Moebius Syndrome is a rare neurological disorder present at birth, which is characterized by facial paralysis and the inability to move the eyes from side to side, they can’t form facial expressions or blink their eyes. Children and adults with facial paralysis may be unable to smile, frown, raise their eyebrows, close their eyelids or pucker their lips. This not only results in lack of facial expression but may also result in drooling and difficulty with speech. Infants can have difficulty with sucking and swallowing. Other cranial nerves may be affected, especially the 3rd, 4th, 5th, 9th, 10th and 12th. There may be skeletal involvement causing hand/feet anomalies and/or club feet.
Social and Lifestyle Effects
Moebius syndrome does not prevent individuals from experiencing personal and professional success. Due to the importance of facial expression and smiling in social interaction, the inability to form either can lead to individuals with Möbius being perceived as unhappy, unfriendly or uninterested in conversations. Individuals who are familiar with Möbius patients such as family or friends can recognize other signals of emotion such as body language, to the point that they sometimes report forgetting that the person has facial paralysis. Indeed, people with Moebius syndrome are often adept at compensating for a lack of expression by using body language, posture, and vocal tone to convey emotion.
Causes
The cause of Moebius syndrome is unknown. Research is ongoing to try and establish environmental and/or genetic factors that may be connected to incidences of Moebius syndrome. What is clear is that the development of the cranial nerves VI and VII is somehow disrupted as the embryo grows in the womb. In more severe cases, additional cranial nerves are involved. A small percentage of cases of Moebius syndrome have been reported to run in families but there is no single clear pattern of inheritance and the majority of cases of Moebius syndrome are sporadic.
There may well be sub-groups of Moebius syndrome and these may have different causes, but this still remains a subject of research.
Symptoms
- Lack of facial expression; inability to smile
- Feeding, swallowing and choking problems
- Keeping head back to swallow
- Eye sensitivity due to inability to squint
- Motor delays due to upper body weakness
- Absence of lateral eye movement
- Absence of blinking
- Strabismus (crossed eyes)
- Drooling
- High palate
- Short or deformed tongue
- Limited movement of tongue
- Submucous cleft palate
- Dental problems
- Hearing impairment
- Articulation / speech disorders
- Minor mid-line anomalies
- Club feet
- Hand/feet deformities
Although they may crawl and walk later, most children with Moebius Syndrome eventually catch up. Speech problems often respond to therapy, but may persist due to impaired mobility of the tongue and/or mouth. As children get older, the lack of facial expression and an inability to smile may become the dominant visible symptoms. Moebius Syndrome is sometimes but usually infrequently, accompanied by Pierre Robin Syndrome and Poland’s Anomaly.
Diagnosis
There is currently no test for Moebius syndrome and diagnosis is based on assessment of the physical symptoms and taking into account the medical history of the individual. Further examinations such as CAT scan or MRI of the brain and brainstem may be carried out in order to confirm the diagnosis. The first indication that an infant has Moebius syndrome may be a mask-like expression when crying.
Treatment
There is no specific course of treatment for Moebius syndrome. Treatment is supportive and in accordance with symptoms. Infants may require feeding tubes or special bottles to maintain sufficient nutrition. Surgery may correct crossed eyes and improve limb and jaw deformities. Physical and speech therapy often improves motor skills and coordination, and leads to better control of speaking and eating abilities. Plastic reconstructive surgery may be beneficial in some individuals. Nerve and muscle transfers to the corners of the mouth have been performed to provide limited ability to smile.
