What is Moebius Syndrome?
What is Moebius Syndrome?
Moebius Syndrome is an extremely rare congenital neurological disorder which is characterized by facial paralysis and the inability tomove the eyes from side to side. Most people with Moebius syndrome are born with facial paralysis, which means they cannot close their eyes or form facial expressions. Limb and chest wall abnormalities sometimes occur with the syndrome. Most people with Moebius syndrome have normal intelligence, and others should take care not to confuse their lack of facial expression with dullness or unfriendliness. It is named for Paul Jullius Mobius, a neurologist who first described the syndrome in 1888.
Moebius syndrome results from the underdevelopment of the sixth and seventh cranial nerve. The sixth cranial nerve controls lateral eye movement, and the seventh cranial nerve controls facial expression. People with Möbius syndrome are born with facial paralysis and the inability to move their eyes laterally. Often, the upper lip is retracted due to muscle shrinkage. Occasionally, the cranial nerves five and eight are affected. If cranial eight is affected, the person experiences hearing loss.
It is estimated that there are, on average, 2 to 20 cases of Moebius syndrome per million births. Although its rarity often leads to late diagnosis, infants with this disorder can be identified at birth by a “mask-like” lack of expression that is detectable during crying or laughing and by an inability to suck while nursing because of paresis (palsy) of the sixth and seventh cranial nerves. Also, because a person with Moebius syndrome cannot follow objects by moving their eyes from side to side, they turn their head instead.
Other symptoms that sometimes occur with Moebius syndrome are:
Limb abnormalities—clubbed feet, missing fingers or toes
Difficulty in breathing and/or in swallowing
Corneal erosion resulting from difficulty in blinking
Children with Moebius syndrome may have delayed speech because of paralysis of the lips. However, with speech therapy, most people with Moebius syndrome can develop understandable speech. Möbius syndrome has been linked to increased occurrence of the symptoms of autism. However, some children with Moebius syndrome are mistakenly labeled as mentally retarded or autistic because of their expressionless faces, and frequent drooling.
There is no single course of medical treatment or cure for Moebius syndrome. Treatment is supportive and in accordance with symptoms. If they have difficulty nursing, infants may require feeding tubes or special bottles to maintain sufficient nutrition. Physical, occupational, and speech therapy can improve motor skills and coordination and can lead to better control of speaking and eating abilities. Often, frequent lubrication with eye drops is sufficient to combat dry eye that results from impaired blinking. Surgery can correct crossed eyes, protect the cornea, and improve limb and jaw deformities. Sometimes called “smile surgery” by the media, muscle transfers grafted from the thigh to the corners of the mouth can be performed to provide the ability to smile. Although “smile surgery” may provide the ability to smile, the procedure is complex and can take twelve hours for each side of the face. Also, the surgery cannot be considered a “cure” for Moebius syndrome, because it does not improve the ability to form other facial expressions.
Living with Moebius syndrome:
Many people with Moebius syndrome lead full lives and experience personal and professional success. Facial expression is important in social interaction, and other people may have difficulty recognizing the emotions of people with Moebius. A person with Moebius syndrome who cannot smile may appear unfriendly or disinterested in a conversation. However, friends and family who are familiar with the person with Moebius syndrome learn to recognize other signals of emotion such as body language, and they sometimes report forgetting that the person has facial paralysis altogether. People with Moebius syndrome can use alternative methods to communicate emotion—such as body language, posture, and vocal tone.