Tracey A. Williams

Cognitive remediation of attention deficits following acquired brain injury: A systematic review and meta-analysis

BACKGROUND Attention deficits are common after acquired brain injury (ABI) and adversely impact academic, vocational and social outcomes. The role of cognitive interventions in post-ABI attention rehabilitation remains unclear. OBJECTIVE To evaluate effectiveness of cognitive interventions in treating attention deficits following ABI and to explore differences in treatment effect between ABI etiologies. METHODS MEDLINE, EMBASE, PsycINFO and CENTRAL databases were searched for randomized controlled trials (RCTs). Studies were selected by three reviewers. Study quality was assessed using Cochrane Collaboration tool for RCTs. Effect sizes (Hedges g) for each attentional domain were meta-analyzed with subgroup analysis by ABI etiology. RESULTS Twelve RCTs with 584 participants were included, representing individuals with stroke, traumatic brain injury (TBI) and CNS-impacting malignancy. Cognitive rehabilitation improved divided attention in stroke survivors (g 0.67; 95% confidence interval, 0.35-0.98; p <  0.0001) but not other ABI populations. Sustained, selective and alternating attention, and inhibition were not significantly improved in any ABI population. Follow-up data showed no evidence of long-term benefit. CONCLUSION Cognitive rehabilitation resulted in short-term improvements in divided attention following stroke, but not after TBI or CNS-impacting malignancy. Cognitive interventions did not significantly improve other attentional domains in participants with stroke, TBI or CNS-impacting malignancy.

Viewing Social Scenes: A Visual Scan-Path Study Comparing Fragile X Syndrome and Williams Syndrome

Fragile X syndrome (FXS) and Williams syndrome (WS) are both genetic disorders which present with similar cognitive-behavioral problems, but distinct social phenotypes. Despite these social differences both syndromes display poor social relations which may result from abnormal social processing. This study aimed to manipulate the location of socially salient information within scenes to investigate the visual attentional mechanisms of: capture, disengagement, and/or general engagement. Findings revealed that individuals with FXS avoid social information presented centrally, at least initially. The WS findings, on the other hand, provided some evidence that difficulties with attentional disengagement, rather than attentional capture, may play a role in the WS social phenotype. These findings are discussed in relation to the distinct social phenotypes of these two disorders.

Social approach and emotion recognition in fragile X syndrome.

Evidence is emerging that individuals with Fragile X syndrome (FXS) display emotion recognition deficits, which may contribute to their significant social difficulties. The current study investigated the emotion recognition abilities, and social approachability judgments, of FXS individuals when processing emotional stimuli. Relative to chronological age- (CA-) and mental age- (MA-) matched controls, the FXS group performed significantly more poorly on the emotion recognition tasks, and displayed a bias towards detecting negative emotions. Moreover, after controlling for emotion recognition deficits, the FXS group displayed significantly reduced ratings of social approachability. These findings suggest that a social anxiety pattern, rather than poor socioemotional processing, may best explain the social avoidance observed in FXS.

Automatic attentional orienting to other people’s gaze in schizophrenia

Explicit tests of social cognition have revealed pervasive deficits in schizophrenia. Less is known of automatic social cognition in schizophrenia. We used a spatial orienting task to investigate automatic shifts of attention cued by another persons eye gaze in 29 patients and 28 controls. Central photographic images of a face with eyes shifted left or right, or looking straight ahead, preceded targets that appeared left or right of the cue. To examine automatic effects, cue direction was non-predictive of target location. Cue–target intervals were 100, 300, and 800 ms. In non-social control trials, arrows replaced eye-gaze cues. Both groups showed automatic attentional orienting indexed by faster reaction times (RTs) when arrows were congruent with target location across all cue–target intervals. Similar congruency effects were seen for eye-shift cues at 300 and 800 ms intervals, but patients showed significantly larger congruency effects at 800 ms, which were driven by delayed responses to incongruent target locations. At short 100-ms cue–target intervals, neither group showed faster RTs for congruent than for incongruent eye-shift cues, but patients were significantly slower to detect targets after direct-gaze cues. These findings conflict with previous studies using schematic line drawings of eye-shifts that have found automatic attentional orienting to be reduced in schizophrenia. Instead, our data indicate that patients display abnormalities in responding to gaze direction at various stages of gaze processing—reflected by a stronger preferential capture of attention by another persons direct eye contact at initial stages of gaze processing and difficulties disengaging from a gazed-at location once shared attention is established.

Facial emotion recognition, face scan paths, and face perception in children with neurofibromatosis type 1.

