Children with facial paralysis due to Moebius syndrome exhibit reduced autonomic modulation during emotion processing

Abstract

Background Facial mimicry is crucial in the recognition of others’ emotional state. Thus, the observation of others’ facial expressions activates the same neural representation of that affective state in the observer, along with related autonomic and somatic responses. What happens, therefore, when someone cannot mimic others’ facial expressions? Methods We investigated whether psychophysiological emotional responses to others’ facial expressions were impaired in 13 children (9 years) with Moebius syndrome (MBS), an extremely rare neurological disorder (1/250,000 live births) characterized by congenital facial paralysis. We inspected autonomic responses and vagal regulation through facial cutaneous thermal variations and by the computation of respiratory sinus arrhythmia (RSA). These parameters provide measures of emotional arousal and show the autonomic adaptation to others’ social cues. Physiological responses in children with MBS were recorded during dynamic facial expression observation and were compared to those of a control group (16 non-affected children, 9 years). Results There were significant group effects on thermal patterns and RSA, with lower values in children with MBS. We also observed a mild deficit in emotion recognition in these patients. Conclusion Results support “embodied” theory, whereby the congenital inability to produce facial expressions induces alterations in the processing of facial expression of emotions. Such alterations may constitute a risk for emotion dysregulation.

Poland Syndrome with Atypical Malformations Associated to a de novo 1.5 Mb Xp22.31 Duplication

“…PS abnormalities can be associated to another clinical entity, the Möbius syndrome, which is characterized by unilateral or bilateral congenital facial nerve paralysis with impairment of ocular abduction frequently associated with limb anomalies. 27 However, the cranial nerve examination in our patient did not find any facial nerve dysfunction.…”

The Pacified Face: Early Embodiment Processes and the Use of Dummies

“…For example, adults with Moebius syndrome, a congenital form of facial paralysis, are able to recognize photographs of emotion expressions with the same accuracy as control participants (Bogart and Matsumoto, 2010). However, a recent study (De Stefani et al, 2019) revealed that, compared with a control group, children with Moebius syndrome were slightly less accurate in labeling videos of facial expressions and showed less pronounced responses of parasympathetic system during observation of social stimuli. In addition, although adults with acute facial palsy are able to accurately recognize facial expression, these judgments can take longer than in control participants (Storbeck et al, 2019).…”

The intensity of emotion: Altered motor simulation impairs processing of facial expressions in congenital facial palsy

“…The third line of evidence is strictly connected to the facial mimicry mechanism, demonstrating that both in case of blockage/alteration by means of various experimental manipulations (e.g. pens or chopsticks [Ponari, Conson, D'Amico, Grossi, & Trojano, 2012], mouthguards [Rychlowska et al, 2014], hardening gels , botulinum [Baumeister, Papa, & Foroni, 2016]) and in case of individuals suffering from congenital or acquired facial and expression production disorders, difficulties in recognizing facial expressions have been reported (Bate, Cook, Mole, & Cole, 2013;Calder, Keane, Cole, Campbell, & Young, 2000;Giannini et al, 1984;McKone & Robbins, 2011;Nicolini et al, 2019;De Stefani et al 2019). This whole body of evidence supports the notion that the simulation mechanism may optimally work when facial feedback is consistent with the internal simulation (Wolpert & Flanagan, 2001); thus, interfering with facial mimicry may cause an incongruent signal with the pattern of sensorimotor activity and would result in a drop of recognition/discrimination accuracy (Wingenbach, Brosnan, Pfaltz, Plichta, & Ashwin, 2018;.…”

“…In this vein, we involved subjects with Moebius Syndrome (MBS) 1 , an extremely rare congenital non-progressive condition resulting in severe or complete bilateral facial paralysis, resulting in a selective alteration of facial mimicry. Given the rarity of the syndrome, only few studies have investigated emotion recognition in MBS subjects, and, further, they present inconsistent results (Bate et al, 2013;Calder et al, 2000;Giannini et al, 1984;Nicolini et al, 2019;De Stefani et al, 2019).…”

“…On the contrary, few studied reported as MBS subjects did not differ from the matched control group or normative data in facial expression recognition accuracy (Bate et al, 2013;Calder et al, 2000;, leading to the conclusion that facial mimicry and facial feedback are not necessary to recognize facial expressions and that accuracy was not related to the MBS ability to produce facial expressions in the first person. With a certain degree of agreement, researchers in this field, embracing a neuroconstructivist perspective, proposed the idea that MBS individuals, who have lived without the ability to mimic facial expressions for their entire lives, have developed compensatory mechanisms for the role played by facial mimicry/simulation in emotion recognition and do not use the same neural pathways as typical developed individuals (McIntosh, Reichmann-Decker, Winkielman, & Wilbarger, 2006;De Stefani et al 2019).…”