Neuropsychological Functioning in Children with Medium Chain Acyl Coenzyme A Dehydrogenase Deficiency (MCADD): The Impact of Early Diagnosis and Screening on Outcome

Children with medium chain acyl coenzyme A dehydrogenase deficiency (MCADD) have been reported to be at high risk for neurocognitive deficits. However this has not been systematically studied and little is known about the exact nature of neuropsychological sequelae or of the impact of early diagnosis and screening on outcome. We examined cognitive and adaptive outcome in children with MCADD (N?=?38, age range: 2 years, 2 months – 10 years, 3 months) diagnosed either through a newborn screening program (tandem mass spectrometry/MSMS) or upon clinical presentation. There was no evidence of overall intellectual impairment in either groups but there was some suggestion of poorer verbal and specific executive functioning (i.e., planning) abilities in the unscreened cohorts. Adaptive functioning was relatively intact with the exception of reduced Daily Living Skills in both our screened and unscreened groups. Early diagnosis and greater number of hospitalizations were related to higher verbal, communication, and socialization skills. Overall, our results highlight the importance of early diagnosis and management for children with MCADD.     

Cognitive Functioning,Behavior, and Quality of Life After Stroke in Childhood

Rationale: To provide a better understanding of cognitive functioning, motor outcome, behavior and quality of life after childhood stroke and to study the relationship between variables expected to influence rehabilitation and outcome (age at stroke, time elapsed since stroke, lateralization, location and size of lesion).

Methods: Children who suffered from stroke between birth and their eighteenth year of life underwent an assessment consisting of cognitive tests (WISC-III, WAIS-R, K-ABC, TAP, Rey-Figure, German Version of the CVLT) and questionnaires (Conner's Scales, KIDSCREEN).

Results: Twenty-one patients after stroke in childhood (15 males, mean 11;11 years, SD 4;3, range 6;10–21;2) participated in the study. Mean Intelligence Quotients (IQ) were situated within the normal range (mean Full Scale IQ 96.5, range IQ 79–129). However, significantly more patients showed deficits in various cognitive domains than expected from a healthy population (Performance IQ p?=?.000; Digit Span p?=?.000, Arithmetic's p?=?.007, Divided Attention p?=?.028, Alertness p?=?.002). Verbal IQ was significantly better than Performance IQ in 13 of 17 patients, independent of the hemispheric side of lesion. Symptoms of ADHD occurred more often in the patients' sample than in a healthy population (learning difficulties/inattention p?=?.000; impulsivity/hyperactivity p?=?.006; psychosomatics p?=?.006). Certain aspects of quality of life were reduced (autonomy p?=?.003; parents' relation p?=?.003; social acceptance p?=?.037). Three patients had a right-sided hemiparesis, mean values of motor functions of the other patients were slightly impaired (sequential finger movements p?=?.000, hand alternation p?=?.001, foot tapping p?=?.043). In patients without hemiparesis, there was no relation between the lateralization of lesion and motor outcome. Lesion that occurred in the midst of childhood (5–10 years) led to better cognitive outcome than lesion in the very early (0–5 years) or late childhood (10–18 years). Other variables such as presence of seizure, elapsed time since stroke and size of lesion had a small to no impact on prognosis.

Conclusion: Moderate cognitive and motor deficits, behavioral problems, and impairment in some aspects of quality of life frequently remain after stroke in childhood. Visuospatial functions are more often reduced than verbal functions, independent of the hemispheric side of lesion. This indicates a functional superiority of verbal skills compared to visuospatial skills in the process of recovery after brain injury. Compared to the cognitive outcome following stroke in adults, cognitive sequelae after childhood stroke do indicate neither the lateralization nor the location of the lesion focus. Age at stroke seems to be the only determining factor influencing cognitive outcome.     

Executive function outcomes of children with traumatic brain injury sustained before three years

Traumatic brain injury (TBI) is a common cause of disability in childhood. While the outcomes of TBI sustained in school years has been heavily researched, very little is known about the impact of TBI in infants and young children. The aim of this study was to investigate the impact of TBI on executive function (EF) in children who sustained a TBI before 3 years of age. A group of 55 children, 19 with a mild TBI, 16 children with a moderate-severe TBI, and 20 uninjured comparison children participated. The EF of children aged 3 to 6 years were compared using child-based measures of attentional control and information processing. Parents completed questionnaires rating their child's EF. Severity groups differed on the child-based EF measure of attentional control with children with TBI performing below the control group. There were no significant group differences for information processing or parent-rated EF. It appears that children who sustain a TBI before the age of 3 years display impairments in some areas of attentional control 3–4 years postinjury. The findings fit with the existing EF literature for older children.     

