The Japanese Journal of Pediatric Dentistry
Online ISSN : 2186-5078
Print ISSN : 0583-1199
ISSN-L : 0583-1199
Möbius Syndrome: Report of a Case
Fumihiko NishidaMizuho Nishino
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JOURNAL FREE ACCESS

1987 Volume 25 Issue 1 Pages 193-198

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Abstract

Möbius syndrome is an extremely rare neurological disorder and its salient features are facial and abducens palsy, usually bilateral. Other cranial neuropathies, hearing loss, ossicular adnormalities, malformed pinnea, micrognathia, and limb defects, have been associated features in 1% to 15% of the cases in the previous reports.
A case of a two-year-old boy with Mobius syndrome, mild-moderate mental retardation is described in this report.
The patient was born after 40 weeks. The birth weight was 2774 g. The family history was negative with respect to any congenital deformity. During the first 8 months of life the boy suffered from severe sucking difficulties. He had facial and abducens palsy and also had a left-sided malformed pinnea. The feeding difficulties remained, but gradually became somewhat less severe. He did not show any contact smile and his gaze was very “stiff”. He had speech, language and hearing problems. He could not walk without support.
All his erupted deciduous teeth were attacked by severe dental caries. The upper and lower deciduous second molars were unerupted. From the panoramic roentgenogram, congenital missing of lower left and right lateral incisors was doubtfull (not seen). Furrowed tongue was observed.

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