Objective: This study aimed to investigate face scan paths and face perception abilities in children with Neurofibromatosis Type 1 (NF1) and how these might relate to emotion recognition abilities in this population. Method: The authors investigated facial emotion recognition, face scan paths, and face perception in 29 children with NF1 compared to 29 chronological age-matched typically developing controls. Correlations between facial emotion recognition, face scan paths, and face perception in children with NF1 were examined. Results: Children with NF1 displayed significantly poorer recognition of fearful expressions compared to controls, as well as a nonsignificant trend toward poorer recognition of anger. Athough there was no significant difference between groups in time spent viewing individual core facial features (eyes, nose, mouth, and nonfeature regions), children with NF1 spent significantly less time than controls viewing the face as a whole. Children with NF1 also displayed significantly poorer face perception abilities than typically developing controls. Facial emotion recognition deficits were not significantly associated with aberrant face scan paths or face perception abilities in the NF1 group. Conclusions: These results suggest that impairments in the perception, identification, and interpretation of information from faces are important aspects of the social–cognitive phenotype of NF1.

The neuropsychological profile of children with basal ganglia encephalitis: a case series

Inflammatory basal ganglia encephalitis (BGE) is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Unlike most brain injuries, BGE is a radiologically pure basal ganglia syndrome. The current study systematically describes the neuropsychological outcomes of four paediatric cases of BGE, and thus the neuropsychological outcomes of focal basal ganglia insult in childhood. Although all patients made significant motor recoveries, all four cases displayed executive dysfunction, fine motor difficulties, and anxiety. Three out of four cases displayed attention deficits. The case who received intravenous immunoglobulin (IVIg) treatment and steroids during the acute phase of the disease had the best cognitive outcome. These findings highlight the need for detailed neuropsychological assessment and long‐term follow‐up.

Social Competence in Children with Neurofibromatosis Type 1: Relationships with Psychopathology and Cognitive Ability

Background: Neurofibromatosis type 1 (NF1) is a neurodevelopmental disorder associated with elevated risk of specific cognitive impairments and a high prevalence of psychological comorbidities. Children with NF1 have also been reported to display significant difficulties with peer relationships, although the exact nature of their social competence difficulties remains unclear. This study aimed to explore the nature of the day to day social competence difficulties observed in children with NF1 and to investigate how these difficulties might relate to cognitive dysfunction and symptoms of psychopathology. Methods and Findings: This study investigated parent ratings of day to day social competence in 23 children with neurofibromatosis type 1 (NF1) compared to 23 chronological age-matched typically developing controls using a brief, standardised questionnaire - the Social Competence with Peers Questionnaire. The relationships between social competence, psychopathology (parent ratings of Attention Deficit Hyperactivity Disorder or Autism Spectrum Disorder symptomatology), and cognitive ability (Full Scale IQ and parent ratings of functional executive behaviour) in children with NF1 were also explored using correlational analyses. Results indicated that children with NF1 displayed significantly poorer day to day social competence than controls. These social competence deficits were not related to Attention Deficit Hyperactivity Disorder symptomatology, Full Scale IQ or functional executive behaviour. However, difficulties with social competence were significantly related to Autism Spectrum Disorder symptomatology and socially anxious/avoidant behaviours in our NF1 cohort. Conclusions: These results indicate that children with NF1 are at significant risk of day to day social competence difficulties, especially those who display high levels of autistic symptomatology and socially anxious behaviour. Our findings suggest a need to incorporate screening for social competence problems and comorbid psychopathology into the more general clinical management of children with NF1.

Hyper-reactivity in fragile X syndrome females: Generalised or specific to socially-salient stimuli? A skin conductance study

Fragile X syndrome (FXS) is characterised by hyper-reactivity, autistic tendencies and social anxiety. It has been hypothesised that the FXS social phenotype is secondary to a generalised hyper-reactivity that leads to social avoidance. No study, however, has investigated whether hyperarousal in FXS is generalised or more specific to socially salient information. We recorded skin conductance responses (SCRs) while females with FXS, as well as chronological age-(CA-) and mental age-(MA-) matched controls, viewed two sets of visual images: direct-gaze emotional faces and affectively arousing scenes. Explicit emotion recognition and subjective ratings of emotions aroused by images were also recorded. Overall, females with FXS displayed hyper-reactivity only when viewing the more socially salient stimuli (emotional faces), compared to CA-matched controls, but not MA-matched controls. Moreover, females with FXS also displayed atypical emotion recognition abilities and subjective ratings of their own emotional states. These findings suggest that any hyper-reactivity observed in FXS may be more specific to socially salient stimuli, rather than generalised.

Attention to faces in social context in children with neurofibromatosis type 1

To examine visual attention to faces within social scenes in children with neurofibromatosis type 1 (NF1) and typically developing peers.