What is the Moebius Syndrome?

The Moebius Syndrome is a rare disorder of which the main factor is the permanent paralysis of the face caused by a reduced or missing formation of the cranial nerves VI and VII. People affected by Moebius Syndrome can't smile, make expressions, and often can't blink or move their eyes side to side. The third, fifth, eighth, ninth, eleventh and the twelth cranial nerves can also present problems.

In some cases people affected by Moebius Syndrome have serious physical problems in various parts of their body. Sometimes the "Pierre Robin Syndrome" and "Poland Syndrome" are associated with the Moebius Syndrome.

What are the symptoms ?

How does Moebius Syndrome occur ?

How is Moebius Syndrome treated ?

To what extent is Moebius Syndrome known in Italy ?

Description of the twelve cranial nerves.

What are the Symptoms ?

The dominant aspect of Moebius Syndrome is the lack of facial expression. In newborn infants the first sign is an impaired ability to suck. Also due to the face muscles that can't move, many patients have problems of drooling as they are neither able to close their mouth well. Cases of strabismus are also frequent and people with deformations of the tongue, jaw, hands and feet (club feet). Many children have low muscle tone, especially in the upper body.

The general symptoms/aspects can be finally resumed as follows:

• Lack of facial expression, inability to smile and make face movements (frowns);
• Feeding, swallowing and choking problems (sometimes special tubes are necessary to feed patients; special care must be given to solid food);
• eye sensitivity due to inability to squint (sun glasses and hats are usually recommended);
• Absence of the lateral eye movement;
• A late general motory development caused by low muscle tone in the upper body (generally children affected by Moebius Syndrome start crawling at a later stage);
• Strabismus (that can be corrected by surgery);
• Drooling;
• High palate, submucous cleft palate;
• Short or deformed tongue, limited tongue movements;
• Teeth problems;
• Hearing problems (due to fluid in the ears, sometimes it's necessary to use special tubes);
• Difficulties to speak (especially with labial letters,    m, p, b).

How does Moebius Syndrome occur

It's present at  birth. The reason why it occurs seems to be of a genetic nature even if it's not certain, infact medical documentation present different theories. The Moebius Syndrome can affect both males and females in the same way, and it seems that there is a major risk, in some cases, of affected parents transmitting the illness to their children. In spite of the fact that no prenatal test is currently available to locate the possibility of a  Moebius Syndrome, genetic analysis in some cases may give useful indications to eventually diagnose the illness.

How is Moebius Syndrome treated?

When babies have problems to suck, they can sometimes be feeded by special feeding bottles or special tubes that guarantee a sufficient nutrition. Strabismus can usually be corrected by surgery. In many cases, a motory improvement of the muscles can be achieved by specific physical therapies. A good level of pronunciation can generally be achieved by speech therapy.

With Doctor Steve Clarke's Treatment Body-brushing is possible to improve coordination and concentration.

"Oral-motor therapy"

with the purpose to improve the muscle motory, especially the face. The Italian Association has already organized some training course with speech pathologists; so this therapy is also performed in Italy.

Deformations of limbs and jaws are very often corrected by surgery, and " Smile-surgery" allows  to gain moving some face muscles among those which allow to smile. Since June 2003 this kind of surgery is performed by the Maxillo-facial staff in Parma Hospital.

To what extent is Moebius Syndrome known in Italy?

The Moebius Syndrome is not very well known in Italy. Due to the lack of information of this disorder, it's very often diagnosed only after some months/years after birth.

The rarity of the Moebius Syndrome therefore becomes another complication besides the physical problems of the disorder itself. The lack of information causes individuals affected by the syndrome and their families to feel insecure, as well as discouragement and distress. Lack of information also limits diagnosis and research which means that people are not informed of the possible treatments and therapies existing worldwide.

Description the twelve cranial nerves:

I  =  Olfactory nerve - relays smell

II = Optic nerve - transmits visual information

III= Oculomotor nerve- supplies external muscles of the eyeball with   motor and sensory fibres

IV = Trochlear nerve - also supplies external muscles of the eyeball with motor and sensory fibres

V= Trigeminal nerve - supplies muscles concerned with chewing and relays sensations from the face

VI= Abducent nerve - concerned with lateral eye movement and blinking

VII= Facial nerve - controls the muscles of facial expression, serves the taste buds on the front two-thirds of the tongue

VIII= Auditory nerve - concerned with hearing and balance

IX = Glossopharyngeal nerve - carries sensation and taste from the back of the tongue and throat; helps control swallowing

X = Vagus nerve - carries both sensory and motor connections to many organs in the chest and abdomen

XI = Accessory nerve - supplies two neck muscles, the sternomastoid and the trapezius

XII = Hypoglossal nerve - supplies the muscles of the tongue and some of the small muscles of the neck.

 

"IT'S ALWAYS WORTHWHILE TO LIVE A LIFE WITHOUTH A SMILE "