Simultaneous attentional guidance by working-memory and selection history reveals two distinct sources of attention

Recent theories of attention have proposed that selection history is a separate, dissociable source of information that influences attention. The current study sought to investigate the simultaneous involvement of selection history and working-memory on attention during visual search. Experiments 1 and 2 used target feature probability to manipulate selection history and found significant effects of both working-memory and selection history, although working-memory dominated selection history when they cued different locations. Experiment 3 eliminated the contribution of voluntary refreshing of working-memory and replicated the main effects, although selection history became dominant. Using the same methodology, but with reduced probability cue validity, both effects were present in Experiment 4 and did not significantly differ in their contribution to attention. Effects of selection history and working-memory never interacted. These results suggest that selection history and working-memory are separate influences on attention and have little impact on each other. Theoretical implications for models of attention are discussed.     

Reaction time measures in deception research: Comparing the effects of irrelevant and relevant stimulus–response compatibility

Evidence regarding the validity of reaction time (RT) measures in deception research is mixed. One possible reason for this inconsistency is that structurally different RT paradigms have been used. The aim of this study was to experimentally investigate whether structural differences between RT tasks are related to how effective those tasks are for capturing deception. We achieved this aim by comparing the effectiveness of relevant and irrelevant stimulus–response compatibility (SRC) tasks. We also investigated whether an intended but not yet completed mock crime could be assessed with both tasks. Results showed (1) a larger compatibility effect in the relevant SRC task compared to the irrelevant SRC task, (2) for both the completed and the intended crime. These results were replicated in a second experiment in which a semantic feature (instead of color) was used as critical response feature in the irrelevant SRC task. The findings support the idea that a structural analysis of deception tasks helps to identify RT measures that produce robust group effects, and that strong compatibility effects for both enacted crimes as well as merely intended crimes can be found with RT measures that are based on the manipulation of relevant SRC.     

The role of perfectionism in cognitive behaviour therapy outcomes for clinically anxious children

The main aim of this study was to determine whether pre-treatment levels of child perfectionism impacted on anxiety treatment outcomes for school-aged children. In addition, it was investigated whether child perfectionism decreased following treatment for anxiety. Participants were sixty-seven clinically anxious children aged 6–13 years (female = 34; majority Caucasian) who were enrolled in a group-based cognitive behaviour therapy program, and their parents. They completed self-report questionnaires on anxiety and depressive symptoms and were administered a diagnostic interview to determine the type and clinician rated severity of anxiety and related disorders pre- and post-treatment and at 6-month follow-up. Self- and parent-rated perfectionism were also measured pre-treatment, while a subset of children completed perfectionism measures post-treatment as well. Self-Oriented Perfectionism, but not Socially Prescribed Perfectionism, predicted poorer self-reported treatment outcome (higher levels of anxiety symptoms) immediately following treatment and at 6-month follow-up when using a multi-informant approach. Additionally, both Self-Oriented and Socially Prescribed child perfectionism significantly reduced immediately following treatment. Despite reductions in child perfectionism following anxiety treatment, higher Self-Oriented Perfectionism may impact negatively on child anxiety treatment outcome.     

Implementation of Cognitive Therapy for PTSD in routine clinical care: Effectiveness and moderators of outcome in a consecutive sample

ObjectiveTrauma-focused psychological treatments are recommended as first-line treatments for Posttraumatic Stress Disorder (PTSD), but clinicians may be concerned that the good outcomes observed in randomized controlled trials (RCTs) may not generalize to the wide range of traumas and presentations seen in clinical practice. This study investigated whether Cognitive Therapy for PTSD (CT-PTSD) can be effectively implemented into a UK National Health Service Outpatient Clinic serving a defined ethnically mixed urban catchment area.MethodA consecutive sample of 330 patients with PTSD (age 17–83) following a wide range of traumas were treated by 34 therapists, who received training and supervision in CT-PTSD. Pre and post treatment data (PTSD symptoms, anxiety, depression) were collected for all patients, including dropouts. Hierarchical linear modeling investigated candidate moderators of outcome and therapist effects.ResultsCT-PTSD was well tolerated and led to very large improvement in PTSD symptoms, depression and anxiety. The majority of patients showed reliable improvement/clinically significant change: intent-to-treat: 78.8%/57.3%; completer: 84.5%/65.1%. Dropouts and unreliable attenders had worse outcome. Statistically reliable symptom exacerbation with treatment was observed in only 1.2% of patients. Treatment gains were maintained during follow-up (M = 280 days, n = 220). Few of the selection criteria used in some RCTs, demographic, diagnostic and trauma characteristics moderated treatment outcome, and only social problems and needing treatment for multiple traumas showed unique moderation effects. There were no random effects of therapist on symptom improvement, but therapists who were inexperienced in CT-PTSD had more dropouts than those with greater experience.ConclusionsThe results support the effectiveness of CT-PTSD and suggest that trauma-focused cognitive behavior therapy can be successfully implemented in routine clinical services treating patients with a wide range of traumas.     

