Möbius syndrome and transposition of the great vessels

Raroque, Henry G.; Hershewe, Gerard L.; Snyder, Russell D.

doi:10.1212/wnl.38.12.1894

Cited by 24 publications

(14 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…26,50,51 During the fourth and fifth week of development, cephalic neural crest cells begin to differentiate, starting from the CN nuclei. 21,52 The proximity between facial nerve fibers and the abducens nucleus at the cerebellopontine angle is a potential trigger of concurrent palsies. 26 During the sixth week of gestation, the blood supply to the nascent hindbrain changes from the basilar to the vertebral artery and specific timing is crucial to guarantee adequate blood supply to the CN nuclei.…”

Section: And Vonmentioning

confidence: 99%

Congenital facial palsy and emotion processing: The case of Moebius syndrome

Stefani

Nicolini

Belluardo

et al. 2019

Genes Brain and Behavior

View full text Add to dashboard Cite

According to the Darwinian perspective, facial expressions of emotions evolved to quickly communicate emotional states and would serve adaptive functions that promote social interactions. Embodied cognition theories suggest that we understand others' emotions by reproducing the perceived expression in our own facial musculature (facial mimicry) and the mere observation of a facial expression can evoke the corresponding emotion in the perceivers. Consequently, the inability to form facial expressions would affect the experience of emotional understanding. In this review, we aimed at providing account on the link between the lack of emotion production and the mechanisms of emotion processing. We address this issue by taking into account Moebius syndrome, a rare neurological disorder that primarily affects the muscles controlling facial expressions. Individuals with Moebius syndrome are born with facial paralysis and inability to form facial expressions. This makes them the ideal population to study whether facial mimicry is necessary for emotion understanding. Here, we discuss behavioral ambiguous/mixed results on emotion recognition deficits in Moebius syndrome suggesting the need to investigate further aspects of emotional processing such as the physiological responses associated with the emotional experience during developmental age.

show abstract

Section: And Vonmentioning

confidence: 99%

Congenital facial palsy and emotion processing: The case of Moebius syndrome

Stefani

Nicolini

Belluardo

et al. 2019

Genes Brain and Behavior

View full text Add to dashboard Cite

show abstract

“…Cardiac defects, including ventricular septal defects and transposition of the great vessels, have been documented. 161,162 Oropharyngeal malformations, including cleft palate, retrogenia, and microstomia, have been described. [163][164][165] Clinical presentation associated with facial nerve palsies includes incomplete eye closure during sleep and oral incompetence.…”

Section: Möbius Syndromementioning

confidence: 99%

Craniofacial Syndromes

Buchanan

Xue

Hollier

2014

Plastic and Reconstructive Surgery

170

View full text Add to dashboard Cite

Craniofacial syndromes fall into two major categories-those associated with craniosynostosis, and those associated with clefts. Each has a different set of potential complications requiring a unique approach for surgical management. Craniosynostosis is a congenital disorder in which one or more of the cranial sutures fuses prematurely. The most common syndromes associated with this condition include Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes. Surgical management of these children requires a multidisciplinary approach and close involvement of the family. Operations must take into consideration the growing potential of the bony structures. Common syndromes associated with clefts include Pierre Robin, Treacher Collins, Nager, Binder, and Stickler syndromes. Many of these children have severe airway issues requiring immediate address before operative reconstruction. As with syndromes associated with craniosynostosis, the key to management is a multidisciplinary approach focused on the right timing. The purpose of this article is to review the clinical presentation, care, and treatment of these patients.

show abstract

“…During the fourth and fifth week of development, cephalic neural crest cells begin to differentiate into neural structures, beginning with the cranial nerve nuclei [11,19]. The close proximity between facial nerve fibers and the abducens nucleus at the cerebellopontine angle provide a possible reason for the development of concurrent palsies.…”

Section: Embryogenesismentioning

confidence: 99%

“…Extremity development and cardiac organogenesis occurs during the fourth through seventh weeks, with the heart being complete at week 5, thus leading to the observed abnormalities. It can be surmised that the proposed disturbance in embryogenesis underlying Moebius syndrome occurs at some point during the fourth to seventh week of gestation [10,11,19].…”

Section: Embryogenesismentioning

confidence: 99%

“…Other abnormalities include lingual hypoplasia, sensorineural hearing loss, craniofacial malformations (epicanthic folds, micrognathia), and abnormalities of the extremities (syndactyly, pes planus, valgus femur) [6,8]. Cardiovascular abnormalities are rarely present but can include dextrocardial, atrial, or ventricular septal defect, transposition of the great vessels, and total anomalous pulmonary venous connection [9][10][11][12][13]. Although most patients have normal intelligence, approximately 10 % of patients have mental retardation, and another 30-40 % may be diagnosed with autism [8,9].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation