Motor measures are sensitive to central lesions, but they are also affected by peripheral injury and motivation. The motor skills profiles of proven brain injury clients were compared with the profiles of healthy postconcussion patients. The chief result was a double dissociation: The traumatic brain injury (TBI) group produced a motor dysfunction gradient consistent with upper motor neuron disease, while the compensation-seeking postconcussion group produced a nonphysiologic pattern. Objective measures of behavioral pain and emotional distress did not correlate with the findings. Motor skill deficiencies in postconcussion syndrome (PCS) are probably functional in nature. (JINS, 1996, 2, 477–485.)

The present study examines differences in functional cerebral asymmetries modulated by gonadal steroid hormones during the menstrual cycle in women. Twenty-one right-handed women with regular menstrual cycles performed a double-stream rapid serial visual presentation (RSVP) task, with one stream in each visual field, during the low steroid menses and the high steroid midluteal phase. They were required to detect a target item, and then a probe item, each of which could appear in either stream. If the probe item appeared 200 ms after the target, detection of the probe was impaired—a phenomenon known as the “attentional blink.” This occurred in both streams in the midluteal phase, but only in the right visual field during menses. Thus low steroid levels appeared to restrict the attentional blink to the left hemisphere, while high levels of estradiol and progesterone in the midluteal phase appeared to reduce functional asymmetries by selectively increasing the attentional blink in the right hemisphere. This effect appears to be mediated by estradiol rather than progesterone, and it is compatible with the assumption of a hormone-related suppression of right hemisphere functions during the midluteal phase. (JINS, 2005, 11, 263–272.)

Recent models of impaired awareness in brain injury draw a distinction between metacognitive knowledge of difficulties and online awareness of errors (emergent and anticipatory). We examined performance of 31 Traumatic Brain Injury (TBI) participants and 31 healthy controls using a three-strand approach to assessing awareness. Metacognitive knowledge was assessed with an awareness interview and discrepancy scores on three questionnaires—Patient Competency Rating Scale, Frontal Systems Behavioral Scale and the Cognitive Failures Questionnaire. Online Emergent Awareness was assessed using an online error-monitoring task while participants performed tasks of sustained attention. Online anticipatory awareness was examined using prediction performance on two cognitive tasks. Results indicated that the TBI Low Self-Awareness (SA) group and High SA group did not differ in terms of severity, chronicity or standard neuropsychological tasks but those with Low SA were more likely to exhibit disinhibition, interpersonal problems and more difficulties in total competency. Sustained attention abilities were associated with both types of online awareness (emergent and anticipatory). There was a strong relationship between online emergent and online anticipatory awareness. Metacognitive knowledge did not correlate with the other two measures. This study highlights the necessity in adopting a multidimensional approach to assessing the multifaceted phenomenon of awareness of deficits. (JINS, 2007, 13, 38–49.)

Brain abnormalities have been documented by neuropsychological assessment as well as a variety of neuroimaging techniques in patients with systemic lupus erythematosus (SLE). Conventional neuroimaging in patients with neuropsychiatric disease (NPSLE) typically discloses periventricular white matter (WM) hyperintensities, infarcts, hemorrhages, and cerebral atrophy. In SLE patients with none of these findings, sophisticated neuroimaging techniques have recently supported associations between microstructural WM abnormalities and abnormal attention, executive function, and processing speed. This mild cognitive dysfunction in SLE (MCD-SLE), which may result from early myelinopathy, precedes the more severe cognitive dysfunction of NPSLE, related to more obvious WM and neuronal damage. (JINS, 2011, 17, 385–392)

The clock drawing test (CDT) is a widely used cognitive screening test. It is useful in identifying focal lesions and cognitive deficits in dementia groups. Lately, several studies attempted its use to differentiate between dementia subtypes. Although many studies have examined the CDT in dementia populations, research into the use of clock drawing in frontotemporal dementia (FTD) is limited. We examined quantitative (global) and qualitative (specific error type) differences on the CDT between FTD (n = 36) and Alzheimer's disease (AD; n = 25) patients and controls without dementia (n = 25). Results showed significantly lower overall scores in the dementia groups compared to the control group, whereas FTD patients scored significantly higher than the AD group. On qualitative analysis, the FTD group had fewer stimulus bound responses, conceptual deficits, and spatial or planning errors compared to the AD group. In conclusion, both global and error analysis of the CDT helped discriminate the FTD group from controls and AD patients. (JINS, 2006, 12, 159–165.)

