Cortical signatures of auditory object binding in children with autism spectrum disorder are anomalous in concordance with behavior and diagnosis.

Organizing sensory information into coherent perceptual objects is fundamental to everyday perception and communication. In the visual domain, indirect evidence from cortical responses suggests that children with autism spectrum disorder (ASD) have anomalous figure-ground segregation. While auditory processing abnormalities are common in ASD, especially in environments with multiple sound sources, to date, the question of scene segregation in ASD has not been directly investigated in audition. Using magnetoencephalography, we measured cortical responses to unattended (passively experienced) auditory stimuli while parametrically manipulating the degree of temporal coherence that facilitates auditory figure-ground segregation. Results from 21 children with ASD (aged 7-17 years) and 26 age- and IQ-matched typically developing children provide evidence that children with ASD show anomalous growth of cortical neural responses with increasing temporal coherence of the auditory figure. The documented neurophysiological abnormalities did not depend on age, and were reflected both in the response evoked by changes in temporal coherence of the auditory scene and in the associated induced gamma rhythms. Furthermore, the individual neural measures were predictive of diagnosis (83% accuracy) and also correlated with behavioral measures of ASD severity and auditory processing abnormalities. These findings offer new insight into the neural mechanisms underlying auditory perceptual deficits and sensory overload in ASD, and suggest that temporal-coherence-based auditory scene analysis and suprathreshold processing of coherent auditory objects may be atypical in ASD.

Is Reconstruction of Unstable Midfoot Charcot Neuroarthropathy Cost Effective from a US Payer's Perspective?

BACKGROUND: Charcot neuroarthropathy is a morbid and expensive complication of diabetes that can lead to lower extremity amputation. Current treatment of unstable midfoot deformity includes lifetime limb bracing, primary transtibial amputation, or surgical reconstruction of the deformity. In the absence of a widely adopted treatment algorithm, the decision to pursue more costly attempts at reconstruction in the United States continues to be driven by surgeon preference. QUESTIONS/PURPOSES: To examine the cost effectiveness (defined by lifetime costs, quality-adjusted life-years [QALYs] and incremental cost-effectiveness ratio [ICER]) of surgical reconstruction and its alternatives (primary transtibial amputation and lifetime bracing) for adults with diabetes and unstable midfoot Charcot neuroarthropathy using previously published cost data. METHODS: A Markov model was used to compare Charcot reconstruction and its alternatives in three progressively worsening clinical scenarios: no foot ulcer, uncomplicated (or uninfected) ulcer, and infected ulcer. Our base case scenario was a 50-year-old adult with diabetes and unstable midfoot deformity. Patients were placed into health states based on their disease stage. Transitions between health states occurred annually using probabilities estimated from the evidence obtained after systematic review. The time horizon was 50 cycles. Data regarding costs were obtained from a systematic review. Costs were converted to 2019 USD using the Consumer Price Index. The primary outcomes included the long-term costs and QALYs, which were combined to form ICERs. Willingness-to-pay was set at USD 100,000/QALY. Multiple sensitivity analyses and probabilistic analyses were performed to measure model uncertainty. RESULTS: The most effective strategy for patients without foot ulcers was Charcot reconstruction, which resulted in an additional 1.63 QALYs gained and an ICER of USD 14,340 per QALY gained compared with lifetime bracing. Reconstruction was also the most effective strategy for patients with uninfected foot ulcers, resulting in an additional 1.04 QALYs gained, and an ICER of USD 26,220 per QALY gained compared with bracing. On the other hand, bracing was cost effective in all scenarios and was the only cost-effective strategy for patents with infected foot ulcers; it resulted in 6.32 QALYs gained and an ICER of USD 15,010 per QALY gained compared with transtibial amputation. As unstable midfoot Charcot neuroarthropathy progressed to deep infection, reconstruction lost its value (ICER USD 193,240 per QALY gained) compared with bracing. This was driven by the increasing costs associated with staged surgeries, combined with a higher frequency of complications and shorter patient life expectancies in the infected ulcer cohort. The findings in the no ulcer and uncomplicated ulcer cohorts were both unchanged after multiple sensitivity analyses; however, threshold effects were identified in the infected ulcer cohort during the sensitivity analysis. When the cost of surgery dropped below USD 40,000 or the frequency of postoperative complications dropped below 50%, surgical reconstruction became cost effective. CONCLUSIONS: Surgeons aiming to offer both clinically effective and cost-effective care would do well to discuss surgical reconstruction early with patients who have unstable midfoot Charcot neuroarthropathy, and they should favor lifetime bracing only after deep infection develops. Future clinical studies should focus on methods of minimizing surgical complications and/or reducing operative costs in patients with infected foot ulcers. LEVEL OF EVIDENCE: Level II, economic and decision analysis.

People with Williams syndrome process faces holistically.

This study compared the performance of 47 adolescents and adults with Williams syndrome to 39 age-matched controls on a face recognition task. Using the whole-part paradigm developed by Tanaka and his colleagues, we found that although performance overall was lower in the participants with Williams syndrome, both groups showed similar patterns of performance across the different conditions. Both groups performed significantly better in the whole-face than in the isolated-part test condition for upright faces, but not for inverted faces. The whole-face advantage only in the upright condition provides strong evidence that people with Williams syndrome encode and recognize faces holistically in the same way as normal controls, suggesting the use of similar underlying neurocognitive mechanisms. These findings contradict earlier reports in the literature that people with Williams syndrome process faces abnormally.

Holistic and part-based face recognition in children with autism.

BACKGROUND: There is substantial evidence that children with autism are impaired in face recognition. Although many researchers have suggested that this impairment derives from a failure of holistic face processing and a tendency to represent and encode faces on a part-by-part basis, this hypothesis has not been tested directly. METHOD: Holistic face processing was assessed by comparing children's ability to recognize a face part (eyes, nose, or mouth) in the context of the whole face in which it was learned with their ability to recognize the same face part in isolation. RESULTS: In Study 1, as expected, typically developing 9-year-olds (n = 27) and 11-year-olds (n = 30) were significantly better at recognizing face parts presented in the whole than in the part test condition, and this effect was limited to upright faces and not found for inverted faces. Consistent with prior findings, typically developing children were most accurate when face recognition depended on the eyes. In Study 2, high-functioning children with autism (n = 22) evidenced a whole-test advantage for mouths only, and were markedly deficient when face recognition depended on the eyes. Their pattern of performance diverged from age- and IQ-matched comparison participants (n = 20), who performed similarly to the typically developing children in Study 1. CONCLUSIONS: These findings suggest that face recognition abnormalities in autism are not fully explained by an impairment of holistic face processing, and that there is an unusual significance accorded to the mouth region when children with autism process information from people's faces.