A Systematic Review of the Psychosocial Adjustment of Children and Adolescents with Facial Palsy: The Impact of Moebius Syndrome.

INTRODUCTION: Facial palsy is often associated with impaired facial function and altered appearance. However, the literature with regards to the psychological adjustment of children and adolescents with facial palsy has not been systematically reviewed to date. This paper aimed to review all published research with regards to psychosocial adjustment for children and adolescents with facial palsy. METHODS: MEDLINE, CINAHL, Embase, PsychInfo and AMED databases were searched and data was extracted with regards to participant characteristics, study methodology, outcome measures used, psychosocial adjustment and study quality. RESULTS: Five studies were eligible for inclusion, all of which investigated psychosocial adjustment in participants with Moebius syndrome, a form of congenital facial palsy. Many parents reported their children to have greater social difficulties than general population norms, with difficulties potentially increasing with age. Other areas of psychosocial adjustment, including behaviour, anxiety and depression, were found to be more comparable to the general population. DISCUSSION: Children and adolescents with Moebius syndrome may experience social difficulties. However, they also demonstrate areas of resilience. Further research including individuals with facial palsy of other aetiologies is required in order to determine the psychosocial adjustment of children and adolescents with facial palsy.

Efficient recognition of facial expressions does not require motor simulation.

What mechanisms underlie facial expression recognition? A popular hypothesis holds that efficient facial expression recognition cannot be achieved by visual analysis alone but additionally requires a mechanism of motor simulation - an unconscious, covert imitation of the observed facial postures and movements. Here, we first discuss why this hypothesis does not necessarily follow from extant empirical evidence. Next, we report experimental evidence against the central premise of this view: we demonstrate that individuals can achieve normotypical efficient facial expression recognition despite a congenital absence of relevant facial motor representations and, therefore, unaided by motor simulation. This underscores the need to reconsider the role of motor simulation in facial expression recognition.

Autonomic Responses to Emotional Stimuli in Children Affected by Facial Palsy: The Case of Moebius Syndrome.

According to embodied simulation theories, others' emotions are recognized by the unconscious mimicking of observed facial expressions, which requires the implicit activation of the motor programs that produce a specific expression. Motor responses performed during the expression of a given emotion are hypothesized to be directly linked to autonomic responses associated with that emotional behavior. We tested this hypothesis in 9 children (M age = 5.66) affected by Moebius syndrome (MBS) and 15 control children (M age = 6.6). MBS is a neurological congenital disorder characterized by underdevelopment of the VI and VII cranial nerves, which results in paralysis of the face. Moebius patients' inability to produce facial expressions impairs their capacity to communicate emotions through the face. We therefore assessed Moebius children's autonomic response to emotional stimuli (video cartoons) by means of functional infrared thermal (fIRT) imaging. Patients showed weaker temperature changes compared to controls, suggesting impaired autonomic activity. They also showed difficulties in recognizing facial emotions from static illustrations. These findings reveal that the impairment of facial movement attenuates the intensity of emotional experience, probably through the diminished activation of autonomic responses associated with emotional stimuli. The current study is the first to investigate emotional responses in MBS children, providing important insights into the role of facial expressions in emotional processing during early development.

Psychological adjustment of young subjects with Möbius sequence and their primary caregivers' strain and life satisfaction: First longitudinal data.

AIMS/METHODS: At present, there is no information about the course of psychological adjustment in young subjects with Möbius sequence (MS) and their parents' strain and life satisfaction. To fill this gap, we performed a four-year follow-up study. Parents were anonymously asked to fill out the Child Behavior Checklist 4-18 [CBCL/ 4-18] or the Young Adult Behavior Checklist 18-30 [YABCL/ 18-30] and the Freiburger Personality Inventory-Revised [FPI-R], subscales strain and life satisfaction. RESULTS: 12 males and 14 females (mean: 15.20 years, standard deviation: 3.48 years) could be included in the follow-up (response rate: 83.9%).Compared to the general population, subjects with MS showed significantly higher scores on almost all CBCL scales (exception: externalizing problems) at T1 and T2. At both study times, parental strain and life satisfaction were not significantly different from findings in the general population. No significant longitudinal changes could be found for CBLC scales, parental strain and life satisfaction. CONCLUSIONS: Problems with psychological adjustment seem to be frequent among younger subjects with MS. Therefore, careful monitoring as well as early and adequate interventions, if indicated, are crucial for subjects with MS, not only with regard to somatic complaints but also to aspects of adjustment.