According to the procedural deficit hypothesis (PDH), abnormal development in the procedural memory system could account for the language deficits observed in specific language impairment (SLI). Recent studies have supported this hypothesis by using a serial reaction time (SRT) task, during which a slower learning rate is observed in children with SLI compared to controls. Recently, we obtained contrasting results, demonstrating that children with SLI were able to learn a sequence as quickly and as accurately as controls. These discrepancies could be related to differences in the statistical structure of the SRT sequence between these studies. The aim of this study was to further assess, in a group of 21 children with SLI, the PDH with second-order conditional sequences, which are more difficult to learn than those used in previous studies. Our results show that children with SLI had impaired procedural memory, as evidenced by both longer reaction times and no sign of sequence-specific learning in comparison with typically developing controls. These results are consistent with the PDH proposed by Ullman and Pierpont (2005) and suggest that procedural sequence-learning in SLI children depends on the complexity of the to-be-learned sequence. (JINS, 2013, 19, 1–8)

Psychotic symptoms are common in Alzheimer's disease (AD) and clinicoanatomical and neuropsychological evidence indicate an association between these symptoms and frontal lobe dysfunction. Neurobehaviors associated with frontal dysfunction were assessed in Alzheimer's disease (AD) patients with (n = 20) and without psychotic symptoms (n = 21) matched for mean age, education, gender, and dementia severity. The Frontal Lobe Personality Scale (FLOPs) was completed by patient caregivers to measure behaviors typically associated with frontal dysfunction. Findings indicated that AD patients with psychotic symptoms exhibited significantly greater neurobehavioral dysfunction (FLOPs M = 130.69, SD = 24.70) than AD patients without psychotic symptoms (FLOPs M = 111.10, SD = 25.83). Subscale analyses indicated that psychotic AD patients were more disinhibited (M = 28.28, SD = 7.54) than patients without psychotic symptoms (M = 20.92, SD = 4.9). Findings are consistent with and contribute to previous neuropsychological and clinicoanatomical research suggesting increased frontal dysfunction in AD with psychotic symptoms and lend additional empirical support to subtyping AD based on the presence of psychotic symptoms. Furthermore, findings provide preliminary evidence indicating which specific type of neurobehavioral abnormalities are related to the presence of distressing psychotic symptoms. (JINS, 2000, 6, 815–820.)

There is increasing recognition that set-shifting, a form of cognitive control, is mediated by different neural structures. However, these regions have not yet been carefully identified as many studies do not account for the influence of component processes (e.g., motor speed). We investigated gray matter correlates of set-shifting while controlling for component processes. Using the Design Fluency (DF), Trail Making Test (TMT), and Color Word Interference (CWI) subtests from the Delis-Kaplan Executive Function System (D-KEFS), we investigated the correlation between set-shifting performance and gray matter volume in 160 subjects with neurodegenerative disease, mild cognitive impairment, and healthy older adults using voxel-based morphometry. All three set-shifting tasks correlated with multiple, widespread gray matter regions. After controlling for the component processes, set-shifting performance correlated with focal regions in prefrontal and posterior parietal cortices. We also identified bilateral prefrontal cortex and the right posterior parietal lobe as common sites for set-shifting across the three tasks. There was a high degree of multicollinearity between the set-shifting conditions and the component processes of TMT and CWI, suggesting DF may better isolate set-shifting regions. Overall, these findings highlight the neuroanatomical correlates of set-shifting and the importance of controlling for component processes when investigating complex cognitive tasks. (JINS, 2010, 16, 640–650.)

