Intraindividual variability of physical status and affect/beliefs as well as their relations with cognition were examined in 3 groups of older adults: healthy elderly, individuals with a nonneurological health-related disturbance (arthritis) and people with neurological compromise (dementia). The findings showed that greater inconsistency in physical performance was observed in groups characterized by central nervous system dysfunction. By contrast, fluctuations in affect appeared to reflect other more transient sources, such as pain. In general, increased inconsistency in non-cognitive domains was associated with poorer cognitive function. There were cross-domain links between inconsistency in physical functioning and fluctuations in cognitive performance, although the nature of the links depended largely upon the neurological status of the individuals. Considered together, the result indicated that measures of cognitive as well as physical variability are important behavioral markers of neurological integrity. (JINS, 2002, 8, 893–906.)

Rapid eye movement (REM) sleep behavior disorder (RBD) is a sleep disturbance that commonly occurs in Dementia with Lewy bodies (DLB). Retrospective examination of DLB course has shown that RBD and cognitive decline may precede the onset of parkinsonism and visual hallucinations. Therefore, some patients with DLB may initially present with dementia and RBD, but would not meet current formal criteria for probable DLB at that time. The purpose of this study is to determine whether patients with dementia and RBD, who do not have parkinsonism or visual hallucinations, have cognitive profiles that can be distinguished from autopsy-confirmed definite AD, but not from clinically probable DLB. If so, this would support the hypothesis that the presence of RBD and dementia, as the only presenting symptoms, reflects the early manifestation of DLB. Results show that early dementia in probable DLB and dementia with RBD are neuropsychologically indistinguishable. Both groups differ from definite AD of a similar early stage with significantly worse visual perceptual organization, sequencing and letter fluency but significantly better confrontation naming and verbal memory. In addition, follow-up data from a subset of patients with dementia and RBD reveal the subsequent development of parkinsonism or hallucinations 1 to 6 years later. Results indicate that the presentation of dementia and RBD is suggestive of underlying Lewy body disease and not Alzheimer's disease. This provides further evidence in support of including RBD as one of the core diagnostic features of DLB. (JINS, 2002, 8, 907–914.)

Prospective remembering has been relatively underinvestigated in neurological patients. This paper describes a group study in which the prospective memory performance of 36 people with brain injury and 28 control participants is compared. We used a new instrument, the Cambridge Behaviour Prospective Memory Test (CBPMT) to assess prospective memory. This comprises 4 time-based and 4 event-based tasks. Participants were allowed to take notes to help them remember the tasks. The relationships between CBPMT scores, scores on formal tests and subjective reports on memory, attention and executive functioning were analyzed. The key findings were that (1) note-taking significantly benefited prospective memory performance, (2) significant relationships were found between scores on the prospective memory test and scores on tests of memory and executive functions, and (3) participants had more difficulty with the time-based than with the event-based prospective memory tasks. The results suggest that compensatory strategies improve prospective memory functioning; memory for content as well as attention and executive functioning processes are involved in prospective memory; and that time-based tasks are more difficult than event-based tasks because they place higher demands on inhibitory control mechanisms. Discussion focuses on the implications of these results for neuropsychological assessment and rehabilitation.

A growing number of studies show postconcussion syndrome (PCS) symptom report is influenced by factors other than head injury, suggesting symptoms typically associated with PCS may not be specific to head injury. Given the role that symptom expectation has been hypothesized to play in PCS symptom etiology, a comparison of symptoms expected for various disorders seems overdue. The present study asked 82 undergraduates to report the symptoms they currently experience, and then to report the symptoms they would expect to experience if they had had suffered either a head injury, an orthopedic injury, posttraumatic stress, or depression. No current differences in overall symptoms or in symptom subscales emerged. Results showed individuals portraying head injury, posttraumatic stress, and depression expected an increase in total symptoms, though individuals portraying an orthopedic injury did not expect such an increase. Results also showed simulators of head injury, posttraumatic stress, and depression expected equivalent rates of overall symptoms, memory/cognitive complaints, somatic concerns, and distracter symptoms, though head-injured individuals reported fewer affective symptoms than those portraying psychological disorders. In all, these findings suggest that individuals have a relative lack of specificity in symptom expectation for various disorders, with the implication that symptom checklists for “PCS” may not be useful for diagnosis. (JINS, 2002, 8, 37–47.)

