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WILSON'S DISEASE—CHRONIC FORM: Clinical-Pathological Observations in a Brother and Sister

WILSON'S DISEASE—CHRONIC FORM: Clinical-Pathological Observations in a Brother and Sister Abstract ALTHOUGH we deplore the general use L of eponyms to designate disease entities, under certain circumstances an eponym is more informative than a purely descriptive clinical pathological term. Such is the case with Wilson's disease, in which the classic clinical triad of cirrhosis of the liver, degeneration of the lenticular nuclei, and Kayser-Fleischer rings has, in recent years, been somewhat overshadowed by the occurrence of massive amino-aciduria and increased urinary excretion of copper. The pathological designation of hepatolenticular degeneration is, in a sense, misleading, since the changes in the brain are certainly not limited to the lenticular nuclei. Wilson's disease generally appears in an acute or a chronic form, although sharp delineations cannot always be made. The chronic form is often mistaken for multiple sclerosis or chronic encephalitic Parkinsonism until its true nature is determined by the recognition of the characteristic pigmented ring at the corneoscleral junction and the clinical References 1. Wilson, S. A. K.: Neurology , edited by A. Ninian Bruce, Baltimore, William Wood & Company, 1941. 2. Wilson, S. A.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295, 1912.Crossref 3. Hall, H. C.: La dégénérescence hepato-lenticulare; maladie de Wilson: pseudosclerosis , Paris, Masson & Cie, 1921. 4. Baker, A. B.: Central Nervous System in Hepatic Disease , J. Neuropath. & Exper. Neurol. 8:283, 1949. 5. Stokes, J. F.; Owen, J. R., and Holmes, E. G.: Neurological Complications of Infantile Hepatitis , Brit. M. J. 2:642, 1945. 6. Lüthy, F.: Über die hepato-lentikuläre Degeneration , Deutsche Ztschr. Nervenh. 123:101, 1932. 7. Uzman, L., and Denny-Brown, D.: Amino-Aciduria in Hepato-Lenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:599, 1948. 8. Moore, S., and Stein, W. H.: Amino-Aciduria in Wilson's Disease, Proceedings Second International Congress of Biochemistry , Paris, 1952, p. 367. 9. Haurowitz, F.: Über eine Anomalie des Kupferstoffwechsels , Ztschr. physiol. Chem. 190:72, 1930. 10. Glazebrook, A. J.: Wilson's Disease , Edinburgh M. J. 52:83, 1945. 11. Cumings, J. N.: Copper and Iron Content of Brain and Liver in the Normal and in Hepatolenticular Degeneration , Brain 71:410, 1948. 12. Mandelbrote, B. M.; Stanier, M. W.; Thompson, R. H. S., and Thruston, M. N.: Studies on Copper Metabolism in Demyelinating Diseases of the Central Nervous System , Brain 71:212, 1948. 13. Denny-Brown, D., and Porter, H.: Effect of BAL (2,3-Dimercaptopropanol) on Hepato-Lenticular Degeneration (Wilson's Disease) , New England J. Med. 245:917, 1951. 14. Scheinberg, I. H., and Gitlin, D.: Deficiency of Coeruloplasmin in Patients with Hepatolenticular Degeneration (Wilson's Disease) , Science 116: -484, 1952. 15. Uzman, L. L.: On the Relationship of Urinary Copper Excretion to the Amino-Aciduria in Wilson's Disease , Am. J. M. Sc. 226:645, 1953. 16. Lichtenstein, B. W.: Sturge-Weber-Dimitri Syndrome , A. M. A. Arch. Neurol. & Psychiat. 71:291, 1954. 17. Lichtenstein, B. W., and Rosenberg, C.: Sturge-Weber-Dimitri's Disease: Report of an Abortive Case with Observations on the Form, Chemical Nature and Pathogenesis of the Cerebral Cortical Concretions , J. Neuropath. & Exper. Neurol. 6:369, 1947. 18. Richter, R.: Pallial Component in Hepatolenticular Degeneration , J. Neuropath. & Exper. Neurol. 71:1, 1948. 19. Ostertag, B.: Über eine neuartige heredodegenerative Erkrankungsform localiziert in Striatum und Rinde mit ausgedehnter Myelolyse , Arch. f. Psychiat. 77:453, 1926. 20. Bielschowsky, M., and Hallervorden, J.: Symmetrische Einschmelzungsherde im Stirnhirn beim Wilson-Pseudosklerosekomplex , J. Psychol. u. Neurol. 42:177, 1931. 21. Gray, S. J.: Colloidal Gold Reaction of Blood Serum in Diseases of the Liver , Arch. Int. Med. 65:524, 1940. 22. Sweet, W. H.; Gray, S. J., and Allen, J. G.: Clinical Detection of Hepatic Disease in Hepatolenticular Degeneration , J. A. M. A. 117:1613, 1941. 23. Franklin, E. C., and Bauman, A.: Liver Dysfunction in Hepatolenticular Degeneration , Am. J. Med. 15:450, 1953. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Neurology & Psychiatry American Medical Association

