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Cerebral gigantism

Sotos syndrome (also known as cerebral gigantism) is a rare genetic disorder characterized by excessive physical growth during the first 2 to 3 years of life. The disorder may be accompanied by mild mental retardation, delayed motor, cognitive, and social development, hypotonia (low muscle tone), and speech impairments. Children with Sotos syndrome tend to be large at birth and are often taller, heavier, and have larger heads (macrocrania) than is normal for their age. Symptoms of the disorder, which vary among individuals, include a disportionately large and long head with a slightly protrusive forehead, large hands and feet, hypertelorism (an abnormally increased distance between the eyes), and downslanting eyes. more...

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Clumsiness, an awkward gait, and unusual aggressiveness or irritability may also occur. Although most cases of Sotos syndrome occur sporadically, familial cases have also been reported.

Incidence

Incidence is approximately 1 in 14,000 births.

Description

Characterized by overgrowth and advanced bone age. Affected individuals are dysmorphic with macrodolichocephaly, downslanting palpebral fissures and a pointed chin. They have motor and speech delays but typically have normal intelligence.

Genetics

Most reported cases of Sotos syndrome have been sporadic and may represent new dominant mutations.

Treatment

There is no standard course of treatment for Sotos syndrome. Treatment is symptomatic.

Prognosis

Sotos syndrome is not a life-threatening disorder and patients may have a normal life expectancy. The initial abnormalities of Sotos syndrome usually resolve as the growth rate becomes normal after the first few years of life. Developmental delays may improve in the school-age years, however, coordination problems may persist into adulthood.

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MY, HOW HE'S GROWN
From Independent on Sunday, The, 10/2/05 by Daniel Utrilla

In a brown corduroy cap, the outsize man with the long, mournful face occupies the driving seat of an old horsedrawn cart. An elderly lady with an orange headscarf rests placidly on a heap of hay in the cart, her legs dangling. By her side, a bald man with gold teeth, and a young girl with a distracted gaze complete a trio of passengers. Dwarfed by the unusual driver, these three villagers look like hobbits from a Tolkein fantasy world.

The cart enters Podoliantsi " a tiny hamlet lost in the vast Ukrainian flatlands, 200km south-west of Kiev " and a plump woman ushers me out of one of the modest cottages by the roadside to meet her son.

Leonid Stadnik parks the cart by a shed and strides over, his remarkable stature making the gnarled apple tree in the courtyard look no bigger than a small bush. For a few of seconds, my hand remains trapped in his, like a ball in a baseball glove, enclosed by an ungraspable palm, some 31cm in diameter.

Stadnik takes off his size 27 shoes, then bows his head and tilts his chest in a synchronised movement that allows him to pass through the front door. We follow inside, to a gaudily carpeted living room, where he sits, hunched, on the edge of the bed which serves as his sofa.

'I'm just an average Ukrainian farmhand,' he says, in a voice like a slowed-down cassette tape. 'I get up at 5.30 in the morning to work my plot of land. At around 9.30 at night, we milk the cows for the third time, feed the animals and then go to bed. The work's very hard. We only take a day off on religious holidays. Often we'll work until we drop, but we're used to it from childhood. When I went to school I already worked in the kolkhoz (Soviet collective farm).'

For Stadnik, though, his daily chores present their own unique challenges. The last time ' he allowed anyone to measure him, in 2004, he reached 8ft 4in, making him by some distance the tallest man in the world, 7in taller than the official Guinness World Record holder, the Mongolian Xi Shun.

Stadnik suffers from a condition known as gigantism, triggered by an operation he underwent at 14 to remove a cerebral tumour. That operation damaged his pituitary gland (which generates the hormones that influence growth), and since then he hasn't stopped growing.

His vision and his legs are both weakened by the condition and carrying his giant frame around the fields can be difficult and exhausting. Particularly as he is still recovering from a fractured foot.