The Homophone Meaning Generation Test (HMGT; Warrington, 2000) is a new measure of verbal fluency that has been demonstrated to be sensitive to the presence of anterior lesions. In the present study we used the HMGT healthy standardization sample (N = 170) and demonstrate that scores on the HMGT do not differ significantly from a normal distribution and that the test has adequate reliability (α = .82). A table for obtaining confidence limits on an individual's score is presented. A regression equation for the estimation of premorbid HMGT performance was constructed using the National Adult Reading Test as the predictor variable. In a sample of 36 cases with anterior lesions estimated premorbid scores were significantly higher than obtained scores (p < .001). Premorbid ability acted to suppress group differences on the HMGT; the partial correlation between neurological status (healthy vs. anterior lesion) and HMGT performance controlling for premorbid ability (.53) was significantly higher than the raw correlation (.44). In addition, hierarchical discriminant function analysis demonstrated that the inclusion of premorbid ability improved classification over that achieved by HMGT scores alone. These results support both the underlying rationale and the clinical utility of controlling for premorbid performance when interpreting verbal fluency scores. (JINS, 2002, 8, 547–554.)

This study tests the hypothesis that retrieval of object and action names declines at different rates with age. Uncued and cued performance on the Boston Naming Test (BNT) and the Action Naming Test (ANT) were examined for 171 individuals from 50 to 88 years old. To control for differences in item difficulty, a subset of items from each of the two tests was selected for which uncued performance was equivalent in individuals in their 50s. With this matched set of items, differences in action and object naming were tested in the 60s and 70+ age groups. Although age-related decline in name retrieval was observed for both the BNT and the ANT subsets, no differences between object and action retrieval were found. Our results, thus, do not confirm previous studies reporting that object names and action names are differentially retrieved with aging. We discuss these new findings in relation to evidence of dissociations in object and action naming in brain-damaged individuals. (JINS, 2002, 8, 764–770.)

Apathy is a common behavioral disturbance in patients with Alzheimer's disease (AD). Recent studies have linked the presence of apathy to alterations in frontal lobe functions, but few studies have explored the relationship using standard neuropsychological measures in patients with AD. We administered a comprehensive battery of neuropsychological tests and a behavior rating scale to 80 patients with AD. We explored the relationship of apathy to executive dysfunction. AD patients with apathy performed significantly worse on tests of executive function (WAIS–R Digit Symbol, Trail-Making, Stroop Color Interference Test) than AD patients without apathy. The presence of dysphoria did not modify these results and no significant relationships were found between tests of executive functions and dysphoria. Performance on executive measures as a group were effective in correctly classifying patients as apathetic or nonapathetic with 75% accuracy. Neuropsychological measures not dependent on executive functions were unrelated to apathy. Apathy is associated with executive dysfunction and not with other neuropsychological deficits. Apathy is distinct from dysphoria. (JINS, 2002, 8, 373–381.)

Past studies find that attention deficit hyperactivity disorder (ADHD) creates a higher risk for adverse driving outcomes. This study comprehensively evaluated driving in adults with ADHD by comparing 105 young adults with the disorder (age 17–28) to 64 community control (CC) adults on five domains of driving ability and a battery of executive function tasks. The ADHD group self-reported significantly more traffic citations, particularly for speeding, vehicular crashes, and license suspensions than the CC group, with most of these differences corroborated in the official DMV records. Cognitively, the ADHD group was less attentive and made more errors during a visual reaction task under rule-reversed conditions than the CC group. The ADHD group also obtained lower scores on a test of driving rules and decision-making but not on a simple driving simulator. Both self- and other-ratings showed the CC group employed safer routine driving habits than the ADHD group. Relationships between the cognitive and driving measures and the adverse outcomes were limited or absent, calling into question their use in screening ADHD adults for driving risks. Several executive functions also were significantly yet modestly related to accident frequency and total traffic violations after controlling for severity of ADHD. These results are consistent with earlier studies showing significant driving problems are associated with ADHD. This study found that these driving difficulties were not a function of comorbid oppositional defiant disorder, depression, anxiety, or frequency of alcohol or illegal drug use. Findings to date argue for the development of interventions to reduce driving risks among adults with ADHD.

A veritable explosion of research in neuropsychology has occurred over the past decade in the search for cognitive and brain changes during a so-called “preclinical” phase of dementia that precedes its overt clinical manifestations. Fueling this explosion, in part, has been the revolution in the genetic bases of disease formulated from the international work of decoding the human genome (International Human Genome Sequencing Consortium, 2001; see also Patenaude et al., 2002, for discussion). The discovery of preclinical cognitive, brain, and genetic markers of dementia is helping to push back the point at which diseases can be reliably identified. Very early detection of dementia is extremely important now that a variety of investigational treatments that might prevent or delay disease progression (e.g., amyloid vaccine, anti-oxidants, nonsteroidal anti-inflammatory agents, cholinesterase inhibitors, estrogens, and others in the case of Alzheimer's disease) are on the horizon.

