| mobius syndrome is characterized by showing unilateral facial nerve palsy of the sixth and seventh nerves, lack of facial expression, inability to smile and to tightly close the right eyelids. In this report, a 7-year-old-boy with mobius syndrome is presented. He had asymmetry of facial expression, anomalies of fingers and severe tooth decay. After dental treatment, the periodic re-care visits should be done according to the eruption pattern. ( info) |
12/40. subclavian artery supply disruption sequence-Klippel-Feil and Mobius anomalies. Fetal vascular disruptions can cause specific patterns of birth depending on the location, extent and timing of the disruptive event in the embryonic life. An example of this is subclavian artery supply disruption sequence occurring around 6 weeks of gestation which causes various combinations of poland, Klippel-Feil and Mobius anomalies. A one-month-old child with features of all three anomalies along with other associated features is described here. ( info) |
| A classic mobius syndrome, including bilateral abducens-facial paralysis, was found in a newborn boy. At least 15 maternal relatives showed partial features of the syndrome. As potential features of mobius syndrome also were found in the father's side of the family, a recessive mode of inheritance is suggested. ( info) |
| PURPOSE: To describe an infant affected by klinefelter syndrome, who also demonstrated clinical signs of Moebius syndrome. methods: A clinical case report. RESULTS: A male infant was born full-term to a healthy couple after an unremarkable pregnancy. Several dysmorphic features and generalized hypotonia were noted at birth. Chromosome study revealed a 47, XXY chromosome pattern, which is consistent with klinefelter syndrome. The patient also demonstrated clinical findings of Moebius syndrome: bilateral horizontal gaze palsy, bilateral cranial nerve seven palsy, pointed tongue, pectoral muscle hypoplasia, and clubfeet. CONCLUSION: We report the first clinical case of a patient with klinefelter syndrome who was also affected by Moebius syndrome. Although clinically intriguing, coexistence of the two syndromes most likely represents a chance occurrence. ( info) |
15/40. Mobius and Mobius-like patients: etiology, diagnosis, and treatment options. The surgical goal in Mobius patients is far more modest and differs from patients with unilateral developmental facial paralysis. It is impossible to restore a true smile in these mask-like, expressionless faces. Despite sophisticated microneurovascular transplantations, movement can only be restored along one vector and enhanced firmness in the cheeks, thus multiple differentiated facial animation is not achievable. A detailed neurological evaluation can identify possible motor donors or residual function, which can be used for additional dynamic restorations. Due to the multiple cranial nerve involvement a thorough clinical and electrophysiological examination is mandatory. In addition, electromyographic survey of the potential motor donors is very helpful to avoid weak wasted regeneration and prevent further downgrading of function. Because of the variety of cranial nerves involved in Mobius' syndrome, a standard procedure for dynamic restoration cannot and should not be promoted; instead, a careful preoperative objective and quantitative assessment should guide the reconstructive surgeon to the optimal reconstruction strategy. Useful movement can be restored in afflicted patients that may signal physical and psychological rehabilitation. ( info) |
16/40. Dynamic restoration in Mobius and Mobius-like patients. mobius syndrome is classically characterized by bilateral facial nerve and abducens nerve paralysis in combination with limb defects. In the past 110 years, physicians diagnosed children as having the syndrome on the basis of heterogeneity of symptoms and used the term "mobius syndrome" or "Mobius-like syndrome" for patients with multiple cranial nerve involvement. The cause and the exact pathogenesis of the syndrome still elude understanding. Genetic work-ups, radiological findings, and data from autopsies differ in their approaches and their findings of the basic causes of mobius syndrome. In the international literature, about 301 case reports are found scattered through the past century. The appearance of the facial deformity is easy to recognize, because the Mobius patient is impaired in his or her ability to communicate nonverbally. Despite ophthalmologic problems, it is the search for a smile that brings these patients to the reconstructive surgeon. Over the past 100 years, surgical efforts attempted to improve the mask-like appearance by static and dynamic procedures, usually local muscle transpositions. Today, combinations of microsurgical procedures and aesthetic techniques are being used to restore some movement to the expressionless face of these patients by nerve and muscle transplantation. This article discusses the heterogeneity of mobius syndrome, advocates a new classification system, presents the clinical findings of 42 patients who were seen and examined in consultation, and discusses the surgical management of 20 patients who underwent dynamic restorative microsurgery. Exemplary cases illustrating the preoperative work-up regimen and possible outcomes are reported. ( info) |
17/40. Athabascan brainstem dysgenesis syndrome. We report a new disorder with diverse neurological problems resulting from abnormal brainstem function. Consistent features of this disorder, which we propose should be called the Atabascan brainstem dysgenesis syndrome, include horizontal gaze palsy, sensorineural deafness, central hypoventilation, and developmental delay. Other features seen in some patients include swallowing dysfunction, vocal cord paralysis, facial paresis, seizures, and cardiac outflow tract anomalies. All affected children described are of Athabascan Indian heritage, with eight children from the Navajo tribe and two patients who are of apache background. The disorder can be distinguished from the Moebius syndrome by the pattern of central nervous system findings, especially the sensorineural deafness, horizontal gaze palsy, and central hypoventilation. Recognition of children with some features of Athabascan brainstem dysgenesis syndrome should prompt investigation for other related abnormalities. Published 2003 Wiley-Liss, Inc. ( info) |
18/40. Moebius sequence and hypogonadotrophic hypogonadism. A distinct form of Moebius sequence is associated with hypogonadotrophic hypogonadism. There have been five such cases to date. We now add a further case with detailed neurologic, endocrine, and autopsy findings and offer a hypothesis drawing parallels with the already established basis of hypogonadotrophic hypogonadism in the X-linked form of Kallman syndrome. ( info) |
19/40. Twenty-year behavioral follow-up of a 1;13 chromosomal translocation and mobius syndrome presenting with poor impulse control, exhibitionism, and aggression. mobius syndrome is a rare disorder characterized by agenesis or aplasia of the facial or abducens nerve motor nuclei and other features including central nervous system and behavioral abnormalities. We report an adult case of mobius syndrome variant presenting with poor impulse control, aggression, and exhibitionism. karyotyping revealed a 46 XY, t(1;13)(p34.3;q12.3) translocation. The subject had been presented 20 years previously as the index case of a three-generation pedigree. ( info) |
20/40. Management of a patient with mobius syndrome: a case report. mobius syndrome is a rare congenital disorder with the primary diagnostic criteria of congenital facial and abducent nerve palsy. Orofacial anomalies and limb malformations may be associated with the disorder. Involvement of other cranial nerves also is common. Occasionally, the V, X, XI, and XII cranial nerves are involved, resulting in difficulty of chewing, swallowing, and coughing, which often leads to respiratory complications. Mental retardation and autism have been reported in some cases. An 18-year-old Hispanic male came to the general practice Residency clinic at the University of illinois at chicago for dental treatment. The patient had a history of mobius syndrome, mental retardation, and behavioral problems. Because of acute situational anxiety and violent behavior, we arranged for the patient to have general anesthesia while we provided complete oral rehabilitation. This article discusses the treatment of a patient, including special considerations taken during anesthesia and dental management. A review of the special challenges concerning patients with such a condition are reviewed. ( info) |