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| The Elephant Man was not the Elephant Man. To be more precise, John Merrick did not suffer from the genetic disorder which has come to be known as "Elephant Man disease." Recent studies of Merrick's remains have confirmed theories that Merrick was the victim of a much rarer disease that was unidentified until a century after his death, in a discovery that would be comparable to learning that Lou Gehrig did not have amyotrophic lateral sclerosis. One early belief was that Merrick suffered from elephantiasis, a disorder of the lymphatic system that causes parts of the body to swell to grotesque proportions. Later it was theorized that he had an extremely severe case of Neurofibromatosis. This nervous system disorder causes nerve cells to grow out of control, creating large, misshapen tumors. Neurofibromatosis is not phenomenally rare, occurring in one of every 4,000 births, although no known case of neurofibromatosis has ever been as profound as Merrick's condition. In 1979, a much more rare disease was identified as causing overgrowth of bone and other tissue. This disorder, named Proteus Syndrome, has been recorded in fewer than 100 cases, ever. Several years ago a U.S. National Institutes of Health panel suggested that Proteus Syndrome may have been the true cause of John Merrick's plight. A study of Merrick's remains at the Royal London Hospital appears to back up this diagnosis. Radiologist Amita Sharma found that Merrick's spine was not as sharply curved as is normally found in cases of neurofibromatosis. In addition, Merrick's ribs do not demonstrate the peculiar notches and thinness associated with neurofibromatosis, and are actually abnormally thick. The extreme overgrowth of bone in Merrick's skull and the right side of his body is consistent with Proteus Syndrome. Sharma based her conclusions on a series of x-rays and CAT scans produced at the Royal London Hospital, where Merrick's remains have been kept since his death. Contrary to popular belief, the Elephant Man's skeleton has never been in the possession of Michael Jackson or any other freak-obsessed private collector. DNA tests will render a final confirmation of the Proteus Syndrome diagnosis, but it might be as long as two years before conclusive results are reached. Source 'Fortean Times' magazine | |
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