Serial neuropsychological testing before and after hemispherectomy in a child with electrical status epilepticus in slow wave sleep.

Electrical status epilepticus in slow wave sleep (ESES) is typically characterized by neuropsychological deterioration to varying extents. While the syndrome is self-limiting for some, for others it has a swiftly declining course that requires aggressive neurosurgical intervention. Here, we present a patient with ESES secondary to a large left middle cerebral artery stroke in utero who experienced behavioral and neuropsychological deterioration that rapidly progressed. Collectively, her neuropsychological status, EEGs, MRIs, and nature of her ESES warranted a left hemispherectomy to prevent further decline. The patient was seen for four neuropsychological evaluations. Over time, neuropsychological test scores did not adequately capture her degree of impairment and change. Behavioral observations during evaluations and parental reports played a critical role in determining her initial decline and mild post-surgical improvement. For all practitioners, this report illustrates one extreme of ESES. It also illustrates, particularly for neurologists and neurosurgeons, the importance of behavioral observations relative to test performances.

Competency-based assessment in clinical neuropsychology at the post-doctoral level: Stages, milestones, and benchmarks as proposed by an APPCN work group.

Objective: In recent years, there has been considerable progress in developing competencies in Clinical Neuropsychology. The field also needs to work towards consistency in competency-based assessment of the development of each competency to ensure competent, independent practice. The purpose of this manuscript is to a) document the relevant literature, b) describe the process applied by an Association of Post-Doctoral Programs in Clinical Neuropsychology (APPCN) workgroup on Competency-Based Assessment, and c) propose a framework and assessment tool for competency-based assessment at the post-doctoral training level. Methods: The work group conducted a literature review of competency-based assessment in Clinical Neuropsychology and related fields, considered various constructs for assessment, delineated a framework that can be flexible for program-specific goals, and created a tool for assessment. The workgroup then asked for review of the framework and assessment tool by APPCN Board of Directors, the APPCN Executive Committee, and Program Directors from APPCN and non-APPCN programs. Revisions were made following this review. Conclusions: This manuscript and proposed assessment tool invite constructive feedback within the community for ongoing evolution of the process and the tool. The proposed assessment tool is intended to be implemented flexibly within post-doctoral programs to respect their specific training goals while simultaneously providing underlying consistency in the method of assessing a recently proposed set of competencies within Clinical Neuropsychology. Creation of competency-based assessment tools across all training levels within Clinical Neuropsychology that facilitate continuity and hierarchical development is a long-term goal.

Influence of age and education on a performance-based measure of everyday functioning.

Sociodemographic variables, such as age and education, influence the determination of impairment on neuropsychological tests, but their influence on impairment determinations for tests of everyday functioning is less well defined. Existing studies suggest that older age and lower education levels are associated with worse everyday functioning when assessed by self- or collateral-report. This relationship, however, has not been thoroughly investigated with performance-based methods of everyday functioning. This study aimed to determine the influence of sociodemographic factors on the Functional Impact Assessment (FIA), a performance-based measure of everyday functioning that includes measures of both accuracy and speed. Seventy-three healthy individuals, ages 42 - 88 years, completed the FIA and an additional everyday functioning self-report questionnaire (Functional Activities Questionnaire). Using a multiple regression statistical approach, age and education predicted overall FIA accuracy, while age alone predicted FIA speed. Sociodemographic variables continued to predict FIA performance when controlling for overall cognitive functioning. Sociodemographic variables were unrelated to FAQ scores. These findings indicate that age and education are associated with scores on a performance-based test of everyday functioning. Demographic corrections may improve accuracy in determining functional impairment, but more research is needed given the complex relationships among demographic factors, healthy aging, and dementia risk.

Using latent profile analysis to evaluate the utility of a DSM-5 symptom checklist in screening children for autism spectrum disorder.

Objective: Currently available screening measures for Autism Spectrum Disorder (ASD) typically do not reflect DSM-5 diagnostic criteria and generally have weak positive predictive values. These factors result in missed opportunities for early intervention, delays in diagnosis, and contribute to inefficient usage of healthcare resources by inadequately discerning those in need of comprehensive assessment. This study examined a DSM-5 ASD symptom checklist to determine whether parent-report response patterns could accurately identify which children received an ASD diagnosis. Method: Data were examined from 376 ASD evaluations in a three-year period. Latent profile analysis was used to determine if subgroups could be identified according to parent response patterns, and network analysis was implemented to examine the relationship among DSM-5 ASD criteria within each latent profile. Results: A four-profile model was best supported based on fit indices and high probability classifications. The model was largely a product of how parents responded regarding their child's sensory behavior and minimally reflected other symptomatology. Subsequent network analyses by profile indicated weak coherence among DSM-5 symptoms within all profiles. Overall, direct assessment of DSM-5 criteria based on parent report did not add diagnostic value beyond that reflected in base rates. Conclusions: Although continued refinement of ASD screening tools is needed to improve accuracy of referrals for evaluations and reduce wait time for diagnosis, this study continues to support the need for behavioral observation and formal assessment by trained clinicians. Continued development of sensitive and specific screening tools, likely with embedded behavioral and/or objective observation, is needed.

A cross-sectional cohort study of post-concussive symptoms and their relationships with depressive symptoms in youth with and without concussion.

OBJECTIVES: To evaluate the frequency and severity of post-concussive symptoms in youth with a history of concussion relative to youth without concussion who had another medical diagnosis, as well as compare the correlations between post-concussive and depressive symptoms between groups. We hypothesized comparable symptom reporting and correlations in each group. METHODS: A total of 564 youth ages 8-18 years were assessed regarding post-concussive symptoms. A subset of youth (n = 360) were compared on correlations between post-concussive and depressive symptoms. Non-parametric statistics were used for most analyses. RESULTS: Youth with concussion reported a comparable number of post-concussive and depressive symptoms as youth with another medical condition without concussion. However, those with concussion reported greater post-concussive symptom severity (but small effect sizes). Relationships between post-concussive and depressive symptoms were comparable for both groups, but for those who sustained a concussion, the correlation was significantly stronger for females than males. CONCLUSIONS: This study further demonstrates that post-concussive symptoms are nonspecific and provide little functional utility. Post-concussive and depressive symptoms are strongly correlated, particularly in females with concussion. Psychiatric comorbidities and other medical diagnoses should be assessed pre-injury because both affect interpretation of post-concussive symptom reports.

Convergent and discriminant validity of the ImPACT with traditional neuropsychological measures.

Neuropsychological assessment of cognitive sequelae secondary to sports concussion is limited by lengthy administration times and lack of readily available neuropsychologists. Brief computerized test batteries are now under development to address this, but the validity of these measures is not yet established. The validity of one such computerized test battery, the Immediate Post-Concussion Assessment and Cognitive Testing (ImPACT), was administered to 93 healthy NCAA Division I athletes, aged 18-24, along with a battery of traditional, well-described neuropsychological tests. Convergent and discriminant validity between the ImPACT and traditional measures was investigated using multitrait-multimethod matrix (MTMM) analysis. As an example, the ImPACT Visual Motor Speed composite demonstrated reasonably good convergent validity secondary to moderate correlations with traditional measures of processing speed, but it demonstrated relatively poor discriminant validity as it significantly correlated with the Reaction Time composite score. MTMM results were variable across ImPACT composites and data for each are presented. The ImPACT composite's validity was further investigated using exploratory factor analysis (EFA). Six principal components were termed processing speed, visual memory, verbal memory, attention & working memory, and verbal fluency, based upon traditional test loadings, and a sixth loaded only on the ImPACT Reaction Time composite. EFA indicated content validity of moderate strength for the Visual Motor Speed and Visual Memory composites, but revealed problems with specificity for the other composites. Based upon the present findings, validity problems render the interpretability of the ImPACT composites somewhat questionable, and more research is necessary prior to using the ImPACT for assessment of clinical populations.

Clinical utility of the Neuropsychological Assessment Battery (NAB) after unilateral stroke.

The NAB is a comprehensive battery assessing five cognitive domains (Attention, Language, Memory, Spatial, Executive Function). Despite the advantage of co-normative domain data, its clinical utility is not well established because few studies have reported full-battery findings. The aim of this study was to determine if the NAB was sensitive to well documented hemispheric differences in language and spatial skills after unilateral stroke. We compared demographically matched control participants (n = 52) and individuals after left (LHD, n = 36) or right (RHD, n = 33) hemisphere damage due to stroke on the NAB, parts of the Western Aphasia Battery, and traditional visuospatial tasks. Both stroke groups showed impaired NAB Attention, Spatial, and Executive Functions relative to controls, while the LHD group was more impaired than control and RHD groups on Language and Memory modules. LHD patients with aphasia on traditional measures performed worse than control and non-aphasic LHD patients on all NAB domains. RHD patients with spatial impairment on traditional measures performed worse than controls, but not RHD patients without spatial impairment, on the NAB Spatial domain. Findings suggest the NAB is generally comparable to traditional language and visuospatial measures, and it sufficiently detects attention and executive deficits.

A critical review of neuroimaging applications in sports concussion.

While abnormalities related to concussion are typically not identified on traditional clinical neuroimaging (i.e., computed tomography [CT] or magnetic resonance imaging [MRI]), more sophisticated neuroimaging techniques have the potential to reveal the complex neurometabolic processes related to concussion and its recovery. Clinically, these techniques may one day provide useful information to guide clinicians in the management and treatment of sports concussion. This article critically reviews the current state of the literature regarding neuroimaging and sports concussion, identifies challenges in the application of these techniques, and identifies areas for future research.

Thalamofrontal circuitry and executive dysfunction in recent-onset juvenile myoclonic epilepsy.

