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The natural history of Möbius syndrome in a 32-year-old man
Affiliations- 1 Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland
- Department of Reproduction and Gynaecological Endocrinology, Medical University of Bialystok, Poland
- Podlaskie Centre of Clinical Genetics in Bialystok, Poland
- Maxillofacial and Plastic Surgery Department, Medical University of Bialystok, Poland
- Karedent, Dental Clinic in Bialystok, Poland
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Abstract
Möbius syndrome (OMIM#157900) is an extremely rare congenital entity involving bilateral or unilateral palsy of the facial nerve, usually with dysfunction of other cranial nerves (second, third, fifth, sixth, ninth, tenth and twelfth). It was estimated that Möbius syndrome occurs in 1 of 50 000 live births. The aetiology and the pathogenesis of the syndrome remain unknown. The majority of published cases were sporadic.
We report on the natural history of a 32-year-old man with de novo Möbius syndrome. The diagnosis was established at the age of 9 months due to partial bilateral facial and abducent nerve palsy. Additionally, the patient demonstrated failure to thrive during infancy and childhood, many dysmorphic features, lower limb anomalies, and hypogonadism in adulthood, but his intelligence was in the normal range. The low quality of life of the patient with Möbius syndrome is emphasized.
Abstract
Möbius syndrome (OMIM#157900) is an extremely rare congenital entity involving bilateral or unilateral palsy of the facial nerve, usually with dysfunction of other cranial nerves (second, third, fifth, sixth, ninth, tenth and twelfth). It was estimated that Möbius syndrome occurs in 1 of 50 000 live births. The aetiology and the pathogenesis of the syndrome remain unknown. The majority of published cases were sporadic.
We report on the natural history of a 32-year-old man with de novo Möbius syndrome. The diagnosis was established at the age of 9 months due to partial bilateral facial and abducent nerve palsy. Additionally, the patient demonstrated failure to thrive during infancy and childhood, many dysmorphic features, lower limb anomalies, and hypogonadism in adulthood, but his intelligence was in the normal range. The low quality of life of the patient with Möbius syndrome is emphasized.
Keywords
Möbius syndrome, facial nerve palsy, cogenital disorder, bilateral/unilateral involvement
About this articleTitle
The natural history of Möbius syndrome in a 32-year-old man
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
74-79
Page views
336
Article views/downloads
683
DOI
10.1016/S0028-3843(14)60063-3
Bibliographic record
Neurol Neurochir Pol 2011;45(1):74-79.
Keywords
Möbius syndrome
facial nerve palsy
cogenital disorder
bilateral/unilateral involvement
Authors
Monika Chorąży
Ryszard Leśniewicz
Renata Posmyk
Dorota Halicka
Aneta Zalewska
Anna Wincewicz-Pietrzykowska
Kamil Chorąży
Wiesław Drozdowski
