Detailed Information on Muenke Syndrome

Muenke Syndrome, also known as FGFR3-relatedA single mutation in the FGFR3 gene causes this
craniosynostosis. Muenke syndrome is an inheritedsyndrome. The protein made by the FGFR3 gene
condition characterized by the early closure ofis a receptor that plays a role in the development
certain bones of the skull (coronal synostosis),and maintenance of bone and brain tissue.
which affects the shape of the head and face.Mutations in this gene result in an abnormal
Many people with this disorder have a prematurereceptor that binds more readily to molecules
fusion of skull bones along the coronal suture, theoutside the cell.
growth line which goes over the head from earThis overactive receptor interferes with normal
to ear. Other parts of the skull may bebone growth, allowing the bones of the head to
malformed as well. These changes can result in anmingle before they should. Most people with this
abnormally shaped head, wide-set eyes, andcondition have normal intellect, but learning
flattened cheekbones. Muenke syndrome occurs indisabilities are common. These signs and
about 1 in 30,000 newborns.symptoms vary between people, and some
Most people with this condition have normalfindings overlap with those seen in other
intellectual, but developmental impediment andcraniosynostosis syndromes. Children with Muenke
learning disabilities are possible. Some have ansyndrome and craniosynostosis are referred to a
enlarged head (macrocephaly). The signs andcraniofacial clinic with experience in pediatrics.
symptoms of Muenke syndrome vary amongAdults with Muenke syndrome should be referred
affected people, and some findings overlap withto a medical geneticist and genetic counselor for
those seen in other craniosynostosis syndromes.assessment and genetic counseling.