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Congenital nonprogressive(bilateral)facial palsy and external ophthalmoplegia are essential clinical features for the diagnosis of Moebium syndrome.One century after its initial thorough description,the precise delineation and pathogenesis of the syndrome still remain obscure. Heuristic hypotheses concerning the latter have been put forward.Three major theories of etiology remain valid:primary brainstem nuclear hypoplasia,secondary brainstem nuclear degeneration,and brainstem atrophy secondary to muscular defect.None of these theories easily explains why, apart from cranial nerve dysfunction,the Moebius syndrome is frequently a part of oral-limb deficiency anomally syndromes.In this article,in vivo support is given to a theory recently formulated in which a disruption sequence in the vascular territory of the subclavian artery might explain the Moebius sequence,among others. |
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abducens nerve paralysis
abducens nerve paralysis,bilateral
brainstem,ischemia
brainstem,lesion of
calcification,intracranial
CAT scan
facial nerve palsy
facial nerve palsy,bilateral
facial weakness
facial weakness,bilateral
gaze palsy,horizontal
gaze palsy,horizontal-bilateral
Moebius syndrome
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