Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation

OBJECTIVE: Objective information about the onset and progression of cognitive impairment in Huntington's disease (HD) is very important in the light of appropriate outcome measures when conducting clinical trials. Therefore, we evaluated the progression of cognitive functions in HD patients and asymptomatic carriers of the HD mutation (AC) over a 2.5-year period.We also sought to detect the earliest markers of cognitive impairment in AC. METHODS: A prospective study comparing HD patients, clinically asymptomatic HD mutation-carriers (AC) and non-carriers (NC). These groups were examined three times during a period of 2.5 years. At baseline the study sample consisted of 49 subjects. Forty-two subjects (19 HD patients, 12 AC and 11 NC) completed three assessments. A battery of neuropsychological tests measuring intelligence, attention, memory, language, visuospatial perception, and executive functions was performed. RESULTS: The performance of HD patients deteriorated on the following cognitive tests: Symbol Digit Modalities Test (SDMT), Stroop Colour and Word, Boston Naming Test (BNT), Object and Space Perception and Trail Making Test-B. Longitudinal comparison of AC and NC revealed that performances on SDMT, Block Span, Digit Span Backwards, Hopkins Verbal Learning Test (learning and delayed recall) and Conditional Associative Learning Test are impaired in AC. CONCLUSIONS: Tasks measuring mainly attention, object and space perception and executive functions adequately assess the progression of HD disease. Other cognitive functions do not significantly deteriorate. Furthermore, problems in attention, working memory, verbal learning, verbal long-term memory and learning of random associations are the earliest cognitive manifestations in AC.     

Cognitive performance in transient global hypoxic brain injury due to moderate drowning

Introduction: Drowning is a serious and frequently neglected public health threat. Primary respiratory impairment after submersion often leads to brain dysfunction. Depending on the period of global hypoxia (respiratory failure), clinical aspects of neurological dysfunction are evident on the first evaluation after the water rescue. Nowadays, many neuropsychological assessments after drowning are inconclusive, with some studies reporting only minor neurological or cognitive impairments. The aim of this study is to identify measures in neuropsychological tests that most contribute to classify volunteers as moderate drowning subjects or healthy controls. To the best of our knowledge, this study is the first neuropsychological prospective case-control study of moderate drowning in a country with large coastal cities. Method: Fifteen moderate drowning patients (DP), who met the inclusion criteria, were compared with 18 healthy controls (HC). All subjects were assessed on memory, learning, visual spatial ability, executive function, attention, and general intellectual functioning and underwent structural magnetic resonance (MR) imaging of the brain at 3.0 T, in order to exclude subjects with anatomic abnormalities. Results: Neuropsychological tests assessing learning, execution function, and verbal fluency—Rey Auditory Verbal Learning Test (RAVLT) general learning ability, Digit Span total, Phonological Verbal Fluency (total FAS correct), and Brief Visuospatial Memory Test Revised (BVMT) correct recognition—have the strongest discriminating ability, using predictive models via the partial least squares (PLS) approach for data classification, while the other tests have shown similar predictive values between groups. Conclusions: Learning, execution function, and verbal fluency domains were the most critically affected domains. Serious impairments in the same domains have already been reported in severe drowning cases, and we hypothesize that subtle alterations found in moderate drowning cases, although not sufficient to be detected in daily routine, may possibly have a negative impact on cognitive reserve.     

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.     

Utility of behavioral versus cognitive measures in differentiating between subtypes of frontotemporal lobar degeneration and Alzheimer's disease