The Clock Drawing Test (CDT) has been recognized as an effective tool for dementia detection. This study investigated the clock drawing performance of 240 non-demented elderly Korean people with a wide-range of educational levels and 28 patients with mild dementia of the Alzheimer’s type (DAT). We examined the effects of demographic factors, including education, and established norms for the elderly population. We found that the educational attainment and literacy status of older people influenced performance on the CDT significantly (p < .001). Furthermore, qualitative error analysis revealed that normal participants with low educational background committed errors similar to errors of the DAT patients. The DAT patients performed significantly worse than the non-demented participants in the CDT Total score (p < .001). However, the CDT has better criterion validity in participants with more than 6 years of education. In conclusion, the CDT performance in older people who are either illiterate or with 6 or less years of education should be interpreted with caution. Conceptual errors in the CDT can be the result of not only dementia but also lack of education. (JINS, 2010, 16, 1138–1146.)

Lesch-Nyhan disease (LND) is a rare, X-linked genetic disorder that involves the nearly complete absence of an enzyme (hypoxanthine-guanine phosphoribosyltransferase, or HPRT) that is essential for purine salvage. In addition to hyperuricemia, all patients with classic LND suffer from movement disorder and compulsive self-injury, and most have mental retardation. Patients with partial HPRT deficiency (variants) always have hyperuricemia and often have neurologic abnormalities, but do not self-injure and usually are described as having normal intelligence. Here we compare 15 patients with LND to 9 variants and 13 normal adolescents and adults. Testing revealed unambiguous and qualitatively similar cognitive deficits in both patient groups. The variants produced scores that were intermediate between those of patients with LND and normal participants on nearly every cognitive measure. We discuss these findings in terms of what is known about the neuropathology of LND. (JINS, 2001, 7, 805–812.)

Scores on the Boston Naming Test (BNT) are frequently lower for African American when compared with Caucasian adults. Although demographically based norms can mitigate the impact of this discrepancy on the likelihood of erroneous diagnostic impressions, a growing consensus suggests that group norms do not sufficiently address or advance our understanding of the underlying psychometric and sociocultural factors that lead to between-group score discrepancies. Using item response theory and methods to detect differential item functioning (DIF), the current investigation moves beyond comparisons of the summed total score to examine whether the conditional probability of responding correctly to individual BNT items differs between African American and Caucasian adults. Participants included 670 adults age 52 and older who took part in Mayo’s Older Americans and Older African Americans Normative Studies. Under a two-parameter logistic item response theory framework and after correction for the false discovery rate, 12 items where shown to demonstrate DIF. Of these 12 items, 6 (“dominoes,” “escalator,” “muzzle,” “latch,” “tripod,” and “palette”) were also identified in additional analyses using hierarchical logistic regression models and represent the strongest evidence for race/ethnicity-based DIF. These findings afford a finer characterization of the psychometric properties of the BNT and expand our understanding of between-group performance. (JINS, 2009, 15, 758–768.)

Difficulties related to inhibition and set-shifting have been suggested as possible endophenotypes of Attention Deficit Hyperactivity Disorder (ADHD). However, such difficulties have not been consistently found in studies using standard neuropsychological tests. This has been partly explained by the complexity of these tests and the need to include contrast measures which control for more basic functions. The purpose of the present study was to examine whether difficulties related to inhibition and set-shifting in adult ADHD patients could be revealed by the Color Word Interference Test (CWIT) from the Delis Kaplan Executive Function System (D-KEFS). A clinically recruited group of adults with ADHD (n = 60) obtained significantly lower scores than population derived controls (n = 60) on both primary summary (p < .001) and contrast measures (p = .004) of set-shifting. The differences between the groups remained statistically significant after controlling for intellectual function and working memory (p = .003). However, no significant differences between the groups were observed on any measure of inhibition. The study indicates that adults with ADHD have specific difficulties with set-shifting as measured by the CWIT, difficulties that probably also reflect problems related to executive function in their daily life. (JINS, 2012, 18, 1–10)