Deficits in planning, self-regulation and attention are a relatively common consequence of traumatic brain injury (TBI). Such “dysexecutive” deficits tend to be most exposed in complex, real world situations. Consequently, clinicians often have to rely on interviews, questionnaires and observation in their assessments. While there is little doubt that dysexecutive symptoms occur across different cultures, the expression of those symptoms, the way in which they are experienced by others, and the propensity of friends/relatives to report negative features may vary considerably. The cross-cultural use of standardized checklists and measures that have predominantly been studied with English speaking, Western groups therefore requires empirical support. Here a group of 68 healthy Chinese speaking volunteers were asked to complete translations of 2 UK developed questionnaires (the Dysexecutive Questionnaire and Cognitive Failures Questionnaire) measures and to perform 2 “executive” tasks (The Six Elements Test and the Tower of Hanoi). Their self ratings and the ratings of close relatives were very close to those seen in the original UK standardization samples—as was their performance on the 2 tasks. Accordingly, the conditions for assessing their clinical sensitivity were met. Comparison between 30 Chinese patients with TBI and matched controls showed that both questionnaires and tests were sensitive to the deficits in this group. (JINS, 2002, 8, 771–780.)

Findings from a recent population-based survey indicate that about 33% of patients with dementia reside alone. Because many of these patients may not have a caregiver who visits them regularly, the need for a neuropsychological (NP) test to predict patients' functional competence to live alone safely is evident. In this study, we compared the accuracy of predicting Instrumental and Basic Activities (IADLs and ADLs) of 22 patients with Alzheimer's disease using several standard NP tests and the newly developed Problems in Everyday Living (PEDL) test. Performance of IADLs and ADLs as rated by caregivers was significantly correlated with performance on the PEDL, the Mini-Mental State Exam (MMSE), and with the Shipley Institute of Living Test of Verbal Abstraction (SILS-A), but not with vocabulary or naming. The PEDL was the best predictor of IADL scores (r = 0.71), compared to the MMSE (r = 0.52) and the SILS-A (r = 0.57), while the MMSE was the best predictor of ADL performance (r = 0.69), compared to the PEDL (r = 0.58) and the SILS-A (r = 0.50). (JINS, 2002, 8, 48–57.)

The present study of right hemisphere stroke patients showed that presence of visuospatial neglect in conventional neglect tests at the postacute stage was strongly associated with an aberrant search pattern in a verbally reported visuo-perceptual scanning test. Compared with normal controls, patients with visuospatial neglect showed a greater proportion of repeated readings of the same target, shorter search sequences, more shifts between horizontal, vertical, and diagonal search, and lower proportion of horizontal search. The relation between spatial neglect and a deficient search pattern was strongly influenced by the asymmetric allocation of attention in the scanning test, with the exception for the proportion of repeated reading which was not influenced by this asymmetry. At follow-up, a significant recovery was noted in the neglect group for the proportion of repeated readings and for the asymmetry in the allocation of attention. However, a high number of omitted targets in the search test was still a common finding in the neglect group and it was suggested that a non-lateralized attentional deficit may have played an important role behind the ineffective search at this point of time. (JINS, 2002, 8, 382–394.)

This study examined the interrater reliability of neuropsychological diagnoses produced by clinical neuropsychologists across 4 medical centers. These diagnoses were based on evaluations using a comprehensive battery of commonly used neuropsychological test instruments, interview, history and medical chart review. The diagnoses of individual neuropsychologists were compared to those made by members of an external review panel for each patient evaluated. Patients were first diagnosed as showing cognitive impairment versus no cognitive impairment. If a patient was diagnosed as impaired, a specific neuropsychological diagnosis was assigned. The diagnostic classification for cognitive impairment was moderately reliable [κ = .48 ± s.e.(κ) = .062]. The interrater reliability for specific diagnoses was in the fair to good range [κ = .44 ± s.e.(κ) = .029]. These levels of reliability are comparable to those found for other psychiatric and neurologic specialties and for medical diagnoses made by other health care disciplines. (JINS, 2002, 8, 555–565.)

Recovery of cognitive function after mild head injury (MHI) is thought to be relatively swift and complete. The present study replicates and extends previous work in which university students with self-reported concussion demonstrated reduced P300 amplitude on a set of easy and difficult attention tasks, in addition to performing more poorly than controls on demanding cognitive tasks many years after injury. In the present study, 13 students with self-reported concussion (MHI group: M time since injury 5 8 years) and 10 controls were matched for age, sex, education, and a variety of cognitive, physical and emotional complaints. Controls outperformed the MHI group on the Digit Symbol substitution task and on a difficult dual task involving tone discrimination and visual working memory. Additionally, controls exhibited larger P300 amplitudes on both an easy and a difficult auditory discrimination task. A combination of electrophysiological, neuropsychological and self-report indices predicted group membership (MHI vs. control) with 88% accuracy. The present results, coupled with previous work, offer preliminary evidence that the combination of event-related potentials and demanding behavioral measures might reveal long-lasting, subtle cognitive problems associated with MHI. These findings may challenge existing notions of complete recovery after MHI.