We hypothesized that a modified version of the Frontal Behavioral Inventory (FBI-mod), along with a few cognitive tests, would be clinically useful in distinguishing between clinically defined Alzheimer's disease (AD) and subtypes of frontotemporal lobar degeneration (FTLD): frontotemporal dementia (dysexecutive type), progressive nonfluent aphasia, and semantic dementia. We studied 80 patients who were diagnosed with AD (n = 30) or FTLD (n = 50), on the basis of a comprehensive neuropsychological battery, imaging, neurological examination, and history. We found significant between-group differences on the FBI-mod, two subtests of the Rey Auditory Verbal Learning Test (verbal learning and delayed recall), and the Trail Making Test Part B (one measure of 'executive functioning'). AD was characterized by relatively severe impairment in verbal learning, delayed recall, and executive functioning, with relatively normal scores on the FBI-mod. Frontotemporal dementia was characterized by relatively severe impairment on the FBI-mod and executive functioning in the absence of severe impairment in verbal learning and recall. Progressive nonfluent aphasia was characterized by severe impairment in executive functioning with relatively normal scores on verbal learning and recall and FBI-mod. Finally, semantic dementia was characterized by relatively severe deficits in delayed recall, but relatively normal performance on new learning, executive functioning, and on FBI-mod. Discriminant function analysis confirmed that the FBI-mod, in conjunction with the Rey Auditory Verbal Learning Test, and the Trail Making Test Part B categorized the majority of patients as subtypes of FTLD or AD in the same way as a full neuropsychological battery, neurological examination, complete history, and imaging. These tests may be useful for efficient clinical diagnosis, although progressive nonfluent aphasia and semantic dementia are likely to be best distinguished by language tests not included in standard neuropsychological test batteries.     

Exploring the relation between learning style and cognitive impairment in patients with acquired brain injury

The way a patient prefers to approach or choose a learning situation represents the patient's learning style. The objective of this chart review study was to explore the relation between learning style and cognitive impairment in patients with acquired brain injury (ABI). We used data from files of 92 adult patients with ABI referred to inpatient rehabilitation, who completed the Adapted Learning Style Inventory (A-LSI) and at least one of the following neuropsychological tests: Trail Making Test, Rey Auditory Verbal Learning Test, WAIS-III Digit Span, Rey-Osterrieth Complex Figure Test-Copy, Stroop Color-Word Test, or the Brixton Spatial Anticipation Test. The A-LSI yielded the following distribution of learning styles: 4 doers, 48 observers, 2 deciders and 38 thinkers. No significant correlation coefficients were found between the neuropsychological tests and the A-LSI. Furthermore, Chi-square tests revealed no significant associations between learning style (observer, thinker) and cognitive impairment. The results of this exploratory study suggest that learning style and cognitive impairment are independent in patients with ABI.     

Only subtle cognitive deficits in non–bulbar amyotrophic lateral sclerosis patients

Neuropsychological investigations of amyotrophic sclerosis (ALS) patients revealed considerable discrepancies regarding neurocognitive functions. Some, but not all studies have suggested executive dysfunctioning and memory impairment, and there is a wide range of applied neuropsychological tests and results. In this study, we investigated the neuropsychological performance of 15 non–bulbar ALS patients, 14 patients with neuromuscular symptoms, and 15 healthy controls. To avoid confounding effects of motor disability, performance was assessed using exclusively motor–free tests of frontal lobe functioning (specific memory functions, conditional–associative learning, attention, and executive functions). ALS patients exhibited poorer performance in two conditions (semantic and alternating condition, respectively) of the Verbal Fluency Test, suggesting a subtle executive deficit. No deficits were found in tests of memory, conditionalassociative learning, or attention. Assessed mood status was not related to neuropsychological performance.Verbal memory (CVLT) and verbal fluency (lexical condition) were positively associated with duration of disease. Our results support the view that there are only subtle cognitive deficits in ALS patients and we assume a possible effect of practice on cognitive tasks following reduced daily motor activity.     

