1990
DOI: 10.1136/jmg.27.2.122
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Moebius syndrome.

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Cited by 154 publications
(123 citation statements)
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“…157900), or better Moebius sequence, is a non progressive congenital condition with an estimated prevalence of 0.0002-0.002% of births [14,27]. Etiology and pathogenesis are still not fully understood [6,15]. The most widely accepted hypothesis, supported by findings of animal experiments [16,20] and computed tomography/ magnetic resonance studies [9,13], is that of a disruption of the primitive subclavian arteries and their branches before establishment of a sufficient blood supply to the brain stem by the vertebral arteries [2,23].…”
Section: Introductionmentioning
confidence: 99%
“…157900), or better Moebius sequence, is a non progressive congenital condition with an estimated prevalence of 0.0002-0.002% of births [14,27]. Etiology and pathogenesis are still not fully understood [6,15]. The most widely accepted hypothesis, supported by findings of animal experiments [16,20] and computed tomography/ magnetic resonance studies [9,13], is that of a disruption of the primitive subclavian arteries and their branches before establishment of a sufficient blood supply to the brain stem by the vertebral arteries [2,23].…”
Section: Introductionmentioning
confidence: 99%
“…The loss of normal function of anterior horn cells leads to akinesis of the fetus, resulting in contractures of the involved joints (Hall 1996). Clubfoot is seen in one-third of Moebius syndrome patients (Henderson 1938;Baraitser 1977;Kumar 1990), and this is also assumed to be associated with vascular defects (Hootnick et al 1982). Although it is not known in the present case whether arthrogryposis and clubfoot had been caused by direct disturbance of the blood supply to the anterior horn cells or by anoxic injury secondary to the general hypoxia, it is assumed that some hypoxic episodes occurred affecting these cells governing limb joints.…”
Section: Discussionmentioning
confidence: 99%
“…Moebius syndrome is classified as a subclavian artery supply disruption sequence, and is characterized by congenital facial nerve palsy and malformations of limbs and the orofacial area (Kumar 1990). It has been suggested that the involvement of the brain is either caused by a defect of the brain stem cranial nerve nuclei due to disruption of blood supply from the primary trigeminal nerve artery, or is associated with an obstruction or hypoplasia of the basilar and vertebral arteries and their branches at around week 6 of gestation (Bavinck and Weaver 1986).…”
Section: Introductionmentioning
confidence: 99%
“…Existem alguns casos familiares e outros com mutações cariotípicas (5) . A maioria, entretanto, é de ocorrência esporádica (1,(5)(6) . Vários fatores ambientais têm sido implicados na gênese da sequência de Möbius, incluindo infecções, hipertermia, vasculite, coleta de vilosidade coriônica, trauma abdominal, falha na tentativa de aborto, uso de drogas tais como: misoprostol; talidomida; álcool ou cocaína (2,(4)(5) .…”
Section: Resumo Introduçãounclassified