Performance of patients with schizophrenia (n = 15) was compared to that of patients with vascular ischemic lesions of the dorsolateral prefrontal cortex (n= 10) and to age-matched controls (n= 15) in delayed reaction tasks known to be specific markers of the functions of the frontal lobe. The performance of patients with schizophrenia was similar to that of patients with prefrontal lesions in the delayed response task (DR) and the spatial discrimination-reversal task (SD and RV) but not in the delayed alternation task (DA) in which they performed as well as controls. The behavioural profile of responses in delayed reaction tasks for the schizophrenic patients was identical to that of patients with basal ganglia lesions reported in a previous study. These results suggest that in some patients with schizophrenia, a frontal lobe deficit could be indirectly induced by a dysfunction of the basal ganglia. This problem could be due either to the pathological process itself or to the effect of the neuroleptic treatment.

Objectives: Neurofibromatosis type 1 (NF1) is a genetic disorder in which the most frequent complication in children is learning disabilities. Over the past decade, growing arguments support the idea that executive dysfunction is a core deficit in children with NF1. However, some data remain inconsistent. The aim of this study was to determine the magnitude of impairment for each executive function (EF) and clarify the impact of methodological choices and participant’s characteristics on EFs. Methods: In this meta-analysis, 19 studies met the selection criteria and were included with data from a total of 805 children with NF1 and 667 controls. Based on the Diamond’s model (2013), EF measures were coded separately according to the following EF components: working memory, inhibitory control, cognitive flexibility, planning/problem solving. The review protocol was registered with PROSPERO (International prospective register of systematic reviews; CRD42017068808). Results: A significant executive dysfunction in children with NF1 is demonstrated. Subgroup analysis showed that the impairment varied as a function of the specific component of executive functioning. The effect size for working memory and planning/problem solving was moderate whereas it was small for inhibitory control and cognitive flexibility. Executive dysfunction seems to be greater with increasing age whereas assessment tool type, intellectual performance, attention deficit hyperactivity disorder and control group composition did not seem to affect EF results. Conclusions: EF deficits are a core feature in children with NF1 and an early identification of executive dysfunctions is essential to limit their impact on the quality of life. (JINS, 2018, 24, 977–994)

Objectives: As surprisingly little is known about the developing brain studied in vivo in youth with Down syndrome (DS), the current review summarizes the small DS pediatric structural neuroimaging literature and begins to contextualize existing research within a developmental framework. Methods: A systematic review of the literature was completed, effect sizes from published studies were reviewed, and results are presented with respect to the DS cognitive behavioral phenotype and typical brain development. Results: The majority of DS structural neuroimaging studies describe gross differences in brain morphometry and do not use advanced neuroimaging methods to provide nuanced descriptions of the brain. There is evidence for smaller total brain volume (TBV), total gray matter (GM) and white matter, cortical lobar, hippocampal, and cerebellar volumes. When reductions in TBV are accounted for, specific reductions are noted in subregions of the frontal lobe, temporal lobe, cerebellum, and hippocampus. A review of cortical lobar effect sizes reveals mostly large effect sizes from early childhood through adolescence. However, deviance is smaller in adolescence. Despite these smaller effects, frontal GM continues to be largely deviant in adolescence. An examination of age-frontal GM relations using effect sizes from published studies and data from Lee et al. (2016) reveals that while there is a strong inverse relationship between age and frontal GM volume in controls across childhood and adolescence, this is not observed in DS. Conclusions: Further developmentally focused research, ideally using longitudinal neuroimaging, is needed to elucidate the nature of the DS neuroanatomic phenotype during childhood and adolescence. (JINS, 2018, 24, 966–976)

Our knowledge of the functions of the prefrontal cortex, often called executive, supervisory, or control, has been transformed over the past 50 years. After operationally defining terms for clarification, we review the impact of advances in functional, structural, and theoretical levels of understanding upon neuropsychological assessment practice as a means of identifying 11 principles/challenges relating to assessment of executive function. Three of these were already known 50 years ago, and 8 have been confirmed or emerged since. Key themes over this period have been the emergence of the use of naturalistic tests to address issues of “ecological validity”; discovery of the complexity of the frontal lobe control system; invention of new tests for clinical use; development of key theoretical frameworks that address the issue of the role of prefrontal cortex systems in the organization of human cognition; the move toward considering brain systems rather than brain regions; the advent of functional neuroimaging, and its emerging integration into clinical practice. Despite these huge advances, however, practicing neuropsychologists are still desperately in need of new ways of measuring executive function. We discuss pathways by which this might happen, including decoupling the two levels of explanation (information processing; brain structure) and integrating very recent technological advances into the neuropsychologist’s toolbox. (JINS, 2017, 23, 755–767)