This review summarizes the clinical, imaging, and pathological features of frontotemporal dementia (FTD). Clinicians have become increasingly sensitive to FTD in the differential diagnosis of Alzheimer's disease. Clinical subgroups of FTD patients have been recognized, including patients with progressive non-fluent aphasia, semantic dementia, and behavioral disorder with executive difficulty. The clinical, neuroimaging and neuropathological profiles associated with these clinically defined subgroups are examined. (JINS, 2002, 8, 566–583.)

Long-term memory (LTM) is one of the diverse cognitive functions adversely affected by multiple sclerosis (MS). The LTM deficits have often been attributed to failure of retrieval, whereas encoding processes are presumed intact. However, support for this view comes primarily from studies in which encoding and retrieval operations were not investigated systematically. In the current study, we used an encoding specificity paradigm to examine the robustness of encoding in MS and to specifically evaluate the impact of the disease on contextual memory. We hypothesized that persons with MS would exhibit a selective impairment in retrieving items from LTM when required to generate new cue-target associations at encoding, but not when cues held a strong preexisting relationship to the targets. The findings supported the hypotheses. We conclude that the mnemonic deficits associated with MS affect both encoding and retrieval. Specifically, problems with binding of contextual information at encoding impair effective retrieval of memories. Nonetheless, access to these memories can be gained through preexisting associations organized in the semantic network. (JINS, 2002, 8, 395–409.)

We evaluated neurocognitive function in 149 HIV-seropositive and 82 seronegative women enrolled in the Women's Interagency HIV Study (WIHS), a large multi-center study of disease progression in women living with HIV/AIDS. We evaluated the prevalence of abnormal neuropsychological (NP) test findings in HIV-seropositive and seronegative women and factors associated with increased risk of abnormal NP test performance. Risk of NP impairment was no higher for HIV positive women receiving antiretroviral therapy at testing than for HIV-negative women (OR = 1.00). However, the risk of abnormal NP performance increased significantly for seropositive women not receiving antiretroviral therapy (OR = 2.43). Further, treatment status was a significant predictor of NP impairment in a multivariate analysis that included viral load (OR = 1.48) and CD4 count (OR = 1.08) which were not significant. The multivariate analyses controlled for substance use, age, education, head injury, ethnicity, estimated IQ, and psychological distress. This study emphasizes the critical association of antiretroviral therapy with the risk of neurocognitive impairment in women living with HIV/AIDS. (JINS, 2002, 8, 781–793.)

Subjects underwent longitudinal neuropsychological assessment in order to retrospectively determine which measures of cognitive function best predicted later development of dementia of the Alzheimer type (DAT). Three groups of subjects were studied: normal controls, patients with early DAT, and questionable dementia subjects (QD). All subjects were assessed using a battery of standard neuropsychological measures and two subtests from the Cambridge Neuropsychological Test Automated Battery (CANTAB), paired associate learning and delayed matching to sample. A structured interview was also used to elicit a profile of the subject's daily functioning. Subjects were assessed every 6 months for 2 years. At the 6 month assessment, almost half of the QD group exhibited significant deterioration in scores on the computerized paired associate learning subtest, while maintaining their scores on standard measures. At the conclusion of the study, all of this QD subgroup fulfilled the NINCDS–ADRDA criteria for probable DAT pertaining to significant cognitive and functional deterioration. Performance on the CANTAB paired associate learning subtest identified the onset of progressive memory deterioration in a subgroup of QD subjects. In almost all cases this was well before significant deterioration was noted on standard neuropsychological measures. Paired associate learning performance may therefore be a valuable tool for the early, preclinical detection and assessment of DAT. (JINS, 2002, 8, 58–71.)

A triplet repeat (CAG) expansion mutation in the huntingtin gene on chromosome 4 is responsible for Huntington's disease (HD). Presymptomatic genetic testing for this mutation has identified clinically normal persons who are virtually certain to develop this dementing illness if they live a normal lifespan. The present study sought to determine whether these “mutation-positive” persons have impairments in cognitive functioning. Seventy-five mutation-positive persons did not differ from 128 mutation-negative persons on tests selected for their sensitivity to early-stage HD. Interestingly, however, those with the mutation viewed themselves as more likely to develop HD than did those without the mutation. Among mutation-positive subjects, having a longer CAG repeat mutation was likewise not associated with cognitive impairment. However, being closer to estimated disease onset (a product of repeat length and parent's age at onset) was associated with selected cognitive impairments. When viewed in light of previous studies showing atrophy of the caudate nucleus and putamen in mutation-carriers who are close to onset but not those far from onset, these results suggest that subtle changes in brain and behavior may be detected shortly before subjects with the HD mutation develop sufficient signs and symptoms for diagnosis. Conceptual and methodological problems associated with the search for presymptomatic cognitive and behavioral indicators of dementing illness are discussed. (JINS, 2002, 8, 918–924.)