PEDIATRICS Vol. 84 No. 3 September 1989, pp. 570-573
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Moebius Sequence and Prenatal Brainstem Ischemia

PAUL GOVAERT MD1, PIET VANHAESEBROUCK MD1, CLAUDINE DE PRAETER MD1, URLIEN FRÄNKEL MD1, and JULES LEROY MD1

1 Department of Pediatrics and Neonatal Medicine, State University of Gent Gent, Belgium

Congenital nonprogressive (bilateral) facial palsy and external ophthalmoplegia are essential clinical features for the diagnosis of Moebius 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 anomaly syndromes.

Submitted on August 15, 1988
Accepted on October 11, 1988


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