open access

Vol 45, No 1 (2011)
OPIS PRZYPADKU
Submitted: 2010-06-21
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The natural history of Möbius syndrome in a 32-year-old man

Monika Chorąży, Ryszard Leśniewicz, Renata Posmyk, Dorota Halicka, Aneta Zalewska, Anna Wincewicz-Pietrzykowska, Kamil Chorąży, Wiesław Drozdowski
DOI: 10.1016/S0028-3843(14)60063-3
·
Neurol Neurochir Pol 2011;45(1):74-79.

open access

Vol 45, No 1 (2011)
OPIS PRZYPADKU
Submitted: 2010-06-21

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.

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Keywords

Möbius syndrome, facial nerve palsy, cogenital disorder, bilateral/unilateral involvement

About this article
Title

The natural history of Möbius syndrome in a 32-year-old man

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 45, No 1 (2011)

Pages

74-79

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

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