Confabulation behavior and false memories in Korsakoff's syndrome: role of source memory and executive functioning

Aims:  Confabulation behavior is common in patients with Korsakoff's syndrome. A distinction can be made between spontaneous and provoked confabulations, which may have different underlying cognitive mechanisms. Provoked confabulations may be related to intrusions on memory tests, whereas spontaneous confabulations may be due to executive dysfunction or a source memory deficit.
Methods:  In 19 chronic Korsakoff patients, spontaneous confabulations were quantified by third-party rating (Likert scale). Provoked confabulations were assessed using the Dalla Barba Confabulation Battery. Furthermore, assessment of executive function was performed using an extensive neuropsychological battery. False memories (i.e. intrusions) and source memory were measured using twoparallelversions of a word-list learning paradigm (a modification of the Rey Auditory Verbal Learning Test).
Results:  There were deficits in source memory, in which patients incorrectly assigned previously learned words to an incorrect word list. Also, Korsakoff patients had extensive executive deficits, but no relationship between the severity of these deficits and the severity of confabulation or intrusions on a memory task was found.
Conclusion:  The present findings provide evidence for a dissociation between spontaneous confabulation, provoked confabulation and false memories.     

精神分裂症首次发病患者及其健康同胞认知功能的比较研究

目的 比较精神分裂症首次发病患者与健康同胞及正常对照认知功能的差异,探讨精神分裂症在认知功能领域的内表型.方法 采用目前常用的范畴流畅测验(CFT)、数字符号编码测验(DSCT)、连线测验(TMT)、韦克斯勒记忆量表第3版(WMS-Ⅲ)空间广度测验(WMS-ⅢSST)、霍普金斯词汇学习测验-修订版(HVLT-R)、简易视觉空间记忆测验-修订版(BVMT-R)、定步调听觉连续加法测验(PASAT)和威斯康星卡片分类测验-64(WCST-64)对92例精神分裂症首次发病患者(患者组)、56例健康同胞(同胞组)和62名健康对照者(对照组)的认知功能进行检测.结果 (1)患者组所有神经心理测验成绩均差于对照组,差异有统计学意义(P＜0.01).(2)同胞组的CFT、DSCT、TMT、HVLT-R即刻记忆和延迟记忆、BVMT-R即刻记忆、PASAT、WCST-64持续错误数、持续反应数和完成分类数的测验成绩差于对照组,差异有统计学意义(P＜0.05).(3)患者组与同胞组的CFT、WCST-64中的持续错误数、持续反应数和完成分类数测验成绩分别为(18.40±12.12)分比( 18.86±5.19)分、(16.48±8.19)分比(14.80±5.86)分、(18.76±10.91)分比(16.86 ±7.73)分、(1.33±2.81)分比(1.63±1.36)分,2组比较差异无统计学意义(P＞0.05),其他神经心理测验成绩比较,患者组差于同胞组,差异有统计学意义(P＜0.05).结论 精神分裂症首次发病患者存在处理速度、工作记忆、言语记忆、空间记忆、注意警觉和执行功能广泛性的认知功能损害,精神分裂症健康同胞存在处理速度、言语记忆、视觉记忆、注意警觉、执行功能的认知缺陷;语义流畅性功能和执行功能可能是精神分裂症的潜在内表型.     

The performance of individuals with mental retardation on cognitive tests assessing effort and motivation

The purpose of this study was to determine which tests of effort and motivation would be appropriate for use with patients with mental retardation when feigning of cognitive deficits is suspected. The seven measures evaluated included the WMS-III Rarely Missed Index Test, forced-choice recognition portion of the California Verbal Learning Test-II, Reliable Digit Span test, Rey 15-Item Test, Rey Dot Counting Test, the Rey 15-Item Test with Recognition Trial, and the Vocabulary (V)-Digit Span (DS) difference score. Results indicated that the forced-choice portion of the CVLT-II, the V-DS difference score, and the Rarely Missed Index Test from the WMS-III might be appropriate for use with this population with passing rates of 89%, 98%, and 91% respectively.     

The Performance of Individuals with Mental Retardation on Cognitive Tests Assessing Effort and Motivation

The purpose of this study was to determine which tests of effort and motivation would be appropriate for use with patients with mental retardation when feigning of cognitive deficits is suspected. The seven measures evaluated included the WMS-III Rarely Missed Index Test, forced-choice recognition portion of the California Verbal Learning Test-II, Reliable Digit Span test, Rey 15-Item Test, Rey Dot Counting Test, the Rey 15-Item Test with Recognition Trial, and the Vocabulary (V)-Digit Span (DS) difference score. Results indicated that the forced-choice portion of the CVLT-II, the V-DS difference score, and the Rarely Missed Index Test from the WMS-III might be appropriate for use with this population with passing rates of 89%, 98%, and 91% respectively.     