Unlike Xi Shun, who visited Britain last month for a promotional event, Stadnik shuns the limelight and is dismayed by his physique. 'I don't even know myself how tall I am or how much I weigh anymore,' he says. He has spurned requests by Guinness officials to measure him, and even avoids looking at his own reflection in mirrors.

In the past three years, though, he has grown by more than a foot and is likely to become the tallest man in recorded history, beating the American Robert Pershing Wadlow, who reached 8ft 11in before his death in 1940.

'It's difficult to live with my height, it's a curse,' he says. 'Not long ago I fell and hurt my lip... To fall from such a height is... Ufff!' With a great sigh, he expels several litres of air.

A qualified veterinarian, he never imagined he would be reduced to farm work. He spent 10 years as the in-house vet for a nearby cattle farm, but had to quit in 2002 after suffering frostbite on his feet because he was unable to afford properly fitted shoes. Now the government pays him a monthly pension of pounds 19 and he has better footwear " a pair of oversized boots made by a local firm. 'A Belgian company has promised to send me winter shoes too,' he says wistfully.

What is not covered is his colossal back. 'I've a smart suit, and shirts, but no working clothes and winter wear,' he says. At home, a coal-fired stove is his only protection against temperatures that, between December and February, plunge to 30 degrees below zero. 'I don't know if it's my height or my large skin surface, but I love the heat,' he confesses.

Despite his size, Stadnik is moderate, even frugal at mealtimes. 'Mostly I eat dairy products and potatoes. We make borscht and shchi [cabbage soup]', he says. His widowed mother, Galina, sitting beside us, nods in agreement. 'He doesn't drink or smoke. He's so good...' she murmurs. Such a devoted a son, in fact, that he won't leave her to seek medical help. Doctors in Britain say that his condition will deteriorate rapidly without surgery.

Stadnik says that he's too busy running the family's 1.8-hectare estate. 'We grow onions, cucumbers, tomatoes, maize... Not long ago one of the cows produced a sweet little calf.'

At least this rooted, pastoral existence affords him a degree of normality that he couldn't hope to achieve elsewhere. A year ago, a Ukrainian friend living in Germany invited him to visit his house and rented a minibus to take him there. The return journey was terrible. 'It took 38 hours. For me, travelling in a bus was the same as an ordinary person travelling in a car boot... I got so tired that I vowed I would never go anywhere ever again.'

A few months ago, though, he was persuaded by a group of villagers to visit the Carpathian mountains. 'We wanted to show him something taller than him,' explains a neighbour.

Stadnik couldn't wait to get back. 'You know, there's a real world and there's my world,' he says. 'On television I see the news about the war in Iraq, drug addiction, alcoholism, crime... It's all horrible, but it doesn't affect me. I live in a very closed space.'

As I prepare to take my leave, he tells me about one recent occasion when the outside world did impinge for a moment at least. Last December, along with the rest of the village, he was swept up by the excitement of the Orange Revolution, which brought the pro- European Viktor Yushchenko to the Ukrainian presidency. Stadnik was even given a mobile phone by local members of Yushchenko's party. Unfortunately, he found it impossible to use: his fingers were simply too large to operate the keypad.

'I've got great expectations of the party " we all want to live better,' he says, and then pauses, casting his eyes downwards, as if contemplating his monumental frame. 'But of course we can't change everything.' n

Another long day, clockwise from left: Stadnik harnesses his horse; with his mother, Galina; and struggling to operate a mobile phone

WHAT IS GIGANTISM?

Gigantism is a rare disease, affecting one in three million people. It is caused by the excessive secretion of growth hormone, which leads to abnormal bone growth and elevated height. Other adverse effects can include facial swelling, weakness, migraine, cardiac illness and diabetes. Sometimes the hormonal imbalance is caused by a benign tumour in the pituitary gland, but other pathologies can be responsible, too. There is no cure for the condition, but if the cause is a tumour, surgery can help. Drugs that reduce the quantity of growth hormone are another possible treatment.

Copyright 2005 Independent Newspapers UK Limited
Provided by ProQuest Information and Learning Company. All rights Reserved.

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