Mobius syndrome is caused by abnormal development of the cranial nerves. This rare disorder has a number of causes. Most often affected are the cranial nerves VI and VII. Occasionally the cranial nerves V and VIII are affected.

If the cranial nerve VI is affected, the patient suffers from loss of lateral gaze. If cranial nerve VII is affected, the patient suffers from bilateral facial palsy — mask-like expressionless face with mouth constantly held open. If cranial VIII is affected the patient suffers from hearing loss.

Although its rarity often leads to late diagnosis, Infants with this disorder can be identified at birth: by a "mask-like" expression detectable during crying or laughing due to paralysis (palsy) of the sixth and seventh cranial nerves. Other characteristics include:

abnormalities in the limbs — their fingers may be webbed, shorter than usual or they may have more than 5 fingers on their hand
impaired sucking ability
inability to follow objects with the eye—instead the child turns his or her head to follow
crossed eyes
inability to smile
limitation of tongue movement

Later on, the child may develop speech difficulties, crossed eyes, abnormally small eyes, and fluid building up in the lungs, causing bronchopneumonia.

Treatment

There is no specific course of treatment for Mobius syndrome. Treatment is supportive and in accordance with symptoms. Infants may require feeding tubes or special bottles, such as the Haberman Feeder, 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.

Pathological picture

The causes of Mobius syndrome are poorly understood. Many cases have no obvious cause. Others may be genetic.

Some cases are associated with reciprocal translocation between chromosomes or maternal illness. Some maternal trauma may result in impaired or interrupted blood flow (Ischemia) or lack of oxygen (Hypoxia) to a developing fetus. The use of drugs and a traumatic pregnancy may also be linked to the development of Mobius syndrome. The use of the drugs Misoprostol or Thalidomide by women during pregnancy has been linked to the development of Mobius syndrome in some cases.

Some researchers have suggested that the underlying problem of this disorder could be congenital hypoplasia or agenesis of the cranial nerve nuclei. Certain symptoms associated with Mobius syndrome may be caused by incomplete development of facial nerves, other cranial nerves, and other parts of the central nervous system.

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PREVIEW: TV CHOICE - The new face of expressionism
From Independent, The (London), 3/4/01 by CHARLOTTE EDWARDS

And now for something completely different. John Cleese's comic turns have never depended on the rubber-faced mobility that made clowns out of Rowan Atkinson and Kenneth Williams. So he might seem a rather surprising choice to front BBC1's new four-part series on the myriad wonders of The Human Face. Cleese must think so, too, as he's hired Liz Hurley - who's not exactly celebrated for her repertoire of expressions - to pull the faces, while he impassively delivers what Jennifer Aniston would call "the science bit".

The silly skits that tie up this first episode - about the importance of our expressions to our emotional and social wellbeing - are worthwhile only for the moment of schadenfreude when Cleese's professor takes off lovely assistant Hurley's mug, revealing the 44 facial muscles. What's interesting are the case studies - the stories of people who find themselves, for a variety of reasons, unable to use or understand the signals that the rest of us send out without a second thought.

Five-year-old Lauren suffers from Mobius Syndrome. A rare condition affecting the nerve that governs facial expressions, it means that she is unable to move her face and eyes. As she prepares to start school, the significance of her lacking these basic tools of communication is beginning to hit home. "I'm worried that people will perceive her as retarded, stupid, not interested or paying attention," says her mother. "You just don't realise how much your face expresses your feelings."

But our facial expressions are only communicative if they're directed at someone who can read them. Cambridge student Chris suffers from Asperger's Syndrome, a type of autism that affects his ability to recognise other people's emotions. There are several expressions that should cause a frenetic response in the part of the brain called the amygdala, allowing us instantly to register fear, happiness, anger or surprise. Chris, however, has sustained damage to his amygdala, and finds it almost impossible to understand facial expressions. He is teaching himself to "read" faces by breaking them down. "You're angry or frightened," he says, considering Cleese's test expression. "I can tell, because the corners of your mouth are turned down."

It's quite a challenge - those of us with fully functioning amygdalas get it wrong from time to time. Even the experts - police, judges, polygraph operators - find it hard to decide when an expression is genuine, and only the secret service (any excuse for Hurley to don her Austin Powers leather suit) excel at recognising the face of a liar. In a test, 80 per cent of agents spotted the "micro-expressions" - nervous tics, furrows and twitches lasting a fraction of a second - that showed the subject wasn't telling the truth. Look again at the footage of Bill Clinton, and watch for the telltale sign that his "I did not have sexual relations with that woman" was the tiniest fib.

"The Human Face" starts on Wednesday at 9.10pm on BBC1.

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