"Rare place where I feel normal": Perceptions of a social support conference among parents of and people with Moebius syndrome.

BACKGROUND: Moebius syndrome is a rare congenital disorder resulting in impaired facial and eye movement. People with rare diseases like Moebius syndrome experience stigma and a lack of specialized information. Support conferences may provide important forms of social support for people with rare disorders. AIMS: To examine reasons for attending, benefits, and limitations of support conferences. METHODS AND PROCEDURES: 50 adults with Moebius syndrome and 57 parents of people with Moebius syndrome completed open-ended items in an online study. OUTCOMES AND RESULTS: Mixed- methods content analysis revealed that companionship and informational support were most frequently mentioned as reasons for and benefits of attending. Finances were the most frequently mentioned reason for not attending. Parents were more likely than people with Moebius to describe instrumental support as a conference benefit. When describing conference limitations, parents were significantly more concerned by lack of information relevance, while people with Moebius noted more often that conference attributes were not relevant to their age. CONCLUSIONS AND IMPLICATIONS: Being surrounded by others who share one's condition offers a unique opportunity for destigmatizing companionship support, which normalizes, reduces isolation, and promotes solidarity. Ways to increase facilitators and decrease barriers to accessing support for rare disorders should be investigated.

Quality of life and adjustment in children and adolescents with Moebius syndrome: Evidence for specific impairments in social functioning.

INTRODUCTION: Moebius syndrome is a rare congenital disorder characterized by congenital facial paralysis and impairment of ocular abduction. The absence of facial expression in people with Moebius syndrome may impair social interaction. The present study aims at replicating prior findings on psychological adjustment in children and adolescents with Moebius syndrome and providing first data on quality of life. PARTICIPANTS AND METHODS: Parents (n=26) and children and adolescents (n=14) with Moebius-Syndrome completed the KINDL(R) (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents), the SDQ (Strengths and Difficulties Questionnaire), and a newly devised questionnaire on the global, social, and emotional impact of Moebius syndrome, including a scale that addressed what conjectures were made concerning the thoughts of other people about a child with Moebius syndrome. RESULTS: In comparison with normative data, quality of life was reduced for the subscale Friends (large effect for both parental ratings and self-report) and the Total Score of the KINDL(R). Parents reported elevated levels of peer problems on the respective subscale of the SDQ. In SDQ self-report data, cases classified as abnormal were observed on Peer Problems only. Moebius-specific scales showed adequate reliabilities, and were related, most notably for parent-report, to quality of life. 29% of children reported feelings of anger when being stared at, and wished that they were not affected by Moebius syndrome. CONCLUSIONS: Although most children and adolescents in our sample did not show behavioral problems or reduced quality of life, our results indicate that their emotional and social development should be closely monitored. Maintaining satisfying peer relationships seems to be a special challenge for children and adolescents with Moebius syndrome.

Síndrome de Mõbius: significados na vida dos portadores / Mõbius syndrome: the meaning in the life of the carriers

JUSTIFICATIVA E OBJETIVO: A síndrome de Mõbius, evento raro, resulta de uma desordem neurológica que se caracteriza por paralisia congênita do sétimo par de nervos cranianos, acompanhada de mal formações límbicas e das demais estruturas orofaciais. Caracteriza-se clinicamente pela ausência de expressão facial e distúrbios da fala, principalmente. Tais alterações influenciam diretamente na vida social do portador. Trata-se de uma síndrome rara, por isso pouco estudada, sendo o aspecto emocional não abordado pela literatura atual. O objetivo deste estudo foi identificar o sentimento e o significado da síndrome na vida de seus portadores. MÉTODOS: Foram entrevistados portadores da síndrome e, por meio do Discurso do Sujeito Coletivo, buscou-se avaliar o significado da síndrome. RESULTADOS: Evidenciou-se que, apesar das malformações e das dificuldades na fala, que impõem dificuldades no dia a dia, há adaptação por parte dos sindrômicos, que vivem normalmente. CONCLUSÃO: Os portadores da síndrome de Mõbius reconhecem as dificuldades impostas por sua condição, mas se adaptam bem a elas vivendo normalmente.