Executive functions and memory in autogenous and reactive subtype of obsessive-compulsive disorder patients

There are concurrently with different results of studies about cognitive functions of Obsessive-Compulsive Disorder (OCD), impairment in non-verbal memory and executive functioning in OCD, has shown consistent results in several studies. In this study, 62 OCD patients and 40 healthy controls were participated. Firstly, cognitive functions of OCD group and healthy control group were compared in terms of scores in Stroop Test, Wisconsin Cart Sorting Test (WCST), Auditory Consonant Trigram Test (ACTT), Controlled Word Association Test (CWAT), Rey Auditory Verbal Learning Test (RAVLT), Digit Span Test (DST). And then, two patient groups of OCD patients (patients with autogenous obsessions and patients with reactive obsessions) were compared in terms of the scores of same tests, with a hypothesis that claims, cognitive functions of patients with autogenous obsessions, who shown schizotypal personality features and thought disorder in higher ratio, will show more impairment than cognitive functions of patients with reactive obsessions. Significant impairment was found in OCD patients in terms of Stroop test and WCST scores when compared to scores of healthy controls. There was no difference pointed out between cognitive functions of patients with autogenous obsessions and reactive obsessions. Due to limited number of patients with autogenous obsessions in current study, any future research with greater sample size will be helpful to explain the cognitive functions in OCD with autogenous and reactive obsessions.     

Evaluation of neuropsychological functions in patients with Friedreich ataxia before and after cognitive therapy

Friedreich ataxia (FA) is a neurodegenerative disease characterized by progressive nervous system damage resulting in severe disability. Cognitive functions and mood disorders in FA have been studied little and with conflicting results. The aim of this study was to investigate cognitive functions and mood disorders in FA subjects and the role of cognitive rehabilitation therapy (sequential treatments) performed during a scheduled study period. The executive functions of 24 subjects with FA were evaluated over one year during three separate periods of in-hospital rehabilitation. The neuropsychological evaluations performed before and after cognitive therapy did not reveal differences in the mean test scores of the MMSE, the Rey 15-item Memorization Test for long-term memory, Raven's Colored Progressive Matrices, the Phonemic Verbal Fluency Test, the Symbol Digit Modalities Test, or the Zung scale. The mean scores of the Stroop color-word interference task and of the Rey 15-item Memorization Test for short-term memory were increased at the final evaluation. This finding of long-lasting stability of neuropsychological test scores is noteworthy, as it suggests that one-year cognitive rehabilitation therapy (sequential treatments) may at least contribute to reducing cognitive decline. A cognitive rehabilitation therapy in addition to the conventional neuromotor rehabilitation treatment may improve the management of subjects with FA.     

Cognitive functioning related to quality of life in schizophrenia

The present study compared the cognitive function of patients with schizophrenia to that of healthy subjects, and investigated the relationships between cognitive function and quality of life (QOL). Participants consisted of 53 patients meeting DSM-IV criteria for schizophrenia and 31 normal controls. All participants completed a neuropsychological test battery assessing executive function, verbal memory, and social knowledge. QOL was rated using the Schizophrenia Quality of Life Scale. Patients with schizophrenia showed lower performance across various cognitive measures of memory, including the Sentence Memory Test, the Verbal Learning Test, and the Script Test, as well as the Rule Shift Cards Test of executive function. Multiple regression analyses were used to evaluate the neuropsychological measures and clinical symptoms to predict QOL. The QOL total score, the social initiative score or the empathy score were significantly predicted by the Script or/and the Sentence Memory. Neuropsychological functioning was unrelated to most QOL scores in the presence of clinical symptoms, while ability of empathy in the QOL was predicted by performance of the Sentence Memory Test. These results demonstrated patients with schizophrenia have deficits in executive function, memory and learning, and social knowledge, and that social knowledge and memory are related to QOL. Thus, in patients with schizophrenia, deficits in social knowledge appear to be associated with current QOL in general, and specifically with the capacity for empathy and social initiative.     

