As of '87, he's Proteus Man.As of '87, he's Proteus Man Neurofibromatosis Neurofibromatosis Definition Neurofibromatosis (NF), or von Recklinghausen disease, is a genetic disease in which patients develop multiple soft tumors (neurofibromas). These tumors occur under the skin and throughout the nervous system. , the genetic disorderthat has come to be known as Elephant Man Elephant Man orig. Joseph (Carey) Merrick (born Aug. 5, 1862, Leicester, Leicestershire, Eng.—died April 11, 1890, London) Englishman disfigured by a disease that caused growths over his skin and bone surfaces. His head was 3 ft (. disease (SN: 6/6/87, p.359), was probably not the cause of the Elephant Man's deformities. That is the conclusion of a National Institutes of Health panel, which last week released its final report on the incurable incurable /in·cur·a·ble/ (in-kur´ah-b'l) 1. not susceptible of being cured. 2. a person with a disease which cannot be cured. in·cur·a·ble adj. disease. The report doesn't address directly the case of Joseph Merrick--the 19th-century "Elephant Man' who later became the subject of a popular movie and play. But according to according to prep. 1. As stated or indicated by; on the authority of: according to historians. 2. In keeping with: according to instructions. 3. panel chairman David A. Stumpf, it was the group's consensus that Merrick actually suffered from an extremely rare disease known as the Proteus syndrome Proteus Syndrome is a congenital disorder that causes skin overgrowth and atypical bone development, often accompanied by tumors on over half the body. Proteus Syndrome is extremely rare. Since Dr. . The updated diagnosis is of more than historical interest, as it may help to free neurofibromatosis victims from the fear of the severe deformation that is more properly associated with the Proteus syndrome. The experts recommend areas for further research, and one panelist predicts that the neurofibromatosis-causing gene will be definitively identified in the next year or two--a critical step in the development of a treatment or cure. |
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