Klinefelter syndrome (KS) is a sex chromosome abnormality associated with male infertility and mild cognitive deficits. Individuals with KS have been reported to have impaired verbal ability, as well as deficits in executive function. To further understand the nature of their deficits, we assessed specific elements of frontal lobe function such as working memory and relational reasoning. Men with KS exhibited a deficit in a transitive inference task in which participants ordered a set of names based on a list of propositions about the relative heights of the people named. This deficit was present even for items in which the propositions were given in order, so a chaining strategy could be used. Men with KS are also impaired on the n-back task, which uses letters as stimuli. In contrast, these men performed as well as controls in nonverbal reasoning (Raven's Progressive Matrices). These results suggest that men with KS have intact nonverbal reasoning abilities, but that a difficulty in encoding verbal information into working memory may underlie their executive and linguistic impairments. (JINS, 2003, 9, 839–846.)

The presence of a persistent postconcussion symptom complex (PPCSC) was examined in a non-referred sample of male veterans with a history of mild head injury and a comparison group without a history of head injury. Hierarchical logistic regression procedures were used to determine possible predictors of PPCSC using variables supported by previous research (i.e., preexisting psychiatric difficulties, demographic and social support variables, and history of an accidental injurious event). Although PPCSC was common in all groups (23% of the total sample), a significantly greater proportion of individuals in the mild head injury with loss of consciousness group (37.2%) had PPCSC compared with three other groups (head injury without loss of consciousness = 26.1%; motor vehicle accident without head injury = 23%; and control = 17.3%). However, the most salient predictors of PPCSC were early life psychiatric difficulties such as anxiety or depression, limited social support, lower intelligence, and interactions among these variables. The predictive value of loss of consciousness was significant, but low (1.4% of unique variance). The findings provide support for the premise that PPCSC is mediated in part by individual resilience, preexisting psychological status, and psychosocial support. (JINS, 2003, 9, 1001–1015.)

Hepatitis C virus (HCV) infection is a major public-health-care problem, with over 170 million infected worldwide. Patients with chronic HCV infection often complain of various cognitive problems as well as symptoms of depression, anxiety, and fatigue. Relatively little is known, however, about the specific cognitive deficits that are common among HCV patients, and the influence of psychiatric symptomatology on cognitive functioning. In the current study of 21 chronically infected HCV patients, we assessed subjective cognitive dysfunction, depression, anxiety, and fatigue and compared these symptom areas to cognitive tests assessing visuoconstruction, learning, memory, visual attention, psychomotor speed, and mental flexibility. Results revealed that cognitive impairment ranged from 9% of patients on a visuoconstruction task to 38% of patients on a measure of complex attention, visual scanning and tracking, and psychomotor speed, and greater HCV disease severity as indicated by liver fibrosis was associated with greater cognitive dysfunction. Objective cognitive impairment was not related to subjective cognitive complaints or psychiatric symptomatology. These findings suggest that a significant portion of patients with chronic HCV experience cognitive difficulties that may interfere with activities of daily living and quality of life. Future research using cognitive measures with HCV-infected patients may assist researchers in identifying if there is a direct effect of HCV infection on the brain and which patients may be more likely to progress to cirrhosis and hepatic encephalopathy. (JINS, 2003, 9, 847–854.)

Myelomeningocele is a common developmental malformation of the central nervous system that usually results in motor deficits. Previous studies of myelomeningocele have not examined motor adaptation, which involves changes in the control of movements that occur as a result of repeated task exposure but do not depend on conscious recall of the exposure. We studied motor adaptation in 17 children with myelomeningocele and shunted hydrocephalus, 19 children with attention deficit/hyperactivity disorder (ADHD), and 20 healthy siblings. All children were 8 to 15 years of age. They were administered 2 measures of motor adaptation known to be sensitive to subcortical abnormalities in adult neurological disorders. One task assessed the biasing in weight judgments that occurs after exposure to heavy versus light weights, and the other assessed the adaptation in reaching movements that occurs when vision is laterally displaced by prisms. Contrary to expectations, the groups did not differ in motor adaptation. Children in all 3 groups displayed significant biasing in their weight judgments and improvement in the accuracy of pointing during prism adaptation trials. Performance on the 2 motor adaptation tasks was not related to age or IQ. Weight biasing was positively related to a measure of response disinhibition. The findings suggest that myelomeningocele does not result in global impairment of motor skills, but instead in a profile of intact and impaired motor functions that potentially may be decomposed in accordance with the neuroscience of motor skills. (JINS, 2003, 9, 642–652.)

