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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Television Review
From Independent, The (London), 3/8/01 by Robert Hanks

LAUGHTER IS, as we all know, the best medicine. In The Human Face (BBC1), John Cleese investigated this idea by travelling to India to meet Dr Madan Kataria, who has founded a string of laughter clubs, called Laughter Heaven, where people gather in their hundreds for group laugh-ins. Dr Kataria claims laughter has all sorts of health benefits - reducing stress, boosting the immune system. Actually, there isn't a lot of scientific evidence for this; but after having a bit of a giggle himself, Cleese seemed convinced. He spoke zealously of the way that laughter breaks down barriers between people: "Laughter is a force for democracy."

The thing is, if Cleese really believes this, why isn't he funny any more? Perhaps that's not quite fair: this programme had some amusing moments, though it stopped a long way short of outright hilarity, unless Elizabeth Hurley sticking her tongue out gets you going. But these days, when it comes to comedy, there's an overpowering sense that Cleese's heart isn't in it. He's funny because that's what's expected of him, and because it's a good way of getting his message across. He's nursed this didactic streak for a long time - in the Seventies, he made a lot of money making training films for people in business. And to begin with, he was still capable of keeping a lid on it, so that he could simultaneously produce Fawlty Towers, a magnificent example of humour at its most pure - heartless, amoral, irresistible.

Since then, though, things have deteriorated: there were the partly political broadcasts for the SDP (I think it was the SDP at that point); the dreary lessons about the need to throw off your inhibitions that wrecked A Fish Called Wanda; those appallingly simple-minded radio shows and books about managing relationships that he produced in conjunction with the late Dr Robyn Skinner; even those Sony ads - I quite enjoyed them at the time, but in retrospect they seem to demonstrate Cleese's fatal willingness to bend the jokes to get his message across.

As it happens, in the case of The Human Face, unlike those other exercises in homiletic excess, Cleese's message is broadly right: it is that face-to-face contact is essential to our well-being, as individuals and as a society; and that technology - and, though he didn't mention this, including TV - is reducing our opportunities for such contact. There were also lots of fascinating titbits: the human face is capable of 7,000 different expressions! People who smile in photos have happier lives and marriages! We met a little girl with Mobius syndrome, which deprives her of all facial expression; we met an Asperger's syndrome sufferer, who has trouble interpreting people's expressions; we met a couple who are trying to save their marriage by cutting down on the lip- curling and eye-rolling. All in all, however, Cleese's moral felt rather de trop, a bit too crammed in.

There are other problems: Cleese's tendency to repeat what has just been said to him in almost identical terms; and my mild sense that if he's so bitter about technology, he should give Sony their money back. But mostly, it's frustration that Cleese doesn't think it's enough to make us laugh; and, damn, he was good at it.

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