Reward processing in autism: a thematic series

This thematic series presents theoretical and empirical papers focused on understanding autism from the perspective of reward processing deficits. Although the core symptoms of autism have not traditionally been conceptualized with respect to altered reward-based processes, it is clear that brain reward circuitry plays a critical role in guiding social and nonsocial learning and behavior throughout development. Additionally, brain reward circuitry may respond to social sources of information in ways that are similar to responses to primary rewards, and recent clinical data consistently suggest abnormal behavioral and neurobiologic responses to rewards in autism. This thematic series presents empirical data and review papers that highlight the utility of considering autism from the perspective of reward processing deficits. Our hope is that this novel framework may further elucidate autism pathophysiology, with the ultimate goal of yielding novel insights with potential therapeutic implications.     

Preferential attention to animals and people is independent of the amygdala

The amygdala is thought to play a critical role in detecting salient stimuli. Several studies have taken ecological approaches to investigating such saliency, and argue for domain-specific effects for processing certain natural stimulus categories, in particular faces and animals. Linking this to the amygdala, neurons in the human amygdala have been found to respond strongly to faces and also to animals. However, the amygdala’s necessary role for such category-specific effects at the behavioral level remains untested. Here we tested four rare patients with bilateral amygdala lesions on an established change-detection protocol. Consistent with prior published studies, healthy controls showed reliably faster and more accurate detection of people and animals, as compared with artifacts and plants. So did all four amygdala patients: there were no differences in phenomenal change blindness, in behavioral reaction time to detect changes or in eye-tracking measures. The findings provide decisive evidence against a critical participation of the amygdala in rapid initial processing of attention to animate stimuli, suggesting that the necessary neural substrates for this phenomenon arise either in other subcortical structures (such as the pulvinar) or within the cortex itself.     

Necrotizing soft tissue infection of the scalp after fronto-facial advancement by internal distraction in a 7-year old girl with Gorlin–Chaudhry–Moss syndrome – A case report

In 1960, Gorlin, Chaudhry and Moss described a syndrome consisting of craniofacial dysostosis in association with hypertrichosis, cardiac, genital, dental and ocular anomalies. Diagnosis is based on typical clinical findings and cannot be performed by molecular genetic analysis until now. There is little in the clinical literature concerning this rare craniofacial syndrome.For functional and psychosocial reasons, surgical correction of the complex craniofacial malformation in a 7-year old Hungarian girl with Gorlin–Chaudhry–Moss syndrome was performed by fronto-facial advancement using internal distraction devices. Postoperatively necrotizing soft tissue infection of the scalp developed leading to termination of the distraction process ahead of schedule and requiring aggressive surgical management. Typical physiological and clinical characteristics were observed both during the initial craniofacial correction as well as during the management of the infectious complication suggesting that the linking of different conditions (surgical trauma plus the selection of toxic microorganisms) has caused tissue destruction rather than the syndromal disorder or the surgical technique of distraction osteogenesis. Although skeletal improvement was achieved residual damage from the infectious complication must be considered as severe.     

Normal recognition of emotional similarity between facial expressions following bilateral amygdala damage.

Bilateral damage to the amygdala in humans has been previously linked to two deficits in recognizing emotion in facial expressions: recognition of individual basic emotions, especially fear, and recognition of similarity among emotional expressions. Although several studies have examined recognition of individual emotions following amygdala damage, only one subject has been examined on recognition of similarity. To assess the extent to which deficits in recognizing similarity among facial expressions might be a general consequence of amygdala damage, we examined this ability in two subjects with complete bilateral amygdala damage. Both subjects had previously demonstrated entirely normal recognition of individual facial emotions. Here we report that these two patients also are intact in their ability to recognize similarity between emotional expressions. These results indicate that, like the recognition of individual basic emotions in facial expressions, the recognition of similarity among emotional expressions does not have an absolute dependence on the amygdala.     

Towards the neural basis for hypersociability in a genetic syndrome.

Williams syndrome (WMS), a rare disorder with a distinctive profile of medical, psychological, neurophysiological and neuroanatomical characteristics, results from hemizygous deletion of about 20 genes. The phenotype exhibits specific dissociations in higher cognitive functions: general cognitive deficits but spared linguistic abilities; extreme spatial cognitive deficits, but intact face processing. Of special interest is an unusual social phenotype in WMS: an overly friendly, engaging personality and excessive sociability with strangers. In this first experimental study of social behavior in WMS, we report that WMS subjects show an abnormal positive bias in their social judgments of unfamiliar individuals, consistent with their behavior in real life. Our findings contribute to an understanding of the neural and genetic bases of human social behavior.     

