Demonstration of a lexical access deficit in relapsing-remitting and secondary progressive forms of multiple sclerosis

The commonly used test to evaluate naming ability in multiple sclerosis (MS) is the Boston Naming Test (BNT). In previous studies the BNP has not shown any specific deficit in MS patients. The BNT score is obtained by adding spontaneously correct answers to correct answers obtained after semantic and phonological clues are given. Our hypothesis was that due to a lexical access deficit based on executive dysfunction, MS patients would need more clues than control subjects to normalize their performances,. Fifteen relapsing-remitting (RR) and 17 secondary progressive (SP) MS patients, and 32 controls matched for sex, age, and educational level, took the BNT. The 32 MS patients also took the BCCog (Short French battery used in MS to evaluate cognitive functions) in order to evaluate their executive functions. MS patients needed significantly more clues than matched controls to normalize their performances (P < 0.001). This lexical access deficit was more frequent in the SP than in the RR group (P < 0.05). A lexical access deficit inducing a denomination problem has thus been shown in MS patients. Further research should aim to better evaluate the executive functions of patients with a lexical access deficit.     

Cognitive profile in Wilson's disease: A case series of 31 patients

BackgroundWilson's disease (WD) is a rare autosomal recessive disorder of copper metabolism. If untreated, WD, which is initially a liver disease, can turn into a multi-systemic disease with neurological involvement. Very few studies have described cognitive impairment in WD. The aim of this study is to report the cognitive profile of 31 treated WD patients.MethodsPatients were classed into two groups using the Unified Wilson Disease Rating Scale (UWDRS): WD patients without neurological signs (WD-N^?) (n = 13), and WD patients with neurological signs (WD-N⁺) (n = 18). The patients participated in a neuropsychological assessment evaluating memory, executive function and visuo-spatial abilities.ResultsBoth groups performed well for verbal intelligence and episodic memory skills. However, the majority of these patients exhibited altered performance for at least one cognitive test, particularly in the executive domain. The WD-N⁺ group performed less well than the WD-N^? group on cognitive tests involving rapid motor function, abstract thinking, working memory and top-down inhibitory control.ConclusionsCognitive impairment in treated WD patients essentially affects executive function involving fronto-striatal circuits. Verbal intelligence and episodic memory abilities seem to be remarkably preserved. Neuropsychological assessment is a valuable tool to evaluate the presence and the consequences of these cognitive impairments in WD patients with or without neurological signs in the course of this chronic disease.     

Decision making and executive function in severe traumatic brain injured patients: validation of a decision-making task and correlated features

OBJECTIVE: At the chronic stage, severe traumatic brain injured (TBI) patients experience difficulty in making decisions. Several studies have demonstrated the involvement of the prefrontal cortex, in particular the orbitofrontal region, in decision-making. The aim of the present study was to validate a decision-making task in this population and to ascertain whether the components of their dysexecutive syndrome may affect their decision-making and lead to difficulties for social rehabilitation. PATIENTS AND METHODS: Fifteen TBI patients and 15 controlled subjects matched for age, sex and years of education were assessed by a battery of executive tests (GREFEX) and by the gambling task (GT). RESULTS: The TBI subjects performed significantly worse than the controlled group in five out of six GREFEX tests. The TBI choices are significantly more disadvantageous than the choices of the control group when considering the three last blocks of 20 cards of the GT. The GT total score correlated significantly with execution time of the Stroop interference condition and the Trail Making Task B, as well as with the two measures (correct sequence span and number of crossed boxes) of the double condition of Baddeley's task. CONCLUSION: We postulate that executive functioning (supervisory attentional system) influence performance in the gambling task through mechanisms of inhibitory control, divided attention and working memory. Thus, this task seems to be determined by multiple factors; the process of decision-making may depend on frontal integrity.     

Diagnostic, évaluation et prise en charge du trouble déficitaire de l’attention avec/sans hyperactivité : le point de vue du neuropsychologue

Despite the advances made regarding both the characterization and classification of the disorder (e.g., DSM-IV), the diagnosis of the attention deficit and hyperactivity disorder (ADHD) in children remains very difficult. The principal aim of this paper is to present the interest of a integrative approach in the understanding, diagnosis and identification of difficulties in ADHD children. More particularly, it aims to underline the interest of the cognitive approach in the understanding of this disorder in the day-to-day life functioning, as well as the benefits of this approach when a specific remediation is planned.     

Le retentissement neuropsychologique de la craniosténose : cas de la scaphocéphalie non syndromique

BackgroundScaphocephaly increases the rate of some modifications of cognitive and mood profile in a manner that remains to be elucidated.ObjectiveWe aimed to describe the impact of scaphocephaly on neuropsychological profile and more particularly on the executive functions.Patients and methodsAn experimental group of 19 children older than 5 years, operated on for scaphocephaly, was compared with a control group of 10 children operated on for trigonocephaly, using IQ tasks, attention tasks and mood scales. A group of 6 children from 2 to 4 years old, operated on for scaphocephaly, and a group of 6 children with non-operated scaphocephaly are also described.ResultsBoth the experimental group and the control group showed unchanged IQ, whereas attention deficit and anxiety disorder were more frequent in the experimental group. Cognitive profiles differed between groups, with a higher rate of impaired inhibitory control of visual processing in the scaphocephaly group, contrasting with a higher rate of impaired auditory verbal working memory in the trigonocephaly group. Comparable profiles were also found in groups of younger or non-operated children with scaphocephaly.ConclusionsMany children with scaphocephaly must cope with a specific neuropsychological profile throughout development. This study suggests the interest for these children and their families of specific follow-up in reference centers.