A referred cohort of 67 clinically defined PPA patients were compared to 99 AD patients with formal language and nonverbal cognitive tests in a case control design. Language fluency was determined at the first and last follow up visits. Quantitation of sulcal and ventricular atrophy on MRI was carried out in 46 PPA and 53 AD patients. Most PPA patients (57%) are relatively fluent when first examined. Visuospatial and memory functions are initially preserved. Aphemic, stuttering, “pure motor” presentation, or agrammatic aphasia are seen less frequently. Later most PPAs become logopenic and nonfluent, even those with semantic aphasia (dementia). In contrast, AD patients were more fluent and had relatively lower comprehension, but better overall language performance. MRI showed significant left sided atrophy in most PPA patients. Subsequent to PPA, 25 patients developed behavioral manifestations of frontotemporal dementia and 15 the corticobasal degeneration syndrome, indicating the substantial clinical overlap of these conditions. Language testing, particularly fluency scores supported by neuroimaging are helpful differentiating PPA from AD. The fluent–nonfluent dichotomy in PPA is mostly stage related. The aphemic-logopenic-agrammatic and semantic distinction is useful, but the outcomes converge. (JINS, 2003, 9, 710–719.)

Shallice and Burgess (1991) reported the utility of the Multiple Errands Test (MET) in discriminating executive deficits in three frontal lobe patients with preserved high IQ, who were otherwise unimpaired on tests of executive function. The aim of this study was to ascertain the value of a simplified version of the MET (MET–SV) for use with the range of people more routinely encountered in clinical practice. Main findings were as follows: 1) The test discriminated well between neurological patients and controls, and the group effects remained when the difference in current general cognitive functions (WAIS–R FSIQ) was taken into account. 2) The best predictors of performance in the healthy control group (n = 46) were age and the number of times participants asked for help (with more requests associated with poorer performance). 3) In the neurological group, two clear patterns of failure emerged, with performance either characterized by rule breaking or failure to achieve tasks. These two patterns were associated with different dysexecutive symptoms in everyday life. 4) The patients not only made more errors than controls, but also different ones. A scoring method that took this into account markedly increased test sensitivity. 5) Many patients passed traditional tests of executive frontal lobe function but still failed the MET–SV. This pattern was strongly associated with observed dysexecutive symptoms in everyday life. The results demonstrate the clinical utility of the test, and suggest that there are two common and independent sources of failure on multitasking tests in a general neurological population: memory dysfunction, and initiation problems. (JINS, 2003, 9, 31–44.)

The purpose of this study was to investigate and extend previously reported sex differences in object location memory by comparing the performance of heterosexual and homosexual males and females. Subjects were 240 healthy, right-handed heterosexual and homosexual males and females. They were instructed to study 16 common, gender-neutral objects arranged randomly in an array and subsequently tested for object recall, object recognition and spatial location memory. Females recalled significantly more objects than males, although there were no group differences in object recognition. Decomposition of significant interactions between sex and sexual orientation on spatial location memory (controlling for differences in object recall, age and IQ) revealed that heterosexual females and homosexual males scored better than heterosexual males, and no different from each other. There were no differences between homosexual and heterosexual females. The findings suggest that homosexual males and heterosexual females encode, store and retrieve positional and relational information about spatial layouts similarly, pointing to within-sex variations in the neural architecture underlying spatial memory. (JINS, 2003, 9, 376–383.)