BACKGROUND AND OBJECTIVE: The Moebius syndrome, a rare disorder, results from a neurological disorder that is characterized by congenital paralysis of the seventh cranial nerve accompanied by limbic and other orofacial structures malformations. It is clinically characterized by the absence of facial expression and speech disorders, especially. These changes directly influence the social life of the patient. This is a rare syndrome, so, little studied, and the emotional aspect not addressed by the current literature. The objective of this article was to identify the feeling and the meaning of the syndrome in the lives of their carriers. METHODS: Patients with the syndrome were interviewed and through the Collective Subject Discourse, we seek to assess the significance of this syndrome. RESULTS: The results showed that despite the defects and difficulties in speech, which imposes difficulties in day-to-day, there is adaptation by the syndromic, which normally live. CONCLUSION: Moebius syndrome carriers recognize the difficulties imposed by their condition, but they adapt well to living normally.

"People are all about appearances": A focus group of teenagers with Moebius Syndrome.

This focus group study examined the social experiences of 10 adolescents aged 12-17 years with Moebius Syndrome, a rare condition involving congenital facial paralysis. Content analysis revealed five themes: social engagement/disengagement, resilience/sensitivity, social support/stigma, being understood/misunderstood, and public awareness/lack of awareness of Moebius Syndrome. Compared to previous research on adults with Moebius Syndrome, adolescents were more likely to experience bullying and social strain. Like adults, teenagers displayed resilience and communication skills. Greater public awareness is key to reducing the stigma associated with this condition, and social skills programs should be developed to support those who struggle with social functioning.

Social interaction experiences of adults with Moebius Syndrome: a focus group.

This focus group study explored the social interaction experiences and strategies of 12 adults with Moebius Syndrome, a rare congenital condition characterized by facial paralysis. Content analysis revealed five themes of social functioning: social engagement/disengagement; resilience/sensitivity; social support/stigma; being understood/misunderstood; and public awareness/lack of awareness of Moebius Syndrome. Participants used compensatory expressive strategies such as vocal tone, gestures, and humor. The combination of being unable to express oneself with the face, having a facial difference, and having a rare disease is particularly stigmatizing. Increasing public awareness and developing social skills programs for people with facial paralysis could facilitate social functioning.

Self-perception of children and adolescents with Möbius sequence.

Möbius sequence is a rare congenital disorder usually defined as a combination of facial weakness with impairment of ocular abduction. Some studies suggest that psychosocial and psychiatric problems might be increased among affected persons. So far, there have been no studies on the self-perception of children and adolescents with the sequence. Seventeen participants with Möbius sequence (9 male, 8 female) aged 9-15 (mean: 11.59) years were studied. None of the probands was mentally retarded or had a diagnosis of autism spectrum disorder. Participants filled out well standardized German questionnaires on depression (Depressionsinventar für Kinder und Jugendliche [DIKJ]), anxiety (Angstfragebogen für Schüler [AFS]) and personality aspects (Persönlichkeitsfragebogen für Kinder von 9-14 Jahren [PFK 9-14]). Additionally, their primary caregivers were asked to complete a special questionnaire to compile the probands' personal, somatic and psychosocial history as well as the German version of the Strengths and Difficulties Questionnaire, Parent Form [SDQ-Deu]. According to the participants' self-perception only one girl scored clinical (t-score ≥ 63) for manifest anxiety [AFS] and depression [DIKJ] (5.9% vs. 10% in the general population). Moreover, the whole sample reported significantly lower test anxiety (p=0.000) and manifest anxiety (p=0.005) [AFS] whereas general anxiety as a personality aspect [PFK 9-14] did not differ from the normative sample. Compared to normative data, subjects expressed significantly less depression (p=0.023) and impulsivity (p=0.042). One out of 17 subjects was rated abnormal for total problems on the SDQ-Deu (5.9% vs. 10% of the normative sample), five participants scored abnormal for social problems (29.4%) and three for emotional problems (17.6%). Social problems correlated significantly with the probands' age (rho=0.707; p=0.002). As Möbius patients have severe difficulties with facial expression of feelings, and others might therefore falsely recognize them as serious or even depressed, the subjects' self-perception is crucial for assessment and diagnosis, especially if it differs considerably from that of the primary caregivers.

Living with Moebius syndrome: adjustment, social competence, and satisfaction with life.

