objective

According to the increase of older people, the need for effective methods to maintain or improve cognitive functions in the elderly has increased. These cognitive enhancing methods may contribute to the prevention of elderly cognitive decline by aging and dementing illness as well. This study aimed to examine the effectiveness of multistrategic memory training with the metamemory concept on cognitive functions in the normal health elderly in Korea.

Methods

The program used in this study was developed by psychiatrists and psychologists in accordance with Korean situations. We applied the training program to the community-dwelling elderly with subjective memory complaints. Twenty participants were randomly received the intervention with 20 non-treatment controls. This program consisted of 10 sessions and was administered once a week. We examined the effects of this memory training for verbal memory, visuospatial memory, working memory, and verbal fluency ability by repeated ANOVA.

Results

There were significant improvements in Word List Short-term Delayed Free and Cued Recall, Word list Long-term Delayed Free and Cued Recall and visuospatial recognition memory. Performance improvements in visuospatial span forwards and the Categorical Fluency Test were also significant. These improvements were still significant after adjusting for depression improvement exact categorical fluency.

Conclusion

This study shows that multistrategic memory training with the metamemory concept may improve memory ability and other cognitive functions which are not trained and that these improvements may be achieved by pure cognitive training effects.     

Testing Measurement Invariance of the Positive and Negative Affect Schedule (PANAS) in American and Arab University Students

Affect describes any feelings, emotions, or moods that a person experiences and is generally divided into two broad dimensions—positive affect and negative affect. The most widely used measure of affect, the Positive and Negative Affect Schedule (PANAS), has recently been translated to Arabic (see Appendix), yet the psychometric equivalence of this adapted measure is not fully understood. Using a series of multigroup confirmatory factor analyses, the current study examined measurement invariance of the English and adapted Arabic versions of the PANAS among 979 American and 1470 Arab university students. Although the two-factor structure of the 20-item PANAS was observed in both groups (configural variance), results did not support full invariance of factor loadings (metric invariance). A partial metric invariance model, however, revealed invariant loadings for all positive affect items and all but four negative affect items; dissimilar factor loadings emerged between groups for irritable, nervous, scared, and jittery. Evidence did not support scalar invariance of the 16 metric-invariant items, with only ten items demonstrating equivalent intercepts across groups. Finally, tests for strict invariance indicated nine of the ten scalar-invariant items had equivalent residual variances across groups.

All told, results suggest that the PANAS is partially invariant in American and Arab groups.

     

On the nature of memory disturbance in multiple sclerosis

Thirty-seven patients with multiple sclerosis (MS) were compared to 26 normal controls of equivalent age, education, and verbal intelligence on measures of verbal learning and memory (Digit Span and Supraspan, Brown-Peterson Distractor Task, Selective Reminding Test, Story Recall, and Free Verbal Recall) and verbal fluency (Letter and Animal Fluency). The MS patients exhibited deficits on measures of secondary (long-term) memory and verbal fluency, but performed normally on measures of primary (short-term) memory, recognition memory, and rate of forgetting from secondary memory. These results suggest that the memory disturbance in MS results primarily from an imparied ability to access information from secondary memory, while encoding and storage capacity is intact. Degree of memory impairment was unrelated to length of illness, severity of disability, or self-reported depression.     

Atrophy of the left dorsolateral prefrontal cortex is associated with poor performance in verbal fluency in elderly poststroke women

This study aimed to investigate the association between atrophy in the prefrontal cortex with executive function and verbal fluency in elderly male and female patients poststroke. Thirty elderly female patients with non-aphasic ischemic stroke aged ≥ 60 years and 30 age-matched non-aphasic male patients with ischemic stroke were recruited. Automatic magnetic resonance imaging segmentation was used to assess the volume of the whole prefrontal cortex, along with its subdivisions: anterior cingulate cortex, orbitofrontal cortex and dorsolateral prefrontal cortex. The Semantic Verbal Fluency Test was administered at 3 and 15 months poststroke. At 3 months poststroke, left dorsolateral prefrontal cortex volume was significantly correlated with Verbal Fluency Test score in female patients only (partial coefficient = 0.453, P = 0.045), after controlling for age, education, diabetes, neurological deficit, white matter lesions volume, as well as the location and volume of infarcts. At 15 months poststroke, there remained a significant association between the left dorsolateral prefrontal cortex volume and Verbal Fluency Test (partial coefficient = 0.661, P = 0.001) and between the left prefrontal cortex volume and Verbal Fluency Test (partial coefficient = 0.573, P = 0.004) in female patients after the same adjustments. These findings indicate that atrophy of the left dorsolateral prefrontal cortex contributes to the impairment of verbal fluency in elderly female patients with stroke. Sex differences may be present in the neuropsychological mechanisms of verbal fluency impairment in patients with stroke.     