Test scores from a comprehensive neuropsychological battery administered to 1602 subjects consisting of 1347 subjects with probable Alzheimer's disease (AD), 100 subjects with questionable dementia (QD) and 155 non-demented elderly control subjects were cross-sectionally analyzed. Subjects with probable AD were categorized as mild (n = 244), moderate (n = 480), severe (n = 376), and very severe (n = 247) according to modified mini mental status exam (mMMSE) scores. Mean scores on individual neuropsychological tests are provided for each group of subjects. Stratified random sampling was performed to select a sample of mild AD subjects who were matched in age and education to non-demented elderly controls, and analyses focused on the performance of QD subjects and mild AD subjects, whose scores were compared to those of the elderly control subjects. Selected scores were organized by cognitive domain and logistic regressions were used to determine the domains and individual tests within each that were most predictive of group status. Results suggested a profile of scores associated with QD and mild AD including impaired recall of verbal information for both groups. Areas of lower functioning in QD subjects as compared to elderly controls included category fluency and visuospatial ability. (JINS, 2003, 9, 720–732.)

The present study aimed to examine the clinical validity and applicability of the Chinese version of Mattis Dementia Rating Scale (CDRS) for elderly Chinese individuals. The scale was found to have good reliability with internal consistency ranging from 0.7 to 0.9. Its significant correlation with the Chinese version of Mini-Mental State Examination (CMMSE) suggested satisfactory construct validity of the scale. The discriminant validity of the CDRS in differentiating Alzheimer's disease (AD) patients and Normal Control (NC) elderly was also supported by the Receiver Operating Characteristics (ROC) analysis. Several cut-off points were presented for different clinical or research applications, and formulas to adjust for the age and educational level of the CDRS total score were provided. The Initiation/Perseveration and Memory subscales were suggested to be an abbreviated version of the CDRS for a quick screening. The present results also showed the association between the impairment of the CDRS performance and the progress of the AD. (JINS, 2003, 9, 45–55.)

We examined the relationship between nocturnal respiratory disturbance and learning and compared learning in children with and without nocturnal respiratory disturbance. Subjects were 149 participants in a prospective cohort study examining sleep in children ages 6–12: The Tucson Children's Assessment of Sleep Apnea study (TuCASA). Sleep was assessed via home polysomnography. Intelligence, learning and memory, and academic achievement were assessed. Parents rated attention. Group comparisons were used to test the hypothesis that the group with an apnea/hypopnea index (AHI) of 5 or more (n = 77) would have weaker performance than the group with AHI less than 5 (n = 72). The group with AHI of 5 or more had weaker learning and memory though differences between groups decreased when arousals were taken into account. There was a greater percentage of Stage 1 sleep in the AHI 5 or more group, and Stage 1 percentage was negatively related to learning and memory in the sample (n = 149). There were negative relationships between AHI and immediate recall, Full Scale IQ, Performance IQ, and math achievement. Hypoxemia was associated with lower Performance IQ. Thus, findings suggest that nocturnal respiratory disturbance is associated with decreased learning in otherwise healthy children, that sleep fragmentation adversely impacts learning and memory, and that hypoxemia adversely influences nonverbal skills. (JINS, 2003, 9, 1016–1026.)

The importance of humor in healthy aging is being recognized. We compared elderly and young participants on their comprehension and appreciation of, and reaction to, verbal and nonverbal humor tests. Cognitive processes—working memory, cognitive flexibility, verbal abstraction, and visual scanning—were studied in relation to humor. Results indicated a relative deficit in the elderly in the cognitive comprehension of humor—selecting punch lines to jokes and in a cartoon array test. Measures of cognitive function correlated with humor comprehension. In contrast to this deficit in comprehension, the elderly showed intact affective appreciation and emotional reactiveness. Because of the hypothesis of frontal lobe degeneration as a basis for changes with aging, we compared the elderly to patients with focal frontal lesions. In this comparison, the elderly were significantly better than the patients in their comprehension of humor. They also displayed intact appreciation of humor compared to patients with frontal lesions. This preliminary study suggests that preserved affective responsiveness may underlie the success in using humor as a coping mechanism in the elderly. (JINS, 2003, 9, 855–863.)

