The tower of Babel: Survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America

Purpose: The terms “electrical status epilepticus during sleep (ESES)” and “continuous spikes and waves during sleep (CSWS)” have been used interchangeably when referring to related but different concepts. In addition, the quantification of epileptiform activity has not been standardized, and different approaches to quantification have been used. The aim of this study was to evaluate the extent to which pediatric neurologists and epileptologists use a homogeneous terminology and conceptualization in CSWS and ESES and to characterize the current understanding of these conditions. Methods: A survey addressing the use of terminology in “ESES” and “CSWS” and the understanding of related concepts was distributed online to all members of the Child Neurology Society and the American Epilepsy Society mailing lists. Surveys were self‐administered and collected using an online survey website ( http://www.surveymonkey.com ). Key Findings: Two hundred nineteen surveys were completed, 137 from the Child Neurology Society mailing list and 82 from the American Epilepsy Society mailing list. ESES and CSWS were considered synonymous by 117 respondents, not synonymous by 61, 21 respondents did not know, and 20 did not respond. Most respondents (63.1%) considered CSWS as a devastating epileptic encephalopathy with severe sequelae even if treated correctly, but 25.1% of respondents indicated that it does not leave sequelae if epilepsy was treated early and another 11.8% noted that cognitive difficulties resolved with age. Cognitive and/or language regression were considered mandatory for the diagnosis of CSWS by only 27% of the respondents. The diagnosis of CSWS was based on electroencephalography (EEG) assessment alone by 31% of respondents. Respondents used different methods for calculation of the epileptiform activity, different EEG samples for calculation, and considered differently the lateralized epileptiform activity. The cut‐off values for percentage of the sleep record occupied by spike‐waves were variable depending on the respondent. There was no agreement on whether these cutoff values were mandatory for the diagnosis of ESES and CSWS. Significance: Our data show that the professionals caring for children with ESES and CSWS in North America use the terms, concepts, and defining features heterogeneously. The lack of a common language may complicate communication among clinicians and jeopardize research in this field. We anticipate that our data will fuel the development of much needed common terminology and conceptualization of ESES and CSWS.     

3R疗法对阿尔茨海默病患者认知功能和血浆β淀粉样蛋白_(1-42)的影响

目的评价3R疗法对轻、中度阿尔茨海默病患者认知功能和血浆β淀粉样蛋白_(1-42)(Aβ_(1-42))的影响。方法选择轻、中度阿尔茨海默病患者34例,随机分为2组,每组17例。对照组给予药物常规治疗,治疗组在药物常规治疗基础上,采用3R疗法进行认知功能训练。测定血浆Aβ_(1-42),并进行神经心理学指标检查:简易智能状态检查量表、日常生活活动能力量表(ADL)、社会功能活动调查(FAQ)、神经精神量表(NPI)以及事件相关电位P300的基线评定,在开始实施训练后的第3、6个月时,分别对患者进行各项指标的评定与分析。结果治疗组经过3、6个月的3R治疗后,ADL、FAQ、NPI评分较治疗前明显减低,且明显低于对照组(P<0.05,P<0.01);P300潜伏期明显缩短,波幅明显增加(P<0.01);治疗组经过6个月的3R治疗后,血浆Aβ_(1-42)含量较治疗前明显下降,且较对照组明显下降(p<0.05)。结论 3R疗法能有效提高阿尔茨海默病患者生活自理能力、社会活动能力,从而延缓认知功能减退;3R治疗后,认知功能改善可能与血浆Aβ_(1-42)含量变化有关。     

Acquisition of an instrumental activity of daily living in patients with Korsakoff's syndrome: A comparison of trial and error and errorless learning

