Möbius syndrome

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Möbius syndrome (also spelled Moebius) is an extremely rare congenital neurological disorder which is characterized by facial paralysis and the inability to move the eyes from side to side. Most people with Möbius syndrome are born with complete facial paralysis, which means they cannot close their eyes or form facial expression. Limb and chest wall abnormalities sometimes occur with the syndrome. Most people with Möbius 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 Julius Möbius, a neurologist who first described the syndrome in 1888.[1]

Möbius syndrome results from the underdevelopment of the VI and VII cranial nerves.[2] The VI cranial nerve controls lateral eye movement, and the VII 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.[3] Occasionally, the cranial nerves V and VIII are affected.[4] If cranial VIII is affected, the person experiences hearing loss.

It is estimated that there are, on average, 2 to 20 cases of Möbius syndrome per million births.[5][6] 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 Möbius syndrome cannot follow objects by moving their eyes from side to side, they turn their head instead.

Other symptoms that sometimes occur with Möbius syndrome are:

Children with Möbius syndrome may have delayed speech because of paralysis of the lips. However, with speech therapy, most people with Möbius syndrome can develop understandable speech.[7] Möbius syndrome has been linked to increased occurrence of the symptoms of autism.[8] However, some children with Möbius syndrome are mistakenly labeled as mentally retarded or autistic because of their expressionless faces, strabismus, and frequent drooling.

There is no single course of medical treatment or cure for Möbius 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 via tarsorraphy), 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 Möbius syndrome, because it does not improve the ability to form other facial expressions.

Many people with Möbius syndrome lead full lives and experience personal and professional success.[9] Facial expression is important in social interaction, and other people may have difficulty recognizing the emotions of people with Möbius. A person with Möbius syndrome who cannot smile may appear unfriendly or disinterested in a conversation. However, friends and family who are familiar with the person with Möbius syndrome learn to recognize other signals of emotion such as body language, and they sometimes report forgetting that the person has facial paralysis altogether.[10] People with Möbius syndrome can use alternative methods to communicate emotion—such as body language, posture, and vocal tone.[11]

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