Memory for verbal information in individuals with HIV-associated dementia complex

Abstract

Patterns of memory performance were examined for 9 participants with HIV-associated dementia (HAD), 15 HIV-seropositive participants without dementia, and 15 HIV-seronegative controls. Episodic and semantic memory were assessed using the California Verbal Learning Test, the Boston Naming Test, and Verbal Fluency tests. The HAD group showed deficits in episodic memory, with relative sparing of semantic memory. In addition, results suggest a retrieval deficit in HAD rather than a deficit in retention of information. This pattern is consistent with the presence of a subcortical dementing process and supports findings from previous neuropathological, neuroimaging, and neuropsychological studies suggesting that subcortical brain dysfunction is frequently associated with HIV disease (e. g., Navia, Jordan, & Price, 1986).     

中国农村地区全身强直-阵挛发作癫癎患者的认知功能评估

目的：评价中国农村地区未经抗癫癎药物（AEDs）治疗的全身强直-阵挛发作（GTCS）癫癎患者的认知功能的状况.方法：采用数字广度测验、言语流畅性测验、听觉词语测验及数字划消测验对144例GTCS患者和144名健康对照者进行认知功能评定.结果：GTCS患者的注意力及计算能力较差,并有瞬间语言记忆、自发的语言运动能力、思维组织能力及干扰抑制能力的下降.结论：未经AEDs治疗的GTCS患者存在广泛的认知功能障碍.AEDs的非规范使用是认知功能损害的主要影响因素.     

Association of nonsteroidal anti-inflammatory drugs and aspirin with cognitive performance in middle-aged adults.

OBJECTIVES: To assess the cross-sectional association of regular use of nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin with cognitive function in 13,153 individuals, aged 48-67 years, participating in the Atherosclerosis Risk in Communities (ARIC) Study. METHODS: Regular use of NSAIDs or aspirin was analyzed in relation to the results of three cognitive tests, the Delayed Word Recall Test, the WAIS-R Digit Symbol Subtest, and the Word Fluency Test. RESULTS: After adjustment for covariates previously found to be associated with cognition in this sample, we found a weak negative association between current use of aspirin and poor Word Recall [OR = 0.84 (95% confidence interval: 0.68-1.04)] and poor Word Fluency [OR = 0.85 (0.70-1.03)]. We found no association between current use of aspirin and Digit Symbol score, nor did we find current NSAID use to be significantly associated with any of the cognitive tests. CONCLUSIONS: This study suggests a modest association, at best, between NSAIDs or aspirin and better cognitive function.     

Normative data for neuropsychological tests in a rural elderly Chinese cohort

Normative information is important for appropriate interpretation of cognitive test scores as a critical component of dementia diagnosis in the elderly population. A cross-sectional evaluation of 1826 participants aged 65 years and older from four rural counties in China was conducted using six cognitive instruments including tests of global cognitive function (the Community Screening Instrument for Dementia), memory (Word List Learning and Recall tasks from the Consortium to Establish a Registry for Alzheimer's Disease, IU Story), language (Animal Fluency Test), and executive function (IU Token). Multiple regression models adjusting for demographic variables were used to provide standardized residuals z-scores and corresponding percentile ranking for each cognitive test. In all cognitive tests older age was associated with worse test performance while exposure to education was related to better cognitive test performance. We also detected a significant gender difference with men scoring better than women and a significant gender by education interaction on two tests. The interaction indicates that gender difference in test scores was much smaller in participants with more education than those who had less or no education. These demographically adjusted, regression-based norms can be a useful tool to clinicians involved with differential diagnosis of cognitive and memory disorders in older adults in rural China.     

A Longitudinal Factor Analytic Study of Children’s Neurocognitive Abilities

The neurocognitive abilities of 503 Portuguese children aged 8–11.9 years at onset of the study were assessed annually for 8 years in 3 functional domains: memory, motor and visual motor functions, and attention. A series of exploratory principal axis factor analyses, with varimax rotation, revealed seven factors: Divided Attention, Selective Attention, Verbal Learning and Recall, Visual Learning and Recall, Motor Speed, Visual-Motor Speed, and Working Memory. The same factors emerged across the 8-year period, suggesting that children's neurocognitive structures are articulated and stable throughout this period of development. From these exploratory analyses a small set of cost-effective neuropsychological tests were found to characterize children's functioning consistently throughout the 8-year period.