Rekonstruktion der Orbita mit Polylaktat-Implantaten: tierexperimentelle Ergebnisse nach 12 Monaten und klinischer Ausblick

In früheren Versuchen am experimentellen Modell gro?er Orbitadefekte beim Schaf konnten wir zeigen, dass bei der Rekonstruktion mit biodegradierbaren Membranen ohne zus?tzliche stützende Knochentransplantate oder Titan-Miniplatten-Osteosynthesen die Heilung durch osteokonduktives Knochenwachstum entlang den Membranen am ehesten ungest?rt verlief. In der vorliegenden Arbeit wurden gleichartige Defekte einseitig mit einer 0,5 mm dicken mikropor?sen Poly (L/DL 80/20)Laktatmembran überbrückt, auf der Gegenseite erfolgte die Rekonstruktion mit 0,25 mm dicken Membranen und stabilen Stützelementen aus dem gleichen Polylaktat. Nach 12-monatiger Beobachtung bei 9 Schafen ergab sich radiologisch eine seitengleiche anatomische Rekonstruktion der Orbitae. Histologisch zeigten sich dagegen ausgepr?gte Fremdk?rperreaktionen um degradierende Polylaktatmassen, welche vor allem um die degradierenden Stützelemente stattfanden, jedoch auch entlang der Membranen h?ufiger nachweisbar waren, als in unserem früheren Versuch nach 4 Monaten. In keiner Orbita waren die Implantate nach 12 Monaten vollst?ndig degradiert. Für eine zukünftige klinische Anwendung bei der Orbitarekonstruktion haben mikropor?se Membranen aus Polylaktat ihr osteokonduktives Potenzial best?tigt. Von der Verwendung stabiler Elemente aus dem gleichen Material muss wegen der sp?t auftretenden, massiven Fremdk?rperreaktionen für die Orbitarekonstruktion abgesehen werden.      

Preferences for visual stimuli following amygdala damage

Bilateral damage to the human amygdala impairs retrieval of emotional and social information from faces. An important unanswered question concerns the specificity of the impairment for faces. To address this question, we examined preferences for a broad class of visual stimuli in two subjects with complete bilateral amygdala damage, both of whom were impaired in judgments of faces. Relative to controls, the subjects showed a positive bias for simple nonsense figures, color patterns, three-dimensional-looking objects and landscapes. The impairment was most pronounced in regard to those stimuli that are normally liked the least. The human amygdala thus appears to play a general role in guiding preferences for visual stimuli that are normally judged to be aversive.     

The influence of autonomic arousal and semantic relatedness on memory for emotional words.

Increased memory for emotional stimuli is a well-documented phenomenon. Emotional arousal during the encoding of a stimulus is one mediator of this memory enhancement. Other variables such as semantic relatedness also play a role in the enhanced memory for emotional stimuli, especially for verbal stimuli. Research has not addressed the contributions of emotional arousal, indexed by self-report and autonomic measures, and semantic relatedness on memory performance. Twenty young adults (10 women) were presented neutral-unrelated words, school-related words, moderately arousing emotional words, and highly arousing taboo words while heart rate and skin conductance were measured. Memory was tested with free recall and recognition tests. Results showed that taboo words, which were both semantically related and high arousal were remembered best. School-related words, which were high on semantic relatedness but low on arousal, were remembered better than the moderately arousing emotional words and semantically unrelated neutral words. Psychophysiological responses showed that within the moderately arousing emotional and neutral word groups, those words eliciting greater autonomic activity were better remembered than words that did not elicit such activity. These results demonstrate additive effects of semantic relatedness and emotional arousal on memory. Relatedness confers an advantage to memory (as in the school-words), but the combination of relatedness and arousal (as in the taboo words) results in the best memory performance.     

Effect of intralesional and systemic BCG-application or a combined cyclophosphamide/BCG treatment on experimental bladder cancer

Summary Infiltrating transitional cell carcinomas of the urinary bladder were induced by ingestion of 0.188% N-[4-(5-nitro-2-furyl)-2-thiazolyl] formamide (FANFT) in 145 female Wistar rats. After 8 months of carcinogen exposure, the animals were divided into different treatment groups. They received cyclophosphamide intraperitoneally as a single injection or BCG either once intralesionally or weekly subcutaneously or a combination of cyclophosphamide followed by subcutaneous BCG. The treatment effect was determined by body weight measurements and bladder tumour weight after 12 months. Compared with a control group statistically significant differences of bladder tumour weights were found after treatment with BCG alone or in combination with cyclophosphamide. Intralesional BCG resulted in an insignificant increase of tumour weights.     

The natural history of nephrogenic adenoma of the urinary bladder.

The nephrogenic adenoma is a peculiar lesion of the urinary bladder characterized partly by villous and partly by gland-like structures. The two layered surface epithelium consists of an eosinophilic cell layer, which covers a clear cell layer, the latter resting upon a delicate basal layer. The clear cells disappear when approaching the tip of the villi. There exists a 200 micron wide zone of transition between the urinary bladder epithelium and that of the neprogenic adenoma. At the base of the villi there are scattered nodules of gland-like structures lined either by eosinophilic cells or by clear cells. These epithelial cells are derived from the corresponding surface epithelial cells from which they had separated by active downwards growth.