HIstory of MöBIus Syndrome
Paul Julius Möbius (1853-1907)
Möbius syndrome was first described by von Graefe in 1880. Paul Möbius then separated the cranial nerve palsies into 6 groups, where the sixth and seventh nerve palsies were grouped into one syndrome (Möbius 1888). Henderson, in 1939, grouped the symptoms of Möbius syndrome into having three main components:
- Facial diplegia with other cranial nerve palsies
- Malfomations, particularly of the limbs
- Mental retardation with an incidence of 10%
Who Gets Möbius?
- Very rare disease
- The incidence is unknown but is estimated to be 1 in 50,000 to 1 in 500,000 in newborns
- Does not affect ethnic groups differently
- Does not seem to have any gender preference
- There may be an increased risk from parent to child, so genetic counseling is recommended
- Said familial risk is no greater than 1 in 50 (about 2%)
Risk Factors
- Exposure during pregnancy to misoprostol or thalidomide seem to increase risk of Möbius syndrome
- Misoprostol is a drug used to induce abortions in Brazil, Argentina, and the United States. Studies have shown that the use of Misoprostol increases risk of getting Möbius syndrome by 30
- In addition, infant exposure to cocaine is considered a risk factor for Möbius
- There is no prenatal test available but genetic counseling is recommended in individuals who have the disorder
- Since incidence of disorder is mostly sporadic, it is believed that there are a variety of environmental and genetic factors at play
Causes
Most cases of Möbius Syndrome do not appear to be genetic but there is evidence of three genetic forms of Möbius Syndrome
- Chromosome 13 indicates Type 1 Möbius Syndrome
- Chromosome 3 indicates Type 2 Möbius Syndrome
- Chromosome 10 indicates Type 3 Möbius Syndrome
References
- Baraitser, M. (1977). Genetics of Mobius Syndrome. Journal of Medical Genetics, 14, 415-417.
- Briegel, W. (2006). Neuropsychiatric findings of Mobius sequence- a review. Clinical Genetics, 70(2), 91-97.
- Kremer, H., Kuyt, L. P., Van den Helm, B., Van Reen, M., Leunissen, J. A.M., Hamel, B. C.J., . . . Padberg, G. W. (1996). Localization of a gene for Möbius syndrome to chromosome 3q by linkage analysis in a Dutch family. Human Molecular Genetics, 5(9), 1367-1371.
- Maccagno, A. (2013, March 16). Mobius Syndrome. Retrieved April 22, 2013, from Mobius Syndrome website: http://flipper.diff.org/app/items/info/5489
- Mobius PJ. Uber angeborene doppelseitige Abducens-Facialis-Lahmung. Munch Med Wochenschr 1888;35:91-4.
- Moebius Syndrome. (2010). Retrieved April 22, 2013, from Moebius Syndrome Global Information Site: http://www.moebiussyndrome.info/research/18-articles/13-mobius-syndrome
- Pastuszak, Anne L; Schüler, Lavinia; Speck-Martins, Carlos E; Coelho, Katia-Edni FA; Cordello, Synthia M; et al. The New England Journal of Medicine338. 26 (Jun 25, 1998): 1881-1885.
- Pollin, T. I. (2010). Moebius Syndrome. In L. J. Fundukian (Ed.), The Gale Encyclopedia of Genetic Disorders (3rd ed., Vol. 2, pp. 88-990). Detroit, MI: Gale Cengage Learning.