Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

HEPATOLENTICULAR DEGENERATION: Review of the Literature and Report of a Case with Dimercaprol (BAL) Therapy

HEPATOLENTICULAR DEGENERATION: Review of the Literature and Report of a Case with Dimercaprol... Abstract PROGRESSIVE lenticular degeneration (hepatolenticular degeneration, Wilson's disease) has been described as a rare disease involving the basal ganglion system and characterized by a chronic progressive fatal course. This disease is probably not so rare as was previously believed. One or two patients may be found in almost every city, and frequently in such cases the erroneous diagnosis of multiple sclerosis or paralysis agitans (Parkinsonism) is made. Recently, interest has been revived in this disease because of the possibility of a new and effective therapeutic agent. Occasional reports of small groups of patients treated with dimercaprol (Bal— 2,3-dimercaptopropanol) have been published in recent years. The most prominent of such reports is the one of Denny-Brown and Porter,1 in which they observed improvement in five patients who were subjected to repeated courses of dimercaprol. Our purpose in this report is to add an additional case report of a patient treated with References 1. Denny-Brown. D., and Porter, H.: The Effect of BAL (2,3-Dimercaptopropanol) on Hepatolenticular Degeneration (Wilson's Disease) , New England J. Med. 245:917-925 ( (Dec. 13) ) 1951.Crossref 2. Alpers, B. J.: Clinical Neurology , Ed. 2, Philadelphia, F. A. Davis Company, 1949, pp. 661-664. 3. Newell, F. W.; Cooper, J. A. D., and Farmer, C. J.: Effect of BAL (2,3-Dimercaptopropanol) on Intraocular Copper , Am. J. Ophth. 32:161-167 ( (June) , Pt. 2) 1949. 4. Haurowitz, F.: Über eine Anomalie des Kupferstoffwechsels , Ztschr. physiol. Chem. 190:72-74, 1930.Crossref 5. Glazebrook, A. J.: Wilson's Disease , Edinburgh M. J. 52:83-87 ( (Feb.) ) 1945. 6. Cumings, J. N.: The Copper and Iron Content of Brain and Liver in the Normal and in Hepatolenticular Degeneration , Brain 71:410-415 ( (Dec.) ) 1948.Crossref 7. Mandelbrote, B. M.; Stanier, M. W.; Thompson, R. H. S., and Thruston, M. N.: Studies on Copper Metabolism in Demyelinating Diseases of Central Nervous System , Brain 71:212-228 ( (June) ) 1948.Crossref 8. Cumings, J. N.: The Effects of BAL in Hepatolenticular Degeneration , Brain 74:10-22 ( (March) ) 1951.Crossref 9. Porter, H.: Copper Excretion in Urine of Normal Individuals and of Patients with Hepatolenticular Degeneration (Wilson's Disease) , Arch. Biochem. 31:262-265 ( (April) ) 1951.Crossref 10. Eden, A., and Green, H. H.: Micro-Determination of Copper in Biological Material , Biochem. J. 34:1202-1208 ( (Sept.) ) 1940. 11. Mallory, F. B., and Parker, F., Jr.: Experimental Copper Poisoning , Am. J. Path. 7: 351-364 ( (July) ) 1931. 12. Cameron, G. R.; Burgess, F., and Trenwith, V. S.: Possibility of Toxic Effects from 2,3-Dimercaptopropanol in Conditions of Impaired Renal or Hepatic Function , Brit. J. Pharmacol. 2:59-64 ( (March) ) 1947. 13. Dent, C. E.: Detection of Amino-Acids in Urine and Other Fluids , Lancet 2:637-639 ( (Nov. 2) ) 1946.Crossref 14. Conference on the Metabolic Aspects of Convalescence: Transactions of the 14th Meeting, New York, Nov. 12-13, 1946, edited by E. C. Reifenstein, Jr., and H. Adams, Josiah Macy, Jr., Foundation, 1947, pp. 126-157. 15. Peters, J. P., and Van Slyke, D. D.: Quantitative Clinical Chemistry: Interpretations , Ed. 2, Baltimore, Williams & Wilkins Company, 1946, Vol. 1. 16. Baker, A. B.: Interrelationship of Diseases of Liver and Brain , Arch. Path. 46:268-286 ( (Sept.) ) 1948. 17. Uzman, L. L., and Denny-Brown, D.: Amino-Aciduria in Hepatolenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:599-611 ( (June) ) 1948.Crossref 18. Uzman, L. L., and Hood, B.: The Familial Nature of the Amino Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392-400 ( (April) ) 1952.Crossref 19. Brick, I. B.: The Clinical Significance of Amino Aciduria , New England J. Med. 247: 635-644 ( (Oct. 23) ) 1952.Crossref 20. Himsworth, H. P.: Lectures on the Liver and Its Diseases , Ed. 2, Cambridge, Mass., Harvard University Press, 1950, pp. 98-101. 21. Hall, E. M., and MacKay, E. M.: Experimental Hepatic Pigmentation and Cirrhosis , Am. J. Path. 7:327-343 ( (July) ) 1931. 22. Wilson, S. A.: Progressive Lenticular Degeneration , Brain 34:295-509, 1912.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Internal Medicine American Medical Association

HEPATOLENTICULAR DEGENERATION: Review of the Literature and Report of a Case with Dimercaprol (BAL) Therapy

Download PDF
9 pages

Loading next page...
 