Patients with Korsakoff's syndrome show devastating amnesia and executive deficits. Consequently, the ability to perform instrumental activities such as making coffee is frequently diminished. It is currently unknown whether patients with Korsakoff's syndrome are able to (re)learn instrumental activities. A good candidate for an effective teaching technique in Korsakoff's syndrome is errorless learning as it is based on intact implicit memory functioning. Therefore, the aim of the current study was two-fold: to investigate whether patients with Korsakoff's syndrome are able to (re)learn instrumental activities, and to compare the effectiveness of errorless learning with trial and error learning in the acquisition and maintenance of an instrumental activity, namely using a washing machine to do the laundry. Whereas initial learning performance in the errorless learning condition was superior, both intervention techniques resulted in similar improvement over eight learning sessions. Moreover, performance in a different spatial layout showed a comparable improvement. Notably, in follow-up sessions starting after four weeks without practice, performance was still elevated in the errorless learning condition, but not in the trial and error condition. The current study demonstrates that (re)learning and maintenance of an instrumental activity is possible in patients with Korsakoff's syndrome.     

Are there cognitive and neurobehavioural correlates of hormonal neuroprotection for women after TBI?

This study examined possible cognitive correlates of hormonal neuroprotection following traumatic brain injury (TBI) using archival neuropsychological findings for 1563 individuals undergoing acute TBI rehabilitation between 1989 and 2002. Presumed age of menopause was based on the STRAW (Stages of Reproductive Aging) staging system (Soules, 2005; Soules et al., 2001) and general linear model (GLM) analysis of performance on neuropsychological testing by participants across gender and age groups (25–34, 35–44, 45–54, and 55–64) was performed. Hypotheses were (1) women with TBI in the oldest age group would have lower scores on neuropsychological tests and functional outcome measures than women in the younger groups, and (2) men in the oldest age group would have higher scores than women of the same age group. Analyses revealed that oldest females had significantly worse Trails B and SDMT written and oral scores than the youngest females. In addition, oldest females had significantly better Trails B, Rey AVLT and SDMT written scores than the oldest males. Possible cohort exposure to hormone replacement therapy, unknown hormonal status at time of testing, and sample-specific injury characteristics may have contributed to these findings.     

Assessing executive performance during cognitive rehabilitation

Executive functioning influences a host of other cognitive processes and people who attend neuropsychological services are more likely to display executive dysfunction than any other cognitive deficit (Stuss & Levine, 2002 Stuss, D. and Levine, B. 2002. Adult clinical neuropsychology: Lessons from studies of the frontal lobes. Annual Review of Psychology, 53: 401–433. [Crossref], [PubMed], [Web of Science ®] , [Google Scholar]). Impairment in executive functioning disrupts a person's ability to effectively employ their intact areas of functioning, and undermines effective self-management of other areas of dysfunction, hampering attempts to employ compensatory strategies. Therefore, assessment of a person's executive functioning is a high priority as part of a comprehensive neurorehabilitation plan. Guided by the International Classification of Functioning, Disability, and Health model (ICF model; Peterson, 2005 Peterson, D. 2005. International Classification of Functioning, Disability and Health: An introduction for rehabilitation psychologists. Rehabilitation Psychology, 50: 105–112. [Crossref], [Web of Science ®] , [Google Scholar]), we suggest that an important development in the field is moving to formal assessment of executive performance in functional contexts, in addition to more traditional assessment of executive impairment. We outline a number of existing studies in this area, review current measures that can provide clinicians with useful information on these issues, and discuss how this research could be further advanced.     

Mild traumatic brain injury and its sequelae: Characterisation of divided attention deficits

Deficits in divided attention occur after a mild traumatic brain injury (MTBI) but many extant tasks lack sensitivity for detecting subtle cognitive difficulties. We use the Test d'Attention Partagée Informatisé (TAPI), a novel dual-task paradigm, to investigate the impact of MTBI on the ability to divide attention between two stimuli sources. Individuals with MTBI (n = 37) were evaluated within the first week following head trauma and at three months post-injury. A healthy control (HC) group (n = 79) was also assessed. The primary outcome was reaction time and there were three different conditions that included visual target detection and auditory digit span tasks. Analyses utilised repeated measures ANOVA and ANCOVA models that adjusted for relevant variables including post-concussive and affective symptoms. Results indicated that at both baseline and follow-up, the MTBI group had significantly slower reaction time than the HC group. Also, both the MTBI and HC groups had slower reaction times as participants progressed through each of the more challenging TAPI conditions. This study supports the usefulness of this novel instrument and allows clinicians and researchers to assess for subtle divided attention deficits that may persist in those with MTBI even three months post-injury.     