Although previous studies have shown that individuals with autism spectrum disorder (ASD) excel at visual search, underlying neural mechanisms remain unknown. This study investigated the neurofunctional correlates of visual search in children with ASD and matched typically developing (TD) children, using an event-related functional magnetic resonance imaging design. We used a visual search paradigm, manipulating search difficulty by varying set size (6, 12, or 24 items), distractor composition (heterogeneous or homogeneous) and target presence to identify brain regions associated with efficient and inefficient search. While the ASD group did not evidence accelerated response time (RT) compared with the TD group, they did demonstrate increased search efficiency, as measured by RT by set size slopes. Activation patterns also showed differences between ASD group, which recruited a network including frontal, parietal, and occipital cortices, and the TD group, which showed less extensive activation mostly limited to occipito-temporal regions. Direct comparisons (for both homogeneous and heterogeneous search conditions) revealed greater activation in occipital and frontoparietal regions in ASD than in TD participants. These results suggest that search efficiency in ASD may be related to enhanced discrimination (reflected in occipital activation) and increased top-down modulation of visual attention (associated with frontoparietal activation). (JINS, 2008, 14, 990–1003.)

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.)

Multiple sclerosis (MS) may be associated with impaired perception of facial emotions. However, emotion recognition mediated by bodily postures has never been examined in these patients. Moreover, several studies have suggested a relation between emotion recognition impairments and alexithymia. This is in line with the idea that the ability to recognize emotions requires the individuals to be able to understand their own emotions. Despite a deficit in emotion recognition has been observed in MS patients, the association between impaired emotion recognition and alexithymia has received little attention. The aim of this study was, first, to investigate MS patient’s abilities to recognize emotions mediated by both facial and bodily expressions and, second, to examine whether any observed deficits in emotions recognition could be explained by the presence of alexithymia. Thirty patients with MS and 30 healthy matched controls performed experimental tasks assessing emotion discrimination and recognition of facial expressions and bodily postures. Moreover, they completed questionnaires evaluating alexithymia, depression, and fatigue. First, facial emotion recognition and, to a lesser extent, bodily emotion recognition can be impaired in MS patients. In particular, patients with higher disability showed an impairment in emotion recognition compared with patients with lower disability and controls. Second, their deficit in emotion recognition was not predicted by alexithymia. Instead, the disease’s characteristics and the performance on some cognitive tasks significantly correlated with emotion recognition. Impaired facial emotion recognition is a cognitive signature of MS that is not dependent on alexithymia. (JINS, 2014, 19, 1–11)

Objectives: The Internet is a fundamental tool for completing many different instrumental activities of daily living (IADL), including shopping and banking. Persons with HIV−associated Neurocognitive Disorders (HAND) are at heightened risk for IADL problems, but the extent to which HAND interferes with the performance of Internet-based household IADLs is not known. Methods: Ninety-three individuals with HIV disease, 43 of whom were diagnosed with HAND, and 42 HIV− comparison participants completed Internet-based tests of shopping and banking. Participants used mock credentials to log in to an experimenter-controlled Web site and independently performed a series of typical online shopping (e.g., purchasing household goods) and banking (e.g., transferring funds between accounts) tasks. Results: Individuals with HAND were significantly more likely to fail the online shopping task than neurocognitively normal HIV+ and HIV− participants. HAND was also associated with poorer overall performance versus HIV+ normals on the online banking task. In the HAND group, Internet-based task scores were correlated with episodic memory, executive functions, motor skills, and numeracy. In the HIV+ sample as a whole, lower Internet-based task scores were uniquely associated with poorer performance-based functional capacity and self-reported declines in shopping and financial management in daily life, but not with global manifest functional status. Conclusions: Findings indicate that HAND is associated with difficulties in using the Internet to complete important household everyday functioning tasks. The development and validation of effective Internet training and compensatory strategies may help to improve the household management of persons with HAND. (JINS, 2017, 23, 605–615)

