Möbius Syndrome

Reports from the MHC data base

Report from the Questionnaire (PDF)
Report from the Observation Chart (PDF)

Synonyms

Möbius Sequence

Codes:

ICD-10: Q87.0W
ORPHA: 570

Estimated occurrence

Approximately 50 individuals with Möbius syndrome have been diagnosed in Sweden to date.

Etiology

Most cases are sporadic without a known etiology. The diagnosis is probably caused by an early fetal damage affecting the development of the cranial nerves.

General symptoms

The primary symptoms are congenital facial palsy (affecting the facial muscles) and abducens palsy (affecting the possibility of lateral eye movements). These palsies usually occur on both sides. Other cranial nerves may be affected, generally the hypoglossal nerve (controls tongue mobility), the glossopharyngeal/vagus nerves (important for swallowing and speech) and/or the trigeminal nerve (controls the muscles involved in chewing and the sensation of the face and mouth). Skeletal anomalies may occur. Some individuals with Möbius syndrome have a neuropsychiatric disorder and intellectual impairment.

Orofacial/odontological symptoms

Facial palsy results in little or no facial expression. Many of these children have sucking, eating and speech difficulties owing to underdevelopment of the cranial nerves and craniofacial deformities including cleft palate and/or microglossia (underdeveloped tongue). There may be drooling problems. A post normal bite a small lower jaw and crowded teeth are all relatively common, as is the absence of some tooth buds.

Orofacial/odontological treatment

  • Early contact with dental services for intensified prophylactic care and oral hygiene information is essential.
  • In cases of craniofacial deformities, a specialist team will be responsible for follow up and treatment.
  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • Orofacial therapy and oral motor skill training should be considered.
  • Speech, language and communication training are often justified.
  • Feeding and swallowing difficulties are investigated and treated by a specialist team at the hospital or multidisciplinary treatment center.

Sources

  • The rare disease database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-13 08:11