Neuropsychological characteristics of Italian children with fetal alcohol spectrum disorders

Background: Children with fetal alcohol spectrum disorders (FASD) display many problems ranging from deficits in intelligence to behavioral difficulties. Thus, many studies have aimed at defining the neuropsychological characteristics of children with FASD. The current article describes the neuropsychological characteristics of Italian children with severe diagnosis within FASD and compares them with controls. It was expected that intellectual functioning, language comprehension, academic skills, and inattention/hyperactivity would discriminate children with FASD from randomly selected peers without FASD. Methods: This article presents data from a second cohort of children examined in 2005 as part of an in‐school epidemiological study of FASD in Italy. Of 80 children, 23 diagnosed with a FASD, and 57 randomly selected control children from the same first‐grade classes, participated. After screening for FASD via growth and dysmorphology, the children were administered a test of general intelligence (WISC‐R) as well as tests of nonverbal reasoning (Raven Colored Progressive Matrices), language comprehension (Rustioni), academic achievement (IPDA), and problem behavior (Disruptive Behavior Disorder Rating Scale). Results: Children diagnosed with a FASD achieved lower scores than control children on Verbal, Performance, and Full Scale IQ. Profile analysis of the WISC‐R indicates overall differences between the groups. However, some intact functioning within the FASD group was found, as the Similarities and Vocabulary subtests were similar to the controls. After an alpha adjustment to 0.004, the Block Design, Object Assembly, and Mazes subtests were significantly different from controls. On tests of nonverbal reasoning, language comprehension, and academic achievement, the children with a FASD scored significantly lower. Moreover, teachers rated children with a severe diagnosis within FASD as showing more inattentive symptoms than controls, while hyperactive/impulsive characteristics among children with a FASD were comparable with the control children. Significant correlations between head circumference, child dysmorphology, WISC‐R, and Raven CPM scores are also reported. Conclusions: This study indicates that a sample of Italian children with a FASD, when compared with control children, display poorer functioning on measures of general intelligence, nonverbal reasoning, academic achievement, and teacher‐rated problem behaviors. The findings also contribute to the formulation of a neuropsychological profile of children diagnosed with a FASD.     

Hepatic safety of once-monthly injectable extended-release naltrexone administered to actively drinking alcoholics

Background:  Hepatoxicity has been reported with oral naltrexone. Hepatic safety data were examined from a 6-month study evaluating the efficacy and safety of a now available extended-release formulation of naltrexone (XR-NTX) in patients with alcohol dependence.
Methods:  In all, 624 patients (68% male; median age of 44 years) were randomly assigned to XR-NTX 380 mg ( n  = 205), XR-NTX 190 mg ( n  = 210), or placebo ( n  = 209).
Results:  There were no significant differences in alanine aminotrasferase, aspartate aminotransferase, or bilirubin levels between the study groups at study initiation or at subsequent assessments. Gamma-glutamyltransferase in the XR-NTX 380 mg group was lower compared with placebo at weeks 4, 8, 12, and 20. Both high (>3 times the upper limit of normal) liver chemistry tests (LCTs) and hepatic-related adverse events were infrequent in all study groups. In patients who were drinking heavily throughout the study, obese subjects, or those taking nonsteroidal anti-inflammatory drugs, there was no increase in frequency of high LCTs or hepatic-related adverse events in patients receiving XR-NTX (either dose) compared with placebo.
Conclusion:  Extended-release formulation of naltrexone does not appear to be hepatotoxic when taken at the recommended clinical doses in actively drinking alcohol-dependent patients.