Prospects for faking believable memory deficits on neuropsychological tests and the use of incentives in simulation research

The vulnerability of several neuropsychological memory tests--the Wechsler Memory Scale--Revised, Complex Figure Test, Auditory Verbal Learning Test, and Rey Memory Test--to faked deficits was evaluated. Subjects were randomly assigned to one of three conditions: Control (n = 28), Malingering with a financial incentive (n = 30), and Malingering without a financial incentive (n = 28). Overall, the performance of the Malingering Groups was not significantly different from one another, but both groups were significantly poorer than Controls. Discriminant functions with a reduced set of predictors correctly classified about 75% of cases in both the Control and the combined Malingering Groups on cross-validation. The results suggest that neuropsychological memory tests are vulnerable to faked deficits, and that recognition tasks are disproportionately affected. The use of incentives in simulation research is also discussed.     

Neurocognitive impairment in patients with co-occurring bipolar disorder and alcohol dependence upon discharge from inpatient care

The current study explored the neurocognitive functioning of patients with co-occurring bipolar disorder and alcohol dependence upon discharge from inpatient care. The study compared scores of neuropsychological tests among three groups of bipolar I inpatients without a history of neurological injury or illness: 1) patients meeting DSM-IV diagnostic criteria for alcohol dependence in the past 6 months (n=13), 2) patients diagnosed with alcohol dependence in full remission (n=9), and 3) patients without a history of a substance use disorder (SUD; n=41). Analyses indicated that patients with co-occurring alcohol dependence exhibited more severe impairment on tests of executive functioning (i.e. Stroop Color-Word Interference Test, Wisconsin Card Sorting Test) than patients without SUD. In addition, the group meeting diagnostic criteria for alcohol dependence in the past 6 months exhibited greater decrements in verbal (California Verbal Learning Test--II) and visual (Rey Complex Figure Test) memory. Analysis further indicated that patients in full SUD remission scored lower on measures of fluid intelligence (Wechsler Abbreviated Scale of Intelligence--Performance IQ). Consistent with previous reports, in the current sample, co-occurring alcohol dependence predicted higher rates of disability status. It is possible that cognitive deficits of greater severity in dually diagnosed patients contribute to this unfavorable outcome. Recognizing the extent of cognitive impairment in dually diagnosed patients may facilitate the effort to ameliorate their condition.     

A neuropsychological investigation of prefrontal cortex involvement in acute mania

OBJECTIVE: Mania has received little attention from a contemporary neuropsychological perspective despite its clear resemblance to the disinhibition syndrome sometimes seen after frontal brain injury, particularly injury to the inferior aspect of the prefrontal cortex. The purpose of this investigation was to describe the neuropsychological profile of severe acute mania by using a range of tasks selected primarily for the detection of localized neural disruption within the prefrontal cortex. METHOD: Fifteen acutely manic inpatients were compared with 30 nonpsychiatric subjects on tasks from the Cambridge Automated Neuropsychological Test Battery (Tower of London, spatial working memory, intradimensional-extradimensional attentional shift, and rapid visual information processing tasks) and on the Iowa Gambling Task, Stroop Color and Word Test, a verbal fluency task, and the California Verbal Learning Test. RESULTS: Discriminant function analysis identified deficits in sustained attention (on the rapid visual information processing task) and verbal learning (on the California Verbal Learning Test) as the best indicators of manic performance, rather than deficits on any of the tests of executive functioning. The model correctly classified 91% of subjects overall and 87% of manic subjects. Manic patients did not resemble patients with ventromedial prefrontal cortex damage in their performance on the Iowa Gambling Task. CONCLUSIONS: Acute mania is characterized by core deficits in verbal memory and sustained attention against a background of milder impairments in functions that are traditional measures of prefrontal cortex integrity (attentional set shifting, planning, working memory). The data do not implicate ventral prefrontal cortex disruption as a locus of pathology in acute mania. Verbal memory and sustained attention deficits may relate differentially to the state and trait characteristics of bipolar disorder.     

