A Case Study On Wilson’s Disease (Westphal- Strümpell Pseudosclerosis)
Case Study On Wilson’s Disease
Keywords:
Cirrhosis, Kayser-Fleischer rings & cerebral damageAbstract
Wilson disease of Progressive Lenticular Degeneration is a familial nervous disease associated with Cirrhosis of the Liver. It has been observed that there is an increased copper content of the liver and brain. Increased excretion of copper in patients with Wilson disease & increased even more after administration of the chelating agent British anti-Lewisite (BAL). Ciruloplasmin, serum protein that binds copper, is reduced. The deposition of copper in tissues is the cause of virtually all the manifestations of the disease in Liver Blood Kidney & Brain We have observed three different cases of different clinical presentations.
References
1. Scheinberg IH, Sternlieb I. Wilson’s disease. In: Smith LH Jr, ed. Major problems in internal medicine. Vol 23. Philadelphia: WB Saunders Company, 1984.
2. Brewer GJ. Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment. CNS Drugs. 2005; 19(3):185-92.
3. Sundaresan S, Eapen CE, Shaji RV, Chandy M, Kurian G, Chandy G. Screening for mutations in ATP7B gene using conformation-sensitive gel electrophoresis in a family with Wilson's disease. Med Sci Monit. Mar 2007; 13(3):CS38-40.
4. Sir William Gowers, S. A. Kinnier Wilson, Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, Brain 2009: 132; 1997–2001 | 1997.
5. Allan H.Ropper,Raymond Delacy Adams,Mourice Victor .The inherited metabolic diseases of the nervous system in Adams and Victor’s principles of neurology,9th edition, McGraw-Hill, 2009,37 940-941.
6. Owen CA Jr, Ludwig J. Inherited copper toxicosis in Bedlington terriers: Wilson's disease (hepatolenticular degeneration). Am J Pathol. Mar 1982; 106(3):432-4.
7. Sinha S, Taly AB, Ravishankar S, Prashanth LK, Vasudev MK. Central pontine signal changes in Wilson's disease: distinct MRI morphology and sequential changes with de-coppering therapy. J Neuroimaging. Oct 2007; 17(4):286-91.
8. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson's disease. Lancet. Feb 3 2007; 369(9559):397-408.
9. Eisenbach C, Sieg O, Stremmel W, Encke J, Merle U. Diagnostic criteria for acute liver failure due to Wilson disease. World J Gastroenterol. Mar 21 2007; 13(11):1711-4.
10. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study. Gut. Jan 2007; 56(1):115-20.
2. Brewer GJ. Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment. CNS Drugs. 2005; 19(3):185-92.
3. Sundaresan S, Eapen CE, Shaji RV, Chandy M, Kurian G, Chandy G. Screening for mutations in ATP7B gene using conformation-sensitive gel electrophoresis in a family with Wilson's disease. Med Sci Monit. Mar 2007; 13(3):CS38-40.
4. Sir William Gowers, S. A. Kinnier Wilson, Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, Brain 2009: 132; 1997–2001 | 1997.
5. Allan H.Ropper,Raymond Delacy Adams,Mourice Victor .The inherited metabolic diseases of the nervous system in Adams and Victor’s principles of neurology,9th edition, McGraw-Hill, 2009,37 940-941.
6. Owen CA Jr, Ludwig J. Inherited copper toxicosis in Bedlington terriers: Wilson's disease (hepatolenticular degeneration). Am J Pathol. Mar 1982; 106(3):432-4.
7. Sinha S, Taly AB, Ravishankar S, Prashanth LK, Vasudev MK. Central pontine signal changes in Wilson's disease: distinct MRI morphology and sequential changes with de-coppering therapy. J Neuroimaging. Oct 2007; 17(4):286-91.
8. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson's disease. Lancet. Feb 3 2007; 369(9559):397-408.
9. Eisenbach C, Sieg O, Stremmel W, Encke J, Merle U. Diagnostic criteria for acute liver failure due to Wilson disease. World J Gastroenterol. Mar 21 2007; 13(11):1711-4.
10. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study. Gut. Jan 2007; 56(1):115-20.
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Published
2012-06-30
How to Cite
Sadatia, D. .Vishal, Vekariya, D., Bavaliya, D., & Patel, D. (2012). A Case Study On Wilson’s Disease (Westphal- Strümpell Pseudosclerosis): Case Study On Wilson’s Disease. National Journal of Integrated Research in Medicine, 3(2), 174–176. Retrieved from http://nicpd.ac.in/ojs-/index.php/njirm/article/view/295
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Case Report