Objectives: Although it has been well documented that traumatic brain injury (TBI) can result in communication impairment, little work to date has examined the relationship between social communication skills and structural brain integrity in patients with TBI. The aim of the current study was to investigate the association between self- and other-perceived communication problems and white matter integrity in patients with mild to severe TBI. Methods: Forty-four individuals (TBI=24) and people with whom they frequently communicate, as well as demographically matched normal healthy comparisons (NC) and their frequent communication partners, were administered, respectively, the La-Trobe Communication Questionnaire Self form (LCQ-SELF) and Other form (LCQ-OTHER). In addition, diffusion tensor imaging data were collected, and fractional anisotropy (FA) measures were extracted for each lobe in both hemispheres. Results: Within the TBI group, but not within the NC group, participants who were perceived by their close others as having more communication problems had lower FA in the left frontal and temporal lobes (p<.01), but not in other brain regions. Conclusions: Frontotemporal white matter microstructural integrity is associated with social communication abilities in adults with TBI. This finding contributes to our understanding of the mechanisms leading to communication impairment following TBI and can inform the development of new neuromodulation therapies as well as diagnostic tools. (JINS, 2016, 22, 705–716)

Decision-making is an essential component of executive function, and a critical skill of political leadership. Neuroanatomic localization studies have established the prefrontal cortex as the critical brain site for executive function. In addition to the prefrontal cortex, white matter tracts as well as subcortical brain structures are crucial for optimal executive function. Executive function shows a significant decline beginning at age 60, and this is associated with age-related atrophy of prefrontal cortex, cerebral white matter disease, and cerebral microbleeds. Notably, age-related decline in executive function appears to be a relatively selective cognitive deterioration, generally sparing language and memory function. While an individual may appear to be functioning normally with regard to relatively obvious cognitive functions such as language and memory, that same individual may lack the capacity to integrate these cognitive functions to achieve normal decision-making. From a historical perspective, global decline in cognitive function of political leaders has been alternatively described as a catastrophic event, a slowly progressive deterioration, or a relatively episodic phenomenon. Selective loss of executive function in political leaders is less appreciated, but increased utilization of highly sensitive brain imaging techniques will likely bring greater appreciation to this phenomenon. Former Israeli Prime Minister Ariel Sharon was an example of a political leader with a well-described neurodegenerative condition (cerebral amyloid angiopathy) that creates a neuropathological substrate for executive dysfunction. Based on the known neuroanatomical and neuropathological changes that occur with aging, we should probably assume that a significant proportion of political leaders over the age of 65 have impairment of executive function.

Prospective memory (PM) is dependent on executive processes known to be impaired in Huntington's disease (HD); however, no study to the authors’ knowledge has investigated PM in this group. We examined performance-based, semi-naturalistic, and self-reported PM in 20 individuals diagnosed with mild–moderate HD and 20 demographically similar controls. Relative to controls, HD participants demonstrated significantly lower scores in time-based PM, event-based PM (at a trend level), and the semi-naturalistic PM trial, all of which were marked by omission errors. HD participants demonstrated comparable recognition memory for the PM intentions relative to controls. HD and control participants also showed comparable scores in self-reported PM complaints. The results suggest that HD is associated with deficits in the strategic aspects of PM. HD-associated PM deficits also are evident in real-world situations, which may relate to an apparent meta-memory deficit for PM functioning as indicated by HD participants’ overestimation of their PM performance on self-report. (JINS, 2014, 20, 1–8)

In theory, semantic memory may trigger and support the execution of everyday activities. This study explored this question by comparing three patients with semantic dementia to 40 normal controls performing different everyday activities. Participants were tested in their home using the Instrumental Activities of Daily Living Profile, an ecological measure of everyday functioning. Participants were informed that they had unknowingly invited two guests for lunch and should prepare accordingly. With these instructions, they dress to go outdoors, go to the grocery store, shop for food, prepare a hot meal, have the meal with the guests, and clean up after the meal. Performance was analyzed on the basis of four operations related to problem solving: formulate a goal, plan, execute, and verify attainment of the goal. Results indicate that compared to normal controls, two patients had significant difficulties and needed assistance with all operations of problem-solving, particularly while preparing a meal and cleaning up after the meal. One patient showed no difficulties despite severe semantic deficits. These results suggest that semantic deficits alone cannot explain the difficulties observed, but may contribute to some aspects of everyday actions such as those involved in everyday problem-solving. (JINS, 2012, 18, 1–11)

Moral judgment involves considering not only the outcome of an action but also the intention with which it was pursued. Previous functional magnetic resonance imaging (fMRI) research has shown that integrating outcome and belief information for moral judgment relies on a brain network including temporo-parietal, precuneus, and medial prefrontal regions. Here, we investigated whether the ventromedial prefrontal cortex (vmPFC) plays a crucial role in this process. Patients with lesions in vmPFC (vmPFC patients), and brain-damaged and healthy controls considered scenarios in which the protagonist caused intentional harm (negative-outcome, negative-belief), accidental harm (negative-outcome, neutral-belief), attempted harm (neutral-outcome, negative-belief), or no harm (neutral-outcome, neutral-belief), and rated the moral permissibility of the protagonists’ behavior. All groups responded similarly to scenarios involving intentional harm and no harm. vmPFC patients, however, judged attempted harm as more permissible, and accidental harm as less permissible, than the control groups. For vmPFC patients, outcome information, rather than belief information, shaped moral judgment. The results indicate that vmPFC is necessary for integrating outcome and belief information during moral reasoning. During moral judgment vmPFC may mediate intentions’ understanding, and overriding of prepotent responses to salient outcomes. (JINS, 2012, 18, 1–10)