CONTEXT: Moebius syndrome is a rare congenital condition that results in bilateral facial paralysis. Several studies have reported social interaction and adjustment problems in people with Moebius syndrome and other facial movement disorders, presumably resulting from lack of facial expression. OBJECTIVE: To determine whether adults with Moebius syndrome experience increased anxiety and depression and/or decreased social competence and satisfaction with life compared with people without facial movement disorders. DESIGN: Internet-based quasi-experimental study with comparison group. PARTICIPANTS: Thirty-seven adults with Moebius syndrome recruited through the United States-based Moebius Syndrome Foundation newsletter and Web site and 37 age- and gender-matched control participants recruited through a university participant database. MEASURES: Anxiety and depression, social competence, satisfaction with life, ability to express emotion facially, and questions about Moebius syndrome symptoms. RESULTS: People with Moebius syndrome reported significantly lower social competence than the matched control group and normative data but did not differ significantly from the control group or norms in anxiety, depression, or satisfaction with life. In people with Moebius syndrome, degree of facial expression impairment was not significantly related to the adjustment variables. CONCLUSIONS: Many people with Moebius syndrome are better adjusted than previous research suggests, despite their difficulties with social interaction. To enhance interaction, people with Moebius syndrome could compensate for the lack of facial expression with alternative expressive channels.

Behaviour problems of patients with Moebius sequence and parental stress.

AIM: Moebius sequence is a rare condition usually defined as congenital facial paralysis with congenital impairment of ocular abduction. At present, there is little information on behavioural problems, parental stress and possible relationships between these factors. To fill this gap, this study investigated these aspects relevant for counselling. METHODS: Parents of 4-17 year old subjects known to the German Möbius syndrome foundation were anonymously asked to fill out several questionnaires, for example, the Child Behavior Checklist (CBCL)4-18. RESULTS: The primary care givers of 41/58 subjects (70.7%) sent back filled-out questionnaires. Ten subjects did not meet the inclusion criteria; 15 males and 16 females (4; 7-17; 0 years, median age: 10; 7 years) were included. Ten out of 31 subjects were rated as clinical on at least one CBCL scale; three had a total problem score in the clinical range. Social problems were the most important problems with rates of 12-17-year old subjects being about five times as high as those of younger subjects. Compared with the general population, but not with other parents of mentally and/or physically handicapped children, the primary care givers experienced higher levels of stress, which were correlated to anxious/depressed behaviour, aggressive behaviour, externalising problems and total problem score of the children. The older a child the higher the primary care giver's life satisfaction was. CONCLUSIONS: Social problems seem to be frequent among 4-17-year old subjects with Moebius sequence, and primary care givers show increased strain. Therefore, families with an affected child need early and adequate support.

Autism spectrum conditions in individuals with Möbius sequence, CHARGE syndrome and oculo-auriculo-vertebral spectrum: diagnostic aspects.

As part of multidisciplinary surveys of three Behavioural Phenotype Conditions (BPCs); Möbius sequence (Möbius), CHARGE syndrome (CHARGE) and oculo-auriculo-vertebral spectrum (OAV), autism spectrum conditions (ASCs) was diagnosed in 45%, 68% and 42% of the individuals, respectively. Diagnostic difficulties due to additional dysfunctions such as mental retardation (MR), impaired vision, reduced hearing and cranial nerve dysfunction, were experienced in all three BPC groups. The applicability of current autism diagnostic instruments, such as the Autism Diagnostic Interview-Revised (ADI-R), the Childhood Autism Rating Scale (CARS) and the Autistic Behaviour Checklist (ABC), in individuals with ASCs and Möbius/CHARGE/OAV was analysed. Use of an extensive battery of diagnostic instruments, including both observational schedules and parent interviews, and, if possible, independent judgements from two clinicians, is essential in the diagnostics of ASCs in these individuals. Further, in individuals who are deaf and blind the applicability of current autism diagnostic instruments is highly questionable.

Cognitive evaluation in children and adolescents with Möbius sequence.

BACKGROUND: Möbius sequence is a rare condition usually defined as uni- or bilateral congenital facial weakness with impairment of ocular abduction. Mental retardation is estimated to occur in 10-15% of cases, but at present there have been no studies focusing on the intellectual capacities of children and adolescents with Möbius sequence. METHODS: Twenty-three children and adolescents aged 6-16 years could be recruited following a request of the German Möbius foundation. The primary caregivers of all subjects filled out a special questionnaire to compile personal, somatic and psychosocial history of the probands. All subjects had a physical examination. To assess intellectual capacities, the German version of the Wechsler Intelligence Test-III (WISC-III) was administered. In case of a severe mental retardation, the Vineland Adaptive Behavior Rating Form was used as an alternative. RESULTS: Twenty-two subjects [12 males, 10 females; mean age: 11.3 (6-16) years] could be included; 21 could be examined with the WISC-III. Compared with the normative sample, Full Scale IQ (mean: 92.05; standard deviation: 14.84) was significantly lower (P = 0.023) which was the consequence of a very low Performance IQ (mean: 80.48; standard deviation: 15.84). Compared with the normative sample, the results of all performance subtests were significantly lower (P = 0.033-0.000), whereas verbal subtest scores did not differ or were even higher ['Similarities' (P = 0.026) and 'Vocabulary' (P = 0.019)]. Verbal IQ (mean: 106.24; standard deviation: 15.31) was not significantly different from the normative sample. Two boys met ICD-10 criteria for mental retardation. Full Scale IQ was not predictive for academic success. CONCLUSIONS: The WISC-III is not an adequate predictor for academic success in Möbius patients; intelligence tests which are less dependant on time constraints should be preferred for subjects with Möbius sequence.

The faces of Moebius syndrome: recognition and anticipatory guidance.

Moebius syndrome is a rare congenital disorder characterized mainly by the inability to move the eyes laterally or produce facial expressions such as smiling. Moebius syndrome creates physical problems for the affected individual that may, in some cases, lead to emotional or social adjustment issues, yet the syndrome is relatively unknown among healthcare professionals. Because early recognition of Moebius syndrome can lead to early diagnosis and treatment, education of nurses in perinatal, pediatric, midwifery, and neonatal specialties is crucial. Through early recognition, maternal-child nurses can offer anticipatory guidance and provide or recommend resources to parents of children with this neurological condition.

Neuropsychiatric findings of Möbius sequence -- a review.

Möbius sequence is a rare condition of heterogeneous, and in most cases, unclear; origin, usually defined as a unilateral or bilateral congenital facial weakness with impairment of ocular abduction, which is frequently associated with limb anomalies. Disturbances in psychomotor and speech development are very common, and mental retardation is estimated to occur in 10-15% of cases. The incidence of autistic spectrum disorders might be increased in patients with Möbius sequence. After a brief overview on aetiology and physical appearance, current knowledge of cognitive capacities and academic achievement; psychomotor development; development of speech, language and communication; behaviour problems; psychiatric comorbidity and possible therapeutic interventions are reviewed.

Eficácia da intervenção cognitivo-comportamental em um caso diagnosticado com a síndrome de moébius / The value of cognitive-behavioral intervention in a moebius syndrome case

Este trabalho teve como objetivo compreender os processos psicológicos decorrentes da Síndrome de Moébius, por meio da avaliação e da intervenção cognitivo-comportamental. Foi sujeito do estudo uma adolescente do sexo feminino, 13 anos e 8 meses de idade, com a queixa de agressividade e inadaptação nas interações familiares e sociais. A terapêutica psicológica foi composta por quatro fases: identificação do problema, análise funcional, seleção dos procedimentos interventivos e reavaliação. As sessões terapêuticas centraram-se na identificação de pensamentos e cognições disfuncionais produtoras de sentimentos e comportamentos mal-adaptativos, seguida da automonitoração e reatribuição das interpretações sobre as pessoas assim como sobre eventos desencadeadores do conflito. As características sintomáticas e os aspectos evolutivos da síndrome foram explorados em níveis da prática clínica, cuja abordagem técnica empregada na compreensão do fenômeno permitiu a reorganização do auto-conceito, reestruturação cognitiva do papel dos familiares e das interações com os seus pares. Desta forma, os procedimentos adotados permitiram a elaboração de recursos pessoais, identificação de valores e capacidades importantes para o relacionamento no contexto e na promoção de comportamentos mais assertivos.(AU)

This study aimed at understanding the psychological processes in Moebius Syndrome by means of cognitive-behavioral evaluation and intervention. The subject of the study was a 13-year-and-8-months-old female adolescent reporting agressiveness and difficult adjustment in social and family interactions. The psychological therapy was divided in four phases: problem identification, functional analysis, selection of the intervention procedures, and re-evaluation. Therapy focused on the identification of dysfunctional thoughts and cognitions that produce maladjusted feelings and behaviors, followed by the self-monitoring and reattribution of interpretations about people and events seen as sources of the conflict. Symptoms characteristics and evolving aspects of the syndrome were explored in the clinical practice whose theoretical approach employed to understand the phenomenon allowed to reorganize the selfconcept, the cognitive reestructuring of family role and peers interactions. Thus, the procedures adopted allowed the elaboration of personal resources, the identification of values and abilities important for the relationship in the context as well as in the promotion of more assertive behaviors.(AU)