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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Ministry of silly faces
From Evening Standard (London), 3/8/01 by VICTOR LEWIS-SMITH

A TV critic may be someone who spends their life heckling trains that have already left the platform, but, on the reviewers' ladder of uselessness, there's one rung even lower. It's occupied by the restaurant critic, and I've been one of those too, having spent several years flirting with obesity on behalf of the toffs' glossy. While observing the cut and thrust of restaurant land, I often heard putdowns that were sharper than Michael Jackson's nose, ranging from the simple but effective "I presume these are capers, or perhaps you have a rabbit in the kitchen?", to the memorable "would you like to see the menu, sir?" "No, I'll just look at the stains on your apron and try to guess." And perhaps the most astringent (albeit fictional) interchange I ever witnessed was hearing a diner bark: "I'd like the crab toasted," whereupon the waiter raised his glass to the customer's wife, and said: "Your health, madame."

But when it comes to Michelin-starred putdowns, we all stand in awe of John Cleese, for he is the unrivalled knees of Eric the half- bee. "The problem with crocodiles as dramatic actors," he told us during last night's The Human Face (BBC1), "is that they only have one expression and that's three less than Michael Palin", and his scientific enquiry into the mysteries of our physiognomy was peppered with dozens of similarly brusque sideswipes, most of them involving his assistant, Liz Hurley. According to our Janet and John presenters, humans can muster about 7,000 distinct expressions (thanks to the 44 muscles that control our face), although there are just five primary ones, "happiness, surprise, anger, fear and disgust".

And as Liz demonstrated this extremely basic quintet, I realised that there was a sixth variety which had somehow been overlooked: the expression of drooling, helpless, hopeless lust which appears on the face of a sad, fat, physiognomically challenged, lecherous food- critic-turned-TV-reviewer of a certain age when he's staring at every part of Ms Hurley except her face.

Cleese's central proposition was that humans learn as much about each other from facial expressions as from words, and, therefore, need to see each other in order to communicate properly.

"We're social animals, that's why the punishment most feared in prison is solitary confinement," he argued, and, although he'd perhaps overlooked the alternative of being banged up two-in-a-cell with a 25-stone pederast, he convincingly demonstrated the vital importance of face-to-face contact.

Research into micro-expressions has revealed telltale signs of deceit, and speaking as someone who spends a good deal of time in front of a video editor, I can confirm that plausible liars do indeed give themselves away by split-second displays of self-revulsion, often captured in a single frame of footage. In fact, that's why I never believed Bill Clinton, back in the days when he was solemnly denying that he'd ever splashed out on Monica Lewinsky's frocks.

Less engrossing were the slushy, Desmond Wilcox-style human- interest inserts, during which dysfunctional human dollies were played with and experimented upon by a gaggle of US hackademics. Five- year-old Lauren had Mobius syndrome (facial paralysis), so surgeons connected her thigh muscle to her cheek bone in an operation which enabled her to smile (but disproved the lyrics of a famous old song about dem dry bones), while a bickering English couple flew to a Seattle laboratory where they were informed that "expressions of disgust and contempt are a very good predictor of divorce".

As the wife made those very faces every time her husband spoke to her, I didn't hold out much hope for their shared future, and by the end I wasn't too optimistic about the shared future of the human race either, given Cleese's dire warnings about the effect that technological isolation is having on our ability to comprehend one another. And how were we all learning this disturbing news?

Siting alone in our houses, solitarily watching the world through the flickering eye of the magic rectangle.

EVEN though we'd all still rather see Cleese doing his silly walk than lecturing us on sensible topics, he remains a master of delivery and timing, and last night he even succeeded in making Liz Hurley seem like more than just a pretty face. In a modern usage of the Shavian sugar-coated-pill technique (as pioneered by George Bernard Shave himself), he helped us to swallow a good deal of unpalatable fact by sweetening it with comedy, and my only complaint was that the review tape I received was of lousy quality, having been dubbed by the BBC on a cheap and nasty VHS player. I'm not an unreasonable man, ladies and gentlemen, but I ask you: would a theatre critic be expected to watch a play through a snowstorm (unless he was reviewing Scott of the Antarctic)? And if the person who dubbed the tape wants to take issue with me, then come on if you think you're hard enough, but let me warn you that I have my own piece of wisdom to impart to you about the human face.

Never pick a fight with an ugly person. They have nothing to lose.

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