Cognitive functioning in young people with first episode psychosis: relationship to diagnosis and clinical characteristics

OBJECTIVE: To examine the extent and nature of neuropsychological deficits in adolescents and young people with first episode psychosis (FEP), and to determine whether the pattern and extent of neuropsychological deficits varied according to diagnosis. METHOD: A total of 83 FEP subjects aged 13-25 years, and 31 healthy controls completed a comprehensive battery of neuropsychological tests, grouped into 10 cognitive domains. First episode psychosis subjects were stratified into three diagnostic groups (schizophrenia, affective disorders, substance-induced psychosis) and differences in cognitive profiles were examined. The contribution of demographic and clinical characteristics to cognitive performance was also explored. RESULTS: The schizophrenia group demonstrated significantly worse performance on tasks of verbal learning and memory than the affective disorders group. Compared to healthy controls, the schizophrenia group also demonstrated global impairment across the majority of cognitive domains. The substance-induced group's performance lay between that of the schizophrenia and affective disorders groups. Analyses of differential deficits revealed that verbal learning, verbal memory and current intellectual functioning were selectively impaired in the schizophrenia group, whereas the affective disorders group demonstrated a selective deficit in speeded processing. Premorbid intellectual functioning, negative symptomatology and medication levels were the strongest predictors of cognitive performance in FEP subjects. CONCLUSIONS: Verbal memory deficits differentiate individuals with schizophrenia from those with psychotic affective disorders. Although significant cognitive deficits are evident across all diagnostic FEP groups, individuals with schizophrenia appear to have more generalized impairment across a broad array of cognitive functions than other psychotic diagnoses. Lower premorbid intellectual functioning does not appear to contribute to greater cognitive deterioration following onset of psychosis, but severity of illness may be a more important factor than levels of mood disturbance.     

Cognitive Function in Breast Cancer Survivors Compared to Healthy Age- and Education-Matched Women

The cognitive function of breast cancer survivors (BC, n = 52) and individually matched healthy controls (n = 52) was compared on a battery of sensitive neuropsychological tests. The BC group endorsed significantly higher levels of subjective memory loss and scored significantly worse than controls on learning and delayed recall indices from the Rey Auditory Verbal Learning Test (AVLT). Defining clinically significant impairment as scores at or below the 7th percentile of the control group, the rate of cognitive impairment in the BC sample was 17% for total learning on the AVLT, 17% for delayed recall on the AVLT, and 25% for either measure. Findings indicate that a sizeable percentage of breast cancer survivors have clinically significant cognitive impairment.     

The Neuropsychological Profile of Infantile Duchenne Muscular Dystrophy

It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.     

The neuropsychological profile of infantile Duchenne muscular dystrophy

It has been shown that children with Duchenne muscular dystrophy (DMD) exhibit specific cognitive deficits. However, the neuropsychological profile has not yet been fully characterized. In order to control for the contribution of motor impairments as a confounding variable that is usually present when assessing children with muscular pathologies, we compared children with DMD to a group of children with an autoimmune pathology that does not entail either brain or cognitive dysfunction but does imply motor impairment: juvenile rheumatoid arthritis (JRA). An extensive neuropsychological evaluation was administered, including intelligence, perception, language, memory and learning, attention, executive function, praxis and reasoning. As the main result, in children with DMD we found significantly lower performances in verbal IQ, verbal short-term memory and phonological abilities, as well as in praxis and executive functioning domains. A wide range of cognitive domains, including verbal abilities, executive functioning, and praxis, must be taken into account when assessing neuropsychological functioning in children with DMD. In particular, subtle executive function impairment could be considered as a subclinical marker of cognitive impairment in developmental disorders.