Möbius syndrome
| Classification according to ICD-10 | |
|---|---|
| Q87.0 | Congenital malformation syndromes with predominant involvement of the face |
| ICD-10 online (WHO version 2019) | |
When Moebius syndrome ( synonym: okulofaziale paresis ) is a rare, congenital syndrome that occurs sporadically and is primarily by bilateral paralysis of the facial facial muscles ( facial paralysis manifested). The cause is suspected to be a nuclear aplasia in the brain stem . The movements are limited to such an extent that, for example, smiling or frowning can only be difficult or even impossible and the face takes on a mask-like expression. Combinations with disorders of the eye muscles , in particular a palsy of the abdomen , as well as paralysis that can be traced back to lesions below (infranuclear) or above ( supranuclear ) the nerve nucleus, for example eye paralysis , are common.
In some cases Möbius syndrome is associated with other physical impairments, such as malformations of the fingers and hands. Another, albeit rare, association has been associated with ear malformations, hearing loss, and deafness. Combinations with other syndromes, for example Kallmann or Poland syndrome , are possible. Studies also show that just under a third of children with Möbius syndrome have an autism spectrum disorder .
A life without facial expressions, and especially facial laughter, repeatedly meets with rejection in the social environment and is associated with specific exclusion. Every phase of socialization presents itself as a great challenge. The disease was first described in 1888 by the German neurologist Paul Julius Möbius (1853–1907).
literature
- Herbert Kaufmann (Ed.): Strabismus. With the collaboration of Wilfried de Decker et al. Enke, Stuttgart 1986, ISBN 3-432-95391-7 .
Web links
- Moebius Syndrome , entry at the self-help association Moebius Syndrome Germany eV
Individual evidence
- ↑ CJ Partsch, E. Schmidt-Wittkamp: hearing defects in Moebius syndrome. In: Archives for ear, nose and larynx medicine. Vol. 184, No. 6, November 30, 1965, ISSN 0365-5245 , pp. 536-549, doi : 10.1007 / BF01972523 .
