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Frontiers in Human Neuroscience 2012Social cognition researchers have become increasingly interested in the ways that behavioral, physiological, and neural coupling facilitate social interaction and...
Social cognition researchers have become increasingly interested in the ways that behavioral, physiological, and neural coupling facilitate social interaction and interpersonal understanding. We distinguish two ways of conceptualizing the role of such coupling processes in social cognition: strong and moderate interactionism. According to strong interactionism (SI), low-level coupling processes are alternatives to higher-level individual cognitive processes; the former at least sometimes render the latter superfluous. Moderate interactionism (MI) on the other hand, is an integrative approach. Its guiding assumption is that higher-level cognitive processes are likely to have been shaped by the need to coordinate, modulate, and extract information from low-level coupling processes. In this paper, we present a case study on Möbius Syndrome (MS) in order to contrast SI and MI. We show how MS-a form of congenital bilateral facial paralysis-can be a fruitful source of insight for research exploring the relation between high-level cognition and low-level coupling. Lacking a capacity for facial expression, individuals with MS are deprived of a primary channel for gestural coupling. According to SI, they lack an essential enabling feature for social interaction and interpersonal understanding more generally and thus ought to exhibit severe deficits in these areas. We challenge SI's prediction and show how MS cases offer compelling reasons for instead adopting MI's pluralistic model of social interaction and interpersonal understanding. We conclude that investigations of coupling processes within social interaction should inform rather than marginalize or eliminate investigation of higher-level individual cognition.
PubMed: 22514529
DOI: 10.3389/fnhum.2012.00081 -
The Cleft Palate-craniofacial Journal :... Mar 2013Objective : Reconstructive surgery to improve psychological well-being is commonly offered to children with craniofacial conditions. Few studies have explored the...
Objective : Reconstructive surgery to improve psychological well-being is commonly offered to children with craniofacial conditions. Few studies have explored the challenges of reconstructive surgery beyond the physical risks: poor treatment outcomes, infection, brain damage, and death. This qualitative study aims to understand the psychological and social implications such interventions can have for individuals with craniofacial conditions. Design : A total of 38 individuals between the ages of 12 and 61 with such craniofacial conditions as Sturge-Weber syndrome, Treacher Collins syndrome, Möbius syndrome, cleft lip and palate, Noonan syndrome, Crouzon syndrome, and amniotic band syndrome participated in semistructured video-recorded interviews. Participants were recruited at conferences, through study flyers, and by word of mouth. Descriptive, thematic analysis was used to identify themes related to reconstructive surgery. Results : Dominant themes included undergoing surgery to reduce stigmatization, the psychological and social implications of the interventions, outcome satisfaction, parental involvement in decision making about surgery, and recommendations for parents considering surgery for their children with craniofacial conditions. Experiences with reconstructive surgery varied, with some participants expressing surgical benefits and others, disillusionment. Conclusions : The range of participant attitudes and experiences reflect the complexity of reconstructive surgery. Pediatric health care teams involved in the care of children with craniofacial conditions play an important role in advising patients (and their parents) about existing treatment options. The psychological and social implications of reconstructive surgery should be relayed to help families weigh the risks and benefits of surgery in an informed and meaningful way.
Topics: Cleft Lip; Cleft Palate; Craniofacial Dysostosis; Humans; Mandibulofacial Dysostosis; Parents; Plastic Surgery Procedures
PubMed: 22315960
DOI: 10.1597/11-127 -
Current Opinion in Ophthalmology Sep 2011The thalidomide tragedy of the early 1960s resulted in a great number of studies and reports involving many specialties of medicine. Because of the estimated large... (Review)
Review
PURPOSE OF REVIEW
The thalidomide tragedy of the early 1960s resulted in a great number of studies and reports involving many specialties of medicine. Because of the estimated large number of affected children (5000+) worldwide exposed to this potent teratogen, and the many informative cases in which the exposure time was known, a teratogenic timetable was constructed relating affected structures to the time of exposure. This demonstrated that thalidomide had a teratogenic effect between approximately 20 to 36 days after fertilization.
RECENT FINDINGS
We found that Duane syndrome and its variants were prominent in individuals who were exposed to thalidomide early in the sensitive period (days 20 to 26±). Other anomalies associated with this early effect were aberrant tearing, facial nerve palsy, ear malformations, and autism. Structural eye malformations were less frequent in this early phase, appearing slightly later in the sensitive period.
SUMMARY
This study summarizes the ophthalmologic findings from a number of studies and compares them with respect to the implications of time of exposure. Because the timing of anomalies such as external ear and limb malformations are well established in the thalidomide literature, correlation with associated eye anomalies gives insight into the approximate timing of the causative teratogen exposure.