PURPOSE: Thalamofrontal abnormalities have been identified in chronic primary generalized epilepsy, specifically in juvenile myoclonic epilepsy (JME). These regions also underlie executive functioning, although their relationship has yet to be examined in JME. This study examined the relationship between thalamic and frontal volumes and executive function in recent-onset JME compared to healthy control subjects and recent-onset benign childhood epilepsy with centrotemporal spikes (BCECTS), a syndrome not typically associated with thalamocortical or executive dysfunction. METHODS: Twenty children with recent-onset JME were compared to 51 healthy controls and 12 children with BCECTS using quantitative magnetic resonance imaging (MRI) and measures of executive abilities. Quantitative thalamic and frontal volumes were obtained through semi-automated software. Subtests from the Delis-Kaplan Executive Function System (D-KEFS) and the Behavior Rating Inventory of Executive Function (BRIEF) were used to measure executive function. RESULTS: Executive functions were impaired in JME subjects compared to control and BCECTS subjects. Subjects with JME had significantly smaller thalamic volumes and more frontal cerebrospinal fluid (CSF) than control and BCECTS subjects. Thalamic and frontal volumes were significantly related to executive functioning in the JME group, but not in the other two groups. DISCUSSION: Children with JME have significant executive dysfunction associated with significantly smaller thalami and more frontal CSF. Children with recent-onset BCECTS do not display the same pattern. Frontal and thalamic volumes appear to mediate the relationship between executive functioning and brain structure in JME.

Association of epilepsy and comorbid conditions.

Comorbid health conditions are common among people with epilepsy. Proposed explanations for this association include the possibility that first, epilepsy (including its treatment) causes the comorbid condition; second, the comorbid condition (including its treatment) causes epilepsy; or third, a common pathogenic mechanism mediates the co-occurrence of epilepsy and the comorbid condition. It is unlikely that a single explanation will suffice for all of the epilepsy comorbid conditions. Determining the basis of the association between epilepsy and its comorbid conditions has important implications for diagnosis and management. In this paper, we discuss this issue in the context of five common epilepsy comorbid conditions: bone health and fractures, stroke, depression, migraine and attention-deficit hyperactivity disorder. Current findings, research limitations and future directions of research efforts are discussed.

Corpus Callosum Maturation and Line Bisection Performance in Healthy Children.

Line bisection performance in children has been hypothesized to be a measure of corpus callosum maturation. Several previous studies have shown that normal prepubescent children bisect lines to the right of true center with their right hand and to the left with their left hand (symmetrical neglect). In contrast, children entering puberty reportedly bisect lines to the left with both the right and left hands (pseudoneglect). The shift from symmetrical to pseudoneglect has been hypothesized to reflect corpus callosum maturation and its involvement in the transfer of attention-based visuospatial processes. In the current study, line bisection performance and MR quantitative corpus callosum volumes were examined in 46 healthy children ages 8-18 years. A linear relationship between corpus callosum volume and age was found. However, the expected age-contingent line bisection performance pattern was not observed. In addition to the expected two patterns of line bisection bias, pseudoneglect and symmetric neglect, two additional distinct patterns of line bisection were identified. These findings, and other findings in the literature, raise important questions about the reliability and validity of the line bisection test. No relationship was found between corpus callosum volume and amount or direction of line bisection deviation. Our findings do not support previous hypotheses regarding line bisection-corpus callosum relationship.

MRI volume loss of subcortical structures in unilateral temporal lobe epilepsy.

Few studies have examined the relative degree of brain volume loss in both the hippocampi and subcortical structures in unilateral temporal lobe epilepsy (TLE) and their association with clinical seizure correlates. In this study, quantitative MRI volumes were measured in the hippocampus, thalamus, caudate, putamen, and corpus callosum in 48 patients with unilateral TLE (26 right, and 22 left) and compared with the volumes of 29 healthy controls. The ipsilateral hippocampus, corpus callosum, and bilateral thalami exhibited the greatest volume loss, reflected by large to moderate effect size differences compared with controls. Bilaterally, the putamen showed the next highest volume reduction. The contralateral hippocampus and bilateral caudate nuclei showed the least volume reduction, characterized by small effect sizes. Furthermore, clinical seizure characteristics (e.g., duration of epilepsy) exhibited different patterns of association with the volume reductions observed across these structures. Findings suggest that distinct neurodevelopmental features may play a role in the volume abnormality observed in these regions.

Cognitive progression in epilepsy.

The issue of cognitive progression in people with epilepsy is of considerable interest and has important clinical and theoretical implications. In this paper, we review recent studies in both the adult and childhood epilepsy literature which have included a longitudinal test-retest design to examine this question. Several important methodological issues of this literature are highlighted and areas which require more investigation are identified.

Quality of life and comorbid medical and psychiatric conditions in temporal lobe epilepsy.

Patients with epilepsy frequently report dissatisfaction with their quality of life (QOL), and there is considerable interest in understanding the factors and mechanisms associated with this perception. To date, investigation has focused on the potential contribution of seizure variables (e.g., seizure control) and psychiatric conditions to QOL, but there has not been an examination of the potential role of comorbid medical conditions. Information was gathered concerning current medical and psychiatric conditions for 93 patients with temporal lobe epilepsy, and their relationship with QOL was examined. The numbers of medical and psychiatric conditions were distinct and significant predictors of QOL satisfaction, and both were stronger correlates than seizure-related factors and demographic variables. The implications of these findings for the treatment and management of patients with epilepsy are discussed.