In this study, we tested the hypothesis that action planning is impaired in children with neurofibromatosis type 1 (NF1). Thirty-six children with NF1 were pair-matched to 36 healthy controls (HC) on age (range, 7–12 years), sex, and parental education level, and both groups were administered three action-planning tasks. To examine the relation of task performance to attention deficit hyperactivity disorder (ADHD), the NF1 group was divided into subsets of children who met or did not meet criteria for ADHD. Children with NF1 performed less well than HC on all planning tasks, and differences remained when controlling for IQ or a measure of visuospatial skill. Both the NF1 with ADHD subset and NF1 without ADHD subset performed more poorly than HC on two of the tasks, whereas only the NF1 with ADHD subset performed worse than HC on the third planning task. The results underscore the importance of evaluating executive function in children with NF1 and suggest that deficits in this domain may be only partially related to ADHD. Planning deficits in children with NF1 may be part of their cognitive phenotype. Identifying these deficits is relevant in determining factors contributing to learning problems and in developing appropriate interventions. (JINS, 2010, 16, 1056–1063.)

Lhermitte (1983) coined the term “utilization behavior” (UB) to define a neurobehavioral syndrome in which the visuo-tactile presentation of objects compels patients to grasp and use them, despite the fact that they have not been instructed to do so. The author suggested that UB was the consequence of frontal lobe damage. Thereafter, Shallice, Burgess, Schon, and Baxter (1989) questioned Lhermitte’s (1983) procedure for eliciting UB, putting forward an alternative research methodology that led to differentiate two forms of UB: “induced” and “incidental.” To date, there has been no direct comparison between these two procedures, nor have any other methodologies been used to explore this clinical sign, which is related to fundamental concepts such as free will and human autonomy. We investigated UB in 70 subjects (25 patients with frontal lobe lesions, 10 patients with posterior brain damage and 35 control subjects) using the methodologies of Lhermitte (1983) and Shallice et al. (1989), as well as an original “verbal generation” procedure. Our results show that the verbal generation procedure reveals UB efficiently and that elicitation of this sign appears to be directly linked to the content of the task. We discuss the interpretation of UB in terms of an executive control deficit. (JINS, 2010, 16, 453–462.)

A multicenter randomized control trial (RCT) was conducted to evaluate the effects of a treatment for dysexecutive problems after acquired brain injury (ABI) on daily life functioning. Seventy-five ABI patients were randomly allocated to either the experimental treatment, multifaceted strategy training for executive dysfunction, or a control treatment, computerized cognitive function training. Assessment took place before, directly after, and 6 months post-treatment. The primary outcome measure, the Role Resumption List (RRL), and two other follow-up measures, the Treatment Goal Attainment (TGA) and the Executive Secretarial Task (EST), were indications of daily life executive functioning. The experimental group improved significantly more over time than the controls on the RRL and attained significantly higher scores on the TGA and EST. We conclude that our treatment has resulted in significant improvements of executive functioning in daily life, lasting at least 6 months post-treatment. Although control patients’ satisfaction and subjective well-being were at the same level, the experimental group had better abilities to set and accomplish realistic goals, to plan, initiate, and regulate a series of real-life tasks, and to resume previous roles with respect to work, social relations, leisure activities, and mobility. (JINS, 2010, 16, 118–129.)

Traditional neuropsychological measures of executive dysfunction (ED) are widely believed to lack adequate sensitivity and selectivity. This may indicate that existing measures are poorly designed and constructed, although an alternative explanation is that executive cognition is multifactorial, such that its assessment necessarily requires administration of multiple measures. This possibility led to the development of a test battery, the Behavioural Assessment of the Dysexecutive Syndrome (BADS). To investigate the sensitivity of the BADS to ED, it and various other measures of ED were administered to 64 persons who had sustained traumatic brain injury. The treating clinical neuropsychologist and occupational therapist for each participant also completed a behavioural rating scale, the Dysexecutive Questionnaire (DEX). Four factors were found to underlie scores on the neuropsychological measures, but few tests were sufficiently powerful to make a significant unique contribution to predicting scores on the DEX. This confirms that multiple tests, drawn from both the BADS and other sources, may be necessary to detect ED in a clinical population. (JINS, 2005, 11, 606–613.)

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.)

Ninety-two mixed etiology neurological patients and 216 control participants were assessed on a range of neuropsychological tests, including 10 neuropsychological measures of executive function derived from 6 different tests. People who knew the patients well (relatives or carers) completed a questionnaire about the patient's dysexecutive problems in everyday life, and this paper reports the extent to which the tests predicted the patients' everyday life problems. All of the tests were significantly predictive of at least some of the behavioral and cognitive deficits reported by patients' carers. However, factor analysis of the patients' dysexecutive symptoms suggested a fractionation of the dysexecutive syndrome, with neuropsychological tests loading differentially on 3 underlying cognitive factors (Inhibition, Intentionality, and Executive Memory), supporting the conclusions that different tests measure different cognitive processes, and that there may be limits to the fractionation of the executive system. (JINS, 1998, 4, 547–558.)

Parkin's criticisms of the central executive are based on a series of misconceptions. The central executive is not an organ that might or might not exist, but a scientific concept. Part of its function is to separate the analysis of executive processes from the question of their anatomical location. Like other components of working memory, it is fractionable into subsystems. How the subsystems interrelate and how they map onto the anatomical substrate are empirical questions under active current investigation. (JINS, 1998, 4, 523–526.)

The use of a mnemonic cueing system (NeuroPage®) and a paper and pencil checklist in the rehabilitation of executive problems in a 50-year-old woman are described. Following a CVA 7 years earlier, the patient, despite intact general intellectual and memory functioning, had specific executive impairments of attention, planning, realizing intended actions, and also exhibited behavioral routines similar in form to obsessive–compulsive rituals. In a series of ABAB single-case experimental designs, the efficacy of 2 external cueing systems in prompting appropriately timed action is demonstrated. It is argued that the combination of external control and increased sustained attention to action were critical to the success of NeuroPage with this patient. Furthermore it is hypothesized that the checklist was effective in facilitating the patient's ability to foresee and recognize the consequences of her actions, which in turn had an impact on the probability of her changing those same actions. (JINS, 1998, 4, 399–408.)