Joseph Merrick -- THE Elephant Man

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Did he suffer from Elephantiasis, Neurofibromatosis or really Proteus Syndrome?

Known as the Elephant Man, Joseph Merrick only lived 27 years. Originally thought to suffer from Elephantiasis, in 1971 a diagnosis of Neurofibromatosis was offered and then finally a diagnosis in 1986 of Proteus Syndrome.

But, could there be still another explanation? Read on to learn more as I've added additional information including Paul Spring's diagnosis.

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Joseph Merrick at a glance 

Joseph Carey Merrick (5 August 1862 ? 11 April 1890) was an Englishman who became known as "The Elephant Man" because of his physical appearance caused by a congenital disorder. Because of his condition, he would garner the sympathy of the United Kingdom of Great Britain and Ireland during the Victorian era. He has often been incorrectly called John Merrick.

 

Deformity at early age ...

Merrick mentions that his deformity began developing at the age of three with small bumps appearing on the left side of his body.

Paul Spring Diagnosis: 

Paul Spiring, a biologist based in the Netherlands, has spanned the dichotomous divide in his solution to the mystery of Joseph Merrick, the so-called Elephant Man.

Merrick, who had been born in Leicester in 1862, came to the attention of the medical profession in 1886, when he was rescued from a freak show by the distinguished physician Sir Frederick Treves.

From the age of five, Merrick's body had become progressively disfigured by abnormal skin and bone development. The cause has never been identified. For many years, the leading explanation was a disease known as neurofibromatosis-1 (NF-1), a genetic condition capable of causing some of Merrick's symptoms. During the mid-1980s another diagnosis emerged: Proteus Syndrome, an extremely rare tissue-distorting genetic disease first identified in the late 1970s. Extensive studies of Merrick's skeleton - still preserved at the London Hospital - have failed to rule either explanation in or out.

Mr Spiring has now suggested a simple resolution of the mystery: both explanations are correct. He points out that NF-1 and Proteus Syndrome each explain some of Merrick's symptoms; in combination, they also explain the singular nature of his condition. NF-1 occurs in about one in 2,500 births, while Proteus Syndrome affects about one in 9 million, so assuming the conditions are independent, these figures imply that we should expect one case of combined NF-1/Proteus Syndrome every 22.5 billion births. No wonder doctors were so baffled by Merrick's symptoms.

Mr Spiring's explanation has the ring of truth. Frustratingly, however, his calculations also mean we must wait at least another 200 years before seeing a second Elephant Man by which to verify his intriguing suggestion. --www.telegraph.co.uk.

1986 Diagnosis: Proteus syndrome

It was postulated in 1986 that Merrick actually suffered from Proteus syndrome (a condition which had been identified by Michael Cohen seven years earlier).

More about Proteus syndrome 

Proteus syndrome is a congenital disorderJames, William; Berger, Timothy; Elston, Dirk (2005). Andrews Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0721629210. that causes skin overgrowth and atypical bone development, often accompanied by tumors over half the body.Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0071380760.

Proteus syndrome is highly variable, and is named after the Greek sea-god Proteus, who could change his shape.

Since Dr. Michael Cohen identified it in 1979, only a few more than 200 cases have been confirmed worldwide, with estimates that about 120 people are currently alive with the condition.Womans 11-stone legs may be lost at BBC As attenuated forms of the disease may exist, there could be many people with Proteus syndrome who remain undiagnosed. Those most readily diagnosed are also, unfortunately, the most severely disfigured.

1971 Diagnosis: Neurofibromatosis

In 1971, Ashley Montagu suggested in his book The Elephant Man: A Study in Human Dignity that Merrick suffered from neurofibromatosis type I, a genetic disorder also known as von Recklinghausen's disease.

