Self-perception of children and adolescents with Mobius sequence

Mobius 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 Mobius 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 fur Kinder und Jugendliche [DIKJ]), anxiety (Angstfragebogen fur Schuler [AFS]) and personality aspects (Personlichkeitsfragebogen fur 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 Mobius 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. (C) 2011 Elsevier Ltd. All rights reserved.