Hispanics are the fastest growing ethnicity in the United States, yet there are limited well-validated neuropsychological tools in Spanish, and an even greater paucity of normative standards representing this population. The Spanish NIH Toolbox Cognition Battery (NIHTB-CB) is a novel neurocognitive screener; however, the original norms were developed combining Spanish- and English-versions of the battery. We developed normative standards for the Spanish NIHTB-CB, fully adjusting for demographic variables and based entirely on a Spanish-speaking sample. A total of 408 Spanish-speaking neurologically healthy adults (ages 18–85 years) and 496 children (ages 3–7 years) completed the NIH Toolbox norming project. We developed three types of scores: uncorrected based on the entire Spanish-speaking cohort, age-corrected, and fully demographically corrected (age, education, sex) scores for each of the seven NIHTB-CB tests and three composites (Fluid, Crystallized, Total Composites). Corrected scores were developed using polynomial regression models. Demographic factors demonstrated medium-to-large effects on uncorrected NIHTB-CB scores in a pattern that differed from that observed on the English NIHTB-CB. For example, in Spanish-speaking adults, education was more strongly associated with Fluid scores, but showed the strongest association with Crystallized scores among English-speaking adults. Demographic factors were no longer associated with fully corrected scores. The original norms were not successful in eliminating demographic effects, overestimating children’s performances, and underestimating adults’ performances on the Spanish NIHTB-CB. The disparate pattern of demographic associations on the Spanish versus English NIHTB-CB supports the need for distinct normative standards developed separately for each population. Fully adjusted scores presented here will aid in more accurately characterizing acquired brain dysfunction among U.S. Spanish-speakers. (JINS, 2016, 21, 364–374)

Cultural differences in time attitudes and their effect on timed neuropsychological test performance were examined in matched non-clinical samples of 100 Russian and American adult volunteers using 8 tests that were previously reported to be relatively free of cultural bias: Color Trails Test (CTT); Ruff Figural Fluency Test (RFFT); Symbol Digit Modalities Test (SDMT); and Tower of London-Drexel Edition (ToLDx). A measure of time attitudes, the Culture of Time Inventory (COTI-33) was used to assess time attitudes potentially affecting time-limited testing. Americans significantly outscored Russians on CTT, SDMT, and ToLDx (p < .05) while differences in RFFT scores only approached statistical significance. Group differences also emerged in COTI-33 factor scores, which partially mediated differences in performance on CTT-1, SDMT, and ToLDx initiation time, but did not account for the effect of culture on CTT-2. Significant effect of culture was revealed in ratings of familiarity with testing procedures that was negatively related to CTT, ToLDx, and SDMT scores. Current findings indicated that attitudes toward time may influence results of time limited testing and suggested that individuals who lack familiarity with timed testing procedures tend to obtain lower scores on timed tests. (JINS, 2011, 17, 692–701)

Objectives: The aim of this study was to identify whether the three main primary progressive aphasia (PPA) variants would show differential profiles on measures of visuospatial cognition. We hypothesized that the logopenic variant would have the most difficulty across tasks requiring visuospatial and visual memory abilities. Methods: PPA patients (n=156), diagnosed using current criteria, and controls were tested on a battery of tests tapping different aspects of visuospatial cognition. We compared the groups on an overall visuospatial factor; construction, immediate recall, delayed recall, and executive functioning composites; and on individual tests. Cross-sectional and longitudinal comparisons were made, adjusted for disease severity, age, and education. Results: The logopenic variant had significantly lower scores on the visuospatial factor and the most impaired scores on all composites. The nonfluent variant had significant difficulty on all visuospatial composites except the delayed recall, which differentiated them from the logopenic variant. In contrast, the semantic variants performed poorly only on delayed recall of visual information. The logopenic and nonfluent variants showed decline in figure copying performance over time, whereas in the semantic variant, this skill was remarkably preserved. Conclusions: This extensive examination of performance on visuospatial tasks in the PPA variants solidifies some previous findings, for example, delayed recall of visual stimuli adds value in differential diagnosis between logopenic variant PPA and nonfluent variant PPA variants, and illuminates the possibility of common mechanisms that underlie both linguistic and non-linguistic deficits in the variants. Furthermore, this is the first study that has investigated visuospatial functioning over time in the PPA variants. (JINS, 2018, 24, 259–268)