1/26. Segmental facial myoclonus in moebius syndrome.Moebius syndrome is characterized by sixth and seventh nerve palsy and is usually the result of bilateral hypoplasia or aplasia of the respective brain stem nuclei. There have been no reports of involuntary facial movements associated with this malformative complex. We report on a 6-year-old boy affected by Moebius syndrome with asymmetric involvement and segmental facial myoclonus with onset at age 2 years, affecting the side with partially conserved motility. Clinical presentation included congenital peripheral palsy of the right seventh cranial nerve and left-sided rhythmic rising of the upper lip and eyebrow. Surface-electromyography (EMG) of the left levator labii and frontalis muscles showed rhythmic bursting (duration: 150-450 ms; frequency: 1-3 Hz). Electroencephalographic (EEG)-polygraphic recordings and burst-locked EEG averaging failed to show any consistent EEG activity preceding the EMG bursts. Study of the blink reflex, somatosensory and motor-evoked potentials showed findings consistent with pontine pathology. Segmental facial myoclonus, although extremely rare in children, must be differentiated from several other paroxysmal motor manifestations associated with structural lesions involving the brain stem. Segmental facial myoclonus stem-Structural lesion.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/26. strabismus surgery in children with mobius syndrome.mobius syndrome is a congenital disorder of facial diplegia associated with lateral gaze paralysis. Although palsy of the sixth and seventh cranial nerves is the minimum diagnostic finding for mobius syndrome, neuropathologic evidence indicates that this is a more complex syndrome.(1) Clinically, it is characterized by a total absence of facial expression and severe esotropia. Other anomalies may be associated with this syndrome, especially other cranial nerve palsies and poland syndrome. The etiology of this syndrome has not been clearly established. brain stem necrosis resulting from a vascular deficiency has been offered as a possible pathogenetic explanation.(2) The strabismus in mobius syndrome is congenital esotropia with bilateral limitation in abduction. Even though many reports have described the various features of mobius syndrome, only a few articles have reported the results of strabismus surgery in children, including bimedial rectus muscle recession. (3-5) Some authors report that bilateral medial rectus muscle recession alone has been disappointing; therefore, a combination of a medial rectus muscle recession and a lateral rectus muscle resection was recommended for satisfactory results. (5-7) In more severe cases, muscle transposition was needed to ensure straight position of the eyes in primary gaze. (8-9)- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/26. magnetic resonance imaging enhancement of cranial nerves in inflammatory bulbar polyneuropathy.A patient with generalized inflammatory polyneuropathy and facial diplegia was studied with magnetic resonance imaging. Multiple cranial nerves showed signal enhancement without sensory or motor dysfunction.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
4/26. MRI findings in mobius syndrome: correlation with clinical features.The authors studied the MRI findings of three patients with mobius syndrome. mobius syndrome is a rare congenital disorder characterized by complete or partial facial diplegia accompanied by other cranial nerve palsies. MRI demonstrated brainstem hypoplasia with straightening of the fourth ventricle floor, indicating an absence of the facial colliculus. These MRI features suggest the diagnosis of mobius syndrome and correlate with the clinical and neurophysiologic findings.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
5/26. Poland-Moebius syndrome: a case report.BACKGROUND: The primary site of pathology in Moebius syndrome is still unknown, although several studies have variably localized the lesion in the extraocular muscles, cranial nerves, or central nervous system. CASE: A 24-year-old man with Poland-Moebius syndrome and acquired progressive bilateral paralytic lower eyelid ectropion is described. OBSERVATIONS: In this patient, magnetic resonance imaging studies revealed a barely detectable pontine hypoplasia and normal recti muscles. Nerve conduction studies of the facial nerves showed a severe demyelinating or dysmyelinating type of neuropathy. Bilateral lower eyelid ectropium of the patient was successfully corrected by canthal tightening procedures. CONCLUSION: Contrary to many reported cases, this patient serves as a rare example of a progressive type of Poland-Moebius syndrome presumably resulting from a combination of a brainstem abnormality and a peripheral neural degenerative process.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/26. Expanded mobius syndrome.A woman presented at 33 weeks gestation with reduced fetal movements and a nonreactive nonstress test. Fetal ultrasound examination revealed a peculiar unilateral arm tremor. At emergency cesarean section, performed for fetal indications, a 1,672-gm male infant was delivered requiring intubation for feeble respiratory effort. After delivery the neonate was transiently hypertonic and later hypotonic. Continuing ventilatory support at minimal settings was necessary. The work-up for aneuploidy, metabolic disorders, and infection was negative. The infant died after being removed from ventilatory support on day 22. Postmortem examination revealed extensive bilateral brain gliosis and mineralization without evidence of inflammation, partial absence of cranial nerve nuclei III-XI, and a total absence of cranial nerve roots VI-XI. Together these finding are compatible with a diagnosis of expanded mobius syndrome.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/26. mobius syndrome: a case report.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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/26. A case of Moebius syndrome in association with klinefelter syndrome.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.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
9/26. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/26. 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.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
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