Animal word list generation (ANWLG) was administered to 47 first-episode schizophrenia (FES) participants and 31 controls. Fifty-nine left temporal lobe epilepsy (LTLE) participants were included as a comparison group with known temporal lobe damage and expected semantic deficits. Semantic knowledge was assessed with the Association Index (AI), a measure of the semantic relatedness of all consecutive ANWLG responses. Neuropsychological tests of language and executive functioning were also administered. Results showed that both FES and LTLE groups generated fewer ANWLG responses than controls, but only the LTLE participants obtained a lower AI relative to controls. FES participants did not differ from controls on the AI. FES and LTLE groups produced fewer semantic subcategories (clusters), however, only the LTLE group produced fewer words per subcategory compared to controls (cluster size). FES participants produced a higher rate of perseverative responses compared to the other groups. Finally, correlation analyses showed that for FES participants both executive and language tests significantly correlated with ANWLG total responses, while the correlation between ANWLG and only 1 language test was significant for LTLE participants. Taken together, the results suggest that reduced ANWLG output in FES participants may be best conceptualized as a deficit in the executive component of word list generation (i.e., semantic search/access, response monitoring) or global cognitive impairment. (JINS, 2003, 9, 384–393.)

As a group, children with myelomeningocele (MM) and early-onset hydrocephalus demonstrate many of the features of the syndrome of nonverbal learning disabilities (NLD). However, the frequency with which individual children display a pattern of neuropsychological functioning consistent with the NLD syndrome is unknown. We addressed this question by comparing the prevalence of NLD in 32 children with MM and shunted hydrocephalus to that in a group of 27 healthy siblings. Participants, who were between 8 and 15 years of age, completed a neuropsychological test battery that included 11 measures of possible assets and 17 measures of possible deficits that define the NLD syndrome. As a group, children with MM and shunted hydrocephalus displayed many of the specific assets and deficits. However, they also displayed significantly more variability in their patterns of assets and deficits than siblings, reflecting the substantial individual differences that characterize children with MM. About 50% of the children with myelomeningocele displayed a pattern of assets and deficits consistent with the NLD syndrome. Classification as NLD was weakly related to cumulative medical risk, as well as to left-handedness. The findings suggest a need for caution in making generalizations regarding the applicability of the NLD model to children with MM and early-onset hydrocephalus. (JINS, 2003, 9, 653–662.)

Neuropsychological deficits are considered by many to be core features of schizophrenia. However, about 20% of patients with schizophrenia appear to have normal neuropsychological function. This study investigates this subgroup by comparing a “neuropsychologically normal” schizophrenia group to a non-schizophrenic, non-brain damaged patient comparison (PC) sample, and to patients with definitive brain damage who performed normally on neuropsychological testing. All patients completed the Halstead-Reitan Neuropsychological Test Battery and were classified as neuropsychologically normal or impaired using the Average Impairment Rating (AIR). In a sample of 113 patients with schizophrenia, 19.5% were classified as neuropsychologically normal. The brain damaged neuropsychologically normal group (BD-NN) consisted of 14.3% of 124 subjects. These groups were compared with a patient non-schizophrenic, non-brain damaged group who were selected on the basis of having an Average Impairment Rating in the neuropsychologically normal range. The neuropsychologically normal schizophrenic group performed less well than the non-brain damaged, non-schizophrenic patient comparison group on a number of tests, indicating that patients in this group may not be completely neuropsychologically normal, and would be better characterized as “high-functioning” or near normal. The results are discussed in regard to possible neurobiological differences between neuropsychologically impaired and intact schizophrenic patients, and implications for course and outcome. (JINS, 2003, 9, 56–63.)

Previous research has demonstrated dissociations between categorization and recognition performance in amnesic patients, supporting the idea that separate memory systems govern these tasks. However, previous research has also demonstrated that these dissociations are predicted by a single-system model that allows for reasonable parameter differences across groups. Generally, previous studies have employed categorization tasks that are less demanding than the recognition tasks. In this study, we distinguish between single-system and multiple-system accounts by testing memory-impaired individuals in a more demanding categorization task. These patients, just like previous amnesic participants, show a dissociation between categorization and recognition when tested in previously employed paradigms. However, they display a categorization deficit when tested in the more challenging categorization task. The results are interpreted as support for a single-system framework in which categorization and recognition depend on one representational system. (JINS, 2003, 9, 394–406.)

Behavioral neurologists and neuropsychologists have debated the role of the thalamus and basal ganglia in cognition and behavior for more than a century (e.g., Bucy, 1942; Marie, 1906; Penfield & Roberts, 1959; Wernicke, 1874). However, over these 100-plus years, there is little consensus regarding whether or how these structures contribute to cognition. Fortunately, recent research findings are rapidly changing this state of affairs. It is now obvious we will not understand how the brain controls complex activities until we understand the contribution of these deep brain structures. In healthy and brain-damaged individuals, application of methodologies such as semantic priming, event related potentials, and functional neuroimaging to the question of subcortical functions is beginning to resolve this conundrum. This symposium demonstrates the utility of combining these different approaches. It features empirical work from six laboratories that have engaged in systematic inquiries regarding the role of the thalamus and basal ganglia in cognition. This body of work represents both new directions and convergence of recent findings in the quest to integrate our understanding of this complex issue.

The coexistence of posttraumatic stress disorder (PTSD) and traumatic head or brain injury (TBI) in the same individual has been proposed to be paradoxical. It has been argued that individuals who sustain a TBI and have no conscious memory of their trauma will not experience fear, helplessness and horror during the trauma, nor will they develop reexperiencing symptoms or establish the negative associations that underlie avoidance symptoms. However, single case reports and incidence studies suggest that PTSD can be diagnosed following TBI. We highlight critical issues in assessment, definitions, and research methods, and propose two possible resolutions of the paradox. One resolution focuses on ambiguity in the criteria for diagnosing PTSD. The other involves accepting that TBI patients do experience similar symptoms to other PTSD patients, but that there are crucial differences in symptom content. (JINS, 2003, 9, 663–676.)

It is well established that some individuals with temporal lobe epilepsy (TLE) demonstrate language deficits at the single word level. However, discourse production rarely has been examined quantitatively within this group. This study compared adult TLE patients with an early seizure onset (≤ age 14 years, n = 27) to a control group (n = 28) on narrative and procedural discourse tasks. As a group, the TLE patients performed normally on the procedural discourse task, but differed significantly from the controls on several narrative discourse variables. At the individual level, 30% of the TLE patients versus 4% of the controls demonstrated impaired discourse ability (p < .01). Within this early onset TLE group, discourse performance was not associated with demographic or seizure history variables. Considering the cognitive domain, discourse performance correlated significantly with working memory. In summary, mild discourse dysfunction was present in a significant minority of early onset TLE patients, but this deficit was not closely associated with other language measures. Discourse ability and its neuropsychological, neuroanatomical and conversational speech correlates deserve further study in TLE patients. (JINS, 2003, 9, 733–739.)

Neuropsychologists often diagnose cerebral dysfunction based, in part, on marked variation in an individual's cognitive test performance. However, little is known about what constitutes the normal range of intraindividual variation. In this study, after excluding 54 individuals with significant health problems, we derived 32 z-transformed scores from 15 tests administered to 197 adult participants in a study of normal aging. The difference between each person's highest and lowest scores was computed to assess his or her maximum discrepancy (MD). The resulting MD values ranged from 1.6 to 6.1 meaning that the smallest MD shown by any person was 1.6 standard deviations (SDs) and the largest MD shown by any person was 6.1 SDs. Sixty-six percent of participants produced MD values that exceeded 3 SDs. Eliminating each person's highest and lowest test scores decreased their MDs, but 27% of the participants still produced MD values exceeding 3. Although MD values appeared to increase with age, adjusting test scores for age, which is standard in clinical practice, did not correct for this. These data reveal that marked intraindividual variability is very common in normal adults, and underscore the need to base diagnostic inferences on clinically recognizable patterns rather than psychometric variability alone. (JINS, 2003, 9, 864–870.)