Topics: Abnormalities, Drug-Induced; Duane Retraction Syndrome; Eye Abnormalities; Female; Humans; Limb Deformities, Congenital; Male; Mobius Syndrome; Pregnancy; Teratogens; Thalidomide; Time Factors
PubMed: 21825994
DOI: 10.1097/ICU.0b013e3283499f24 -
Eye (London, England) Oct 2011Congenital loss of innervation to the extra-ocular muscles (EOMs) can have a profound effect on the target muscle. This has been well recognised in Duane's retraction... (Review)
Review
Congenital loss of innervation to the extra-ocular muscles (EOMs) can have a profound effect on the target muscle. This has been well recognised in Duane's retraction syndrome. However, it has been less emphasised in other congenital oculo-motor disorders. Such congenital ocular motor defects have been expanded to include DRS, congenital fibrosis of EOMs, monocular elevation defect, Möbius syndrome, as well as several other non-ocular muscles supplied by cranial nerves such as facial muscles. Such loss of innervation to motor muscles can be unified as a defined clinical entity, which can be labelled as congenital innervation dysgenesis syndrome or CID for short. CID may also affect other muscles supplied by nerves other than the cranial nerves and may be sensory as well as motor.
Topics: Duane Retraction Syndrome; Female; Humans; Male; Mobius Syndrome; Oculomotor Muscles; Pedigree
PubMed: 21720410
DOI: 10.1038/eye.2011.38 -
Neurologia (Barcelona, Spain) Oct 2011
Topics: Abortifacient Agents, Nonsteroidal; Adolescent; Anti-Ulcer Agents; Comorbidity; Female; Humans; Infant; Male; Misoprostol; Mobius Syndrome; Poland Syndrome; Pregnancy; Prenatal Exposure Delayed Effects
PubMed: 21420202
DOI: 10.1016/j.nrl.2011.01.019 -
BMJ Case Reports Feb 2011A case of Moebius syndrome is reported in an infant of a mother known to have pyridoxine-unresponsive homocystinuria. The authors suggest that Moebius syndrome could...
A case of Moebius syndrome is reported in an infant of a mother known to have pyridoxine-unresponsive homocystinuria. The authors suggest that Moebius syndrome could result from early vascular insufficiency or disruption occurring early in development related to maternal homocystinuria. Moebius syndrome consists of congenital complete or partial facial nerve palsy with or without paralysis of other cranial nerves and often in association with other malformations of the limbs and orofacial structures, but usually without gross structural brain abnormalities.
Topics: Female; Homocystinuria; Humans; Infant, Newborn; Mobius Syndrome; Pregnancy; Pregnancy Complications; Vascular Diseases
PubMed: 22707369
DOI: 10.1136/bcr.09.2010.3331 -
Der Ophthalmologe : Zeitschrift Der... Aug 2010This article reviews the spectrum of possible motility disorders and ocular misalignment in patients with Möbius sequence. The various options for strabismus surgery... (Review)
Review
This article reviews the spectrum of possible motility disorders and ocular misalignment in patients with Möbius sequence. The various options for strabismus surgery are discussed and a stepwise algorithm is presented.
Topics: Abducens Nerve Diseases; Algorithms; Humans; Mobius Syndrome; Oculomotor Muscles; Strabismus
PubMed: 20533050
DOI: 10.1007/s00347-010-2147-2 -
Der Ophthalmologe : Zeitschrift Der... Aug 2010Moebius sequence is a congenital disorder that not only affects the oculomotor system but also the eyes themselves. Ocular involvement might be sight-threatening and... (Review)
Review
Moebius sequence is a congenital disorder that not only affects the oculomotor system but also the eyes themselves. Ocular involvement might be sight-threatening and needs regular follow-up by an ophthalmologist.
Topics: Amblyopia; Bell Palsy; Diplopia; Humans; Lacrimal Apparatus Diseases; Mobius Syndrome; Refractive Errors; Tears; Trigeminal Nerve Diseases
PubMed: 20454898
DOI: 10.1007/s00347-010-2148-1 -
Journal of AAPOS : the Official... Feb 2010The HOXA1-related syndromes result from autosomal-recessive truncating mutations in the homeobox transcription factor, HOXA1. Limited horizontal gaze and sensorineural...
The HOXA1-related syndromes result from autosomal-recessive truncating mutations in the homeobox transcription factor, HOXA1. Limited horizontal gaze and sensorineural deafness are the most common features; affected individuals can also have facial weakness, mental retardation, autism, motor disabilities, central hypoventilation, carotid artery, and/or conotruncal heart defects. Möbius syndrome is also phenotypically heterogeneous, with minimal diagnostic criteria of nonprogressive facial weakness and impaired ocular abduction; mental retardation, autism, motor disabilities, additional eye movements restrictions, hearing loss, hypoventilation, and craniofacial, lingual, and limb abnormalities also occur. We asked, given the phenotypic overlap between these syndromes and the variable expressivity of both disorders, whether individuals with Möbius syndrome might harbor mutations in HOXA1. Our results suggest that HOXA1 mutations are not a common cause of sporadic Möbius syndrome in the general population.
Topics: Child; Duane Retraction Syndrome; Exons; Genetic Predisposition to Disease; Homeodomain Proteins; Humans; Introns; Mobius Syndrome; Phenotype; Polymorphism, Single Nucleotide; Transcription Factors
PubMed: 20227628
DOI: 10.1016/j.jaapos.2009.11.007 -
Singapore Medical Journal Oct 2009
Topics: Child, Preschool; Echocardiography; Heart Septal Defects, Atrial; Humans; Male; Mobius Syndrome; Phenotype
PubMed: 19907896
DOI: No ID Found