Attentional bias and emotional reactivity as predictors and moderators of behavioral treatment for social phobia

Cognitive behavioral therapy (CBT) is a well-established treatment for anxiety disorders, and evidence is accruing for the effectiveness of acceptance and commitment therapy (ACT). Little is known about factors that relate to treatment outcome overall (predictors), or who will thrive in each treatment (moderators). The goal of the current project was to test attentional bias and negative emotional reactivity as moderators and predictors of treatment outcome in a randomized controlled trial comparing CBT and ACT for social phobia. Forty-six patients received 12 sessions of CBT or ACT and were assessed for self-reported and clinician-rated symptoms at baseline, post treatment, 6, and 12 months. Attentional bias significantly moderated the relationship between treatment group and outcome with patients slow to disengage from threatening stimuli showing greater clinician-rated symptom reduction in CBT than in ACT. Negative emotional reactivity, but not positive emotional reactivity, was a significant overall predictor with patients high in negative emotional reactivity showing the greatest self-reported symptom reduction.     

A randomized pilot study of anger treatment for Iraq and Afghanistan veterans

Objective

Anger and aggression are serious problems for a significant proportion of veterans who have served in combat. While prior research has suggested that cognitive behavioral treatments may be effective for anger problems, there are few controlled studies of anger treatment in veterans and no studies of anger treatment focusing exclusively on veterans from the Iraq and Afghanistan wars. This randomized pilot study compared an adapted cognitive behavioral intervention (CBI) to a supportive intervention (SI) control condition for the treatment of anger problems in veterans returning from deployment in Iraq or Afghanistan.

Methods

25 veterans with warzone trauma, problems with anger, and one or more additional hyperarousal symptoms were randomized and 23 started treatment (CBI, n = 12; SI, n = 11). Outcome measures were administered at pre- and post- treatment and at 3 months post-treatment.

Results

CBI was associated with significantly more improvement than SI on measures of anger and interpersonal functioning. Gains were maintained at follow-up.

Conclusions

Findings suggest that CBI may be more effective than an active control providing psychoeducation, relaxation, and supportive therapy for treating anger problems in returning veterans. The findings need to be replicated in an adequately powered and more diverse sample.     

Changes in illness-related cognitions rather than distress mediate improvements in irritable bowel syndrome (IBS) symptoms and disability following a brief cognitive behavioural therapy intervention

Objective

A previous randomised controlled trial demonstrated that a cognitive behavioural therapy (CBT) self-management intervention significantly improved irritable bowel syndrome (IBS) symptoms and disability compared to treatment as usual (TAU). The current study analysed additional data to establish whether; 1) cognitive, behavioural and emotional factors hypothesized to perpetuate IBS symptoms and disability changed following CBT and, 2) ascertain if changes in these factors over the intervention period mediated treatment effects 6-months later.

Method

IBS patients (CBT = 31, TAU = 33) completed measures pre-and-post intervention including: Brief Illness Perception Questionnaire, Hospital Anxiety & Depression Scale and Cognitive and Behavioural Responses to Symptoms Questionnaire. Path models were evaluated to determine whether changes in cognitive and behavioural factors over the treatment period mediated treatment effects.

Results

Compared to TAU, CBT patients showed significant positive changes on several cognitive variables but not anxiety and depression following intervention. Positive change in illness perceptions following intervention mediated the treatment effect on improved IBS symptom severity and social adjustment six months later. Changes in damaging beliefs mediated the effect on social adjustment.

Conclusions

Change in cognition rather than mood mediated treatment related improvements. Changing negative perceptions of IBS appears to be a particularly important treatment mechanism.