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Paramyotonia congenita

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365 children? No, but a strange birth did happen
From Deseret News (Salt Lake City), 1/6/05 by Bill Sones

Question: If Countess Margaret of the village of Henneberg, the Netherlands, didn't really give birth to 365 children on Good Friday of 1276, what might she have given birth to?

Answer: Wooden plates in the village still commemorate this "strangest miracle in the world," says Jan Bondeson in "The Two- Headed Boy and Other Medical Marvels."

As the text on the baptismal church tells it, Margaret had insulted a poor woman with a pair of twins in her arms, who then cursed the Countess to "have as many children as there are days in the year." Or so the legend goes.

Today, it is obvious that it is physiologically impossible to give birth to 365, or even 36, children in one birth, says Bondeson. In 1971, a set of nine children was born in Sydney, Australia. A German woman "with prodigious belly" supposedly gave birth to 11 in 1501.

Regarding Countess Margaret, two gynecologists in the 1930s suggested the 365 children may have really been a "hydatidiform mole." Here the diseased placenta takes on the appearance of "a bunch of grapes," actually cysts, possibly a hundred or more of different sizes and shapes. After their spontaneous expulsion, the patient might bleed to death -- the fate of the Countess. The people of the time understandably may have likened the cysts to children, and the legend was born. Nowadays these moles occur once in about 2,000 pregnancies, but 10 times that in developing countries, which "speaks in favor of the disease being by no means unknown in Holland of the 13th century."

Question: Baby's on the way: Whether a boy or a girl is roughly 50- 50, but if you bet even odds on a girl every time -- in absence of fetal testing to take away the gamble -- you'd either lose your shirt or clean up. Which is it?

Answer: The shirt goes. Demographers know that the sex ratio at birth is typically about 105 males to 100 females (51 percent vs. 49 percent) and that this holds generally across societies, says Bart Holland in "What Are the Chances?"

Thus, if you always bet on boys and an opponent bets on girls, your profit margin in the long run would be about as good as a Monte Carlo casino on a roulette wheel! But males have a higher mortality rate at every age, from birth on, so the surplus proportion of boys shrinks with every passing year of age, until finally widows far outnumber widowers.

Question: Rabid Pittsburgh Steelers fan loves to go to football games with "Steelers" emblazoned on his forehead. He doesn't use paints, inks, tattoos, or the like. So what is his trick? Clue: Try asking his dermatologist.

Answer: "Dermographia," or "skin-writing," occurs in about 5 percent of the population, who experience a linear itchy hives -- like swelling within minutes of stroking the skin with an object, says Heather Brannon MD on Dermatology.About.com. (This is in contrast to healthy people who when scratched have a linear reddening without the itching.) Fortunately for this Steelerite, the reaction generally resolves within hours.

The tendency to be dermographic may last for weeks or years, possibly preceded by a viral infection, antibiotic therapy or emotional upset. In most cases, however, the cause is unknown, says Dr. Brannon. Dermographia, or dermatographia, can be uncomfortable but isn't serious, says MayoClinic.com. Often it goes away on its own, or antihistamines may help. "People with this condition should avoid irritating their skin whenever possible."

Question: What happens when someone with rare inherited "paramyotonia congenita" goes out into winter cold?

Answer: When the temperature drops, sufferers become paralyzed, unable even to release their grip on a snow shovel or the cold metal handlebars of a bicycle, says Frances Ashcroft in "Life at the Extremes: The Science of Survival."

Even just drinking iced tea or eating ice cream can bring on stiffness of the tongue and slurred speech. These patients have a mutation in a gene that codes for a protein critical for the conduction of electrical signals along muscle fibers. "The condition is not life-threatening -- it does not paralyze the respiratory muscles -- but it is undoubtedly very inconvenient."

Send STRANGE questions to brothers Bill and Rich at strangetrue@compuserve.com

Copyright C 2005 Deseret News Publishing Co.
Provided by ProQuest Information and Learning Company. All rights Reserved.

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