More about Neurofibromatosis 

Neurofibromatosis (commonly abbreviated NF) is a genetically-inherited disease in which the nerve tissue grows tumors (i.e., neurofibromas) that may be harmless or may cause serious damage by compressing nerves and other tissues. The disorder affects all neural crest cells (Schwann cells, melanocytes, endoneurial fibroblasts). Cellular elements from these cell types proliferate excessively throughout the body forming tumors and the melanocytes function abnormally resulting in disordered skin pigmentation.The tumors may cause bumps under the skin, colored spots, skeletal problems, pressure on spinal nerve roots, and other neurological problems.http://www.merck.com/mmhe/sec06/ch088/ch088d.html Merck Manual Home Edition,

"Neurofibromatosis"

Neurofibromatosis is autosomal dominant, which means that it affects males and females equally and is dominant (only one copy of the affected gene is needed to get the disorder). Therefore, if only one parent has neurofibromatosis, his or her children have a 50% chance of developing the condition as well. Disease severity in affected individuals, however, can vary (this is called variable expressivity). Moreover, in around half of cases there is no other affected family member because a new mutation has occurred.

The Elephant Man, movie 

The Elephant Man [VHS]

Amazon Price: (as of 11/11/2009)Buy Now

You could only see his eyes behind the layers of makeup, but those expressive orbs earned John Hurt a well-deserved Oscar nomination for his moving portrayal of John Merrick, the grotesquely deformed Victorian-era man better known as The Elephant Man. Inarticulate and abused, Merrick is the virtual slave of a carnival barker (Freddie Jones) until dedicated London doctor Frederick Treves (Anthony Hopkins in a powerfully understated performance) rescues him from the life and offers him an existence with dignity. Anne Bancroft costars as the actress whose visit to Merrick makes him a social curiosity, with John Gielgud and Wendy Hiller as dubious hospital staffers won over by Merrick.

David Lynch earned his only Oscar nominations as director and cowriter of this somber drama, which he shot in a rich black-and-white palette, a sometimes stark, sometimes dreamy visual style that at times recalls the offbeat expressionism of his first film, Eraserhead. It remains a perfect marriage between traditional Hollywood historical drama and Lynch's unique cinematic eye, a compassionate human tale delivered in a gothic vein. The film earned eight Oscar nominations in all, and though it left the Oscar race empty-handed, its dramatic power and handsome yet haunting imagery remain just as strong today. --Sean Axmaker

The Elephant Man trailer 

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The buzz on Joseph Merrick 

A compliment...and a concern - Warwick - The Bass Forum
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Joseph Merrick was born on August 5th, 1862, at 50 Lee Street, Leicester, England. His parents were...
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Very Special model needed: Joseph Merrick's card church - Zealot ...
Very Special model needed: Joseph Merrick's card church Architecture Models.

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Drop me a line ... 

Had you heard about Joseph Merrick before? Seen the movie, The Elephant Man?

AndyPo wrote...

Excellent lens. Very interesting. The film is very good too.

ReplyPosted June 30, 2009

lilkon wrote...

What a sad commentary on the world we live in when it is a freak of nature that is a crowd pleaser as it was back in those times in England and is now in the U.S. Sci-Fi and even cartoons are doing the trick nowadays.

ReplyPosted June 04, 2009

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It was originally thought that Merrick suffered from Elephantiasis 

Elephantiasis (/??l?f?n?ta??s?s, -fæn-/ -fan-) is a disease that is characterized by the thickening of the skin and underlying tissues, especially in the legs, male genitals and female breasts. In some cases, the disease can cause certain body parts, such as the scrotum, to swell to the size of a softball or basketball. "Elephantitis" is a common mis-hearing or mis-remembering of the term, from confusing the ending -iasis (process or resulting condition) with the more commonly heard Category: wikt - :-itis|-itis (irritation or inflammation). The proper medical term is Elephantiasis,http://www.who.int/mediacentre/factsheets/fs102/en/ Retrieved on May 17, 2009 and it is caused by lymphatic filariasis.

Elephantiasis of the Ankles

Woman with Elephantiasis

More lenses on Elephantiasis 

The disease responsible for his appearance ...

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