/lp/american-medical-association/hepatolenticular-degeneration-review-of-the-literature-and-report-of-a-mbEndNWQeD

References (25)

Publisher
American Medical Association
Copyright
Copyright © 1953 American Medical Association. All Rights Reserved.
ISSN
0888-2479
DOI
10.1001/archinte.1953.00240160127009
Publisher site
See Article on Publisher Site

Abstract

Abstract PROGRESSIVE lenticular degeneration (hepatolenticular degeneration, Wilson's disease) has been described as a rare disease involving the basal ganglion system and characterized by a chronic progressive fatal course. This disease is probably not so rare as was previously believed. One or two patients may be found in almost every city, and frequently in such cases the erroneous diagnosis of multiple sclerosis or paralysis agitans (Parkinsonism) is made. Recently, interest has been revived in this disease because of the possibility of a new and effective therapeutic agent. Occasional reports of small groups of patients treated with dimercaprol (Bal— 2,3-dimercaptopropanol) have been published in recent years. The most prominent of such reports is the one of Denny-Brown and Porter,1 in which they observed improvement in five patients who were subjected to repeated courses of dimercaprol. Our purpose in this report is to add an additional case report of a patient treated with References 1. Denny-Brown. D., and Porter, H.: The Effect of BAL (2,3-Dimercaptopropanol) on Hepatolenticular Degeneration (Wilson's Disease) , New England J. Med. 245:917-925 ( (Dec. 13) ) 1951.Crossref 2. Alpers, B. J.: Clinical Neurology , Ed. 2, Philadelphia, F. A. Davis Company, 1949, pp. 661-664. 3. Newell, F. W.; Cooper, J. A. D., and Farmer, C. J.: Effect of BAL (2,3-Dimercaptopropanol) on Intraocular Copper , Am. J. Ophth. 32:161-167 ( (June) , Pt. 2) 1949. 4. Haurowitz, F.: Über eine Anomalie des Kupferstoffwechsels , Ztschr. physiol. Chem. 190:72-74, 1930.Crossref 5. Glazebrook, A. J.: Wilson's Disease , Edinburgh M. J. 52:83-87 ( (Feb.) ) 1945. 6. Cumings, J. N.: The Copper and Iron Content of Brain and Liver in the Normal and in Hepatolenticular Degeneration , Brain 71:410-415 ( (Dec.) ) 1948.Crossref 7. Mandelbrote, B. M.; Stanier, M. W.; Thompson, R. H. S., and Thruston, M. N.: Studies on Copper Metabolism in Demyelinating Diseases of Central Nervous System , Brain 71:212-228 ( (June) ) 1948.Crossref 8. Cumings, J. N.: The Effects of BAL in Hepatolenticular Degeneration , Brain 74:10-22 ( (March) ) 1951.Crossref 9. Porter, H.: Copper Excretion in Urine of Normal Individuals and of Patients with Hepatolenticular Degeneration (Wilson's Disease) , Arch. Biochem. 31:262-265 ( (April) ) 1951.Crossref 10. Eden, A., and Green, H. H.: Micro-Determination of Copper in Biological Material , Biochem. J. 34:1202-1208 ( (Sept.) ) 1940. 11. Mallory, F. B., and Parker, F., Jr.: Experimental Copper Poisoning , Am. J. Path. 7: 351-364 ( (July) ) 1931. 12. Cameron, G. R.; Burgess, F., and Trenwith, V. S.: Possibility of Toxic Effects from 2,3-Dimercaptopropanol in Conditions of Impaired Renal or Hepatic Function , Brit. J. Pharmacol. 2:59-64 ( (March) ) 1947. 13. Dent, C. E.: Detection of Amino-Acids in Urine and Other Fluids , Lancet 2:637-639 ( (Nov. 2) ) 1946.Crossref 14. Conference on the Metabolic Aspects of Convalescence: Transactions of the 14th Meeting, New York, Nov. 12-13, 1946, edited by E. C. Reifenstein, Jr., and H. Adams, Josiah Macy, Jr., Foundation, 1947, pp. 126-157. 15. Peters, J. P., and Van Slyke, D. D.: Quantitative Clinical Chemistry: Interpretations , Ed. 2, Baltimore, Williams & Wilkins Company, 1946, Vol. 1. 16. Baker, A. B.: Interrelationship of Diseases of Liver and Brain , Arch. Path. 46:268-286 ( (Sept.) ) 1948. 17. Uzman, L. L., and Denny-Brown, D.: Amino-Aciduria in Hepatolenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:599-611 ( (June) ) 1948.Crossref 18. Uzman, L. L., and Hood, B.: The Familial Nature of the Amino Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392-400 ( (April) ) 1952.Crossref 19. Brick, I. B.: The Clinical Significance of Amino Aciduria , New England J. Med. 247: 635-644 ( (Oct. 23) ) 1952.Crossref 20. Himsworth, H. P.: Lectures on the Liver and Its Diseases , Ed. 2, Cambridge, Mass., Harvard University Press, 1950, pp. 98-101. 21. Hall, E. M., and MacKay, E. M.: Experimental Hepatic Pigmentation and Cirrhosis , Am. J. Path. 7:327-343 ( (July) ) 1931. 22. Wilson, S. A.: Progressive Lenticular Degeneration , Brain 34:295-509, 1912.Crossref

Journal

A.M.A. Archives of Internal Medicine – American Medical Association

Published: Apr 1, 1953

There are no references for this article.