WILSON'S DISEASE—CHRONIC FORM: Clinical-Pathological Observations in a Brother and Sister

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Publisher
American Medical Association
Copyright
Copyright © 1955 American Medical Association. All Rights Reserved.
ISSN
0096-6886
DOI
10.1001/archneurpsyc.1955.02330070015003
Publisher site
See Article on Publisher Site

Abstract

Abstract ALTHOUGH we deplore the general use L of eponyms to designate disease entities, under certain circumstances an eponym is more informative than a purely descriptive clinical pathological term. Such is the case with Wilson's disease, in which the classic clinical triad of cirrhosis of the liver, degeneration of the lenticular nuclei, and Kayser-Fleischer rings has, in recent years, been somewhat overshadowed by the occurrence of massive amino-aciduria and increased urinary excretion of copper. The pathological designation of hepatolenticular degeneration is, in a sense, misleading, since the changes in the brain are certainly not limited to the lenticular nuclei. Wilson's disease generally appears in an acute or a chronic form, although sharp delineations cannot always be made. The chronic form is often mistaken for multiple sclerosis or chronic encephalitic Parkinsonism until its true nature is determined by the recognition of the characteristic pigmented ring at the corneoscleral junction and the clinical References 1. Wilson, S. A. K.: Neurology , edited by A. Ninian Bruce, Baltimore, William Wood & Company, 1941. 2. Wilson, S. A.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295, 1912.Crossref 3. Hall, H. C.: La dégénérescence hepato-lenticulare; maladie de Wilson: pseudosclerosis , Paris, Masson & Cie, 1921. 4. Baker, A. B.: Central Nervous System in Hepatic Disease , J. Neuropath. & Exper. Neurol. 8:283, 1949. 5. Stokes, J. F.; Owen, J. R., and Holmes, E. G.: Neurological Complications of Infantile Hepatitis , Brit. M. J. 2:642, 1945. 6. Lüthy, F.: Über die hepato-lentikuläre Degeneration , Deutsche Ztschr. Nervenh. 123:101, 1932. 7. Uzman, L., and Denny-Brown, D.: Amino-Aciduria in Hepato-Lenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:599, 1948. 8. Moore, S., and Stein, W. H.: Amino-Aciduria in Wilson's Disease, Proceedings Second International Congress of Biochemistry , Paris, 1952, p. 367. 9. Haurowitz, F.: Über eine Anomalie des Kupferstoffwechsels , Ztschr. physiol. Chem. 190:72, 1930. 10. Glazebrook, A. J.: Wilson's Disease , Edinburgh M. J. 52:83, 1945. 11. Cumings, J. N.: Copper and Iron Content of Brain and Liver in the Normal and in Hepatolenticular Degeneration , Brain 71:410, 1948. 12. Mandelbrote, B. M.; Stanier, M. W.; Thompson, R. H. S., and Thruston, M. N.: Studies on Copper Metabolism in Demyelinating Diseases of the Central Nervous System , Brain 71:212, 1948. 13. Denny-Brown, D., and Porter, H.: Effect of BAL (2,3-Dimercaptopropanol) on Hepato-Lenticular Degeneration (Wilson's Disease) , New England J. Med. 245:917, 1951. 14. Scheinberg, I. H., and Gitlin, D.: Deficiency of Coeruloplasmin in Patients with Hepatolenticular Degeneration (Wilson's Disease) , Science 116: -484, 1952. 15. Uzman, L. L.: On the Relationship of Urinary Copper Excretion to the Amino-Aciduria in Wilson's Disease , Am. J. M. Sc. 226:645, 1953. 16. Lichtenstein, B. W.: Sturge-Weber-Dimitri Syndrome , A. M. A. Arch. Neurol. & Psychiat. 71:291, 1954. 17. Lichtenstein, B. W., and Rosenberg, C.: Sturge-Weber-Dimitri's Disease: Report of an Abortive Case with Observations on the Form, Chemical Nature and Pathogenesis of the Cerebral Cortical Concretions , J. Neuropath. & Exper. Neurol. 6:369, 1947. 18. Richter, R.: Pallial Component in Hepatolenticular Degeneration , J. Neuropath. & Exper. Neurol. 71:1, 1948. 19. Ostertag, B.: Über eine neuartige heredodegenerative Erkrankungsform localiziert in Striatum und Rinde mit ausgedehnter Myelolyse , Arch. f. Psychiat. 77:453, 1926. 20. Bielschowsky, M., and Hallervorden, J.: Symmetrische Einschmelzungsherde im Stirnhirn beim Wilson-Pseudosklerosekomplex , J. Psychol. u. Neurol. 42:177, 1931. 21. Gray, S. J.: Colloidal Gold Reaction of Blood Serum in Diseases of the Liver , Arch. Int. Med. 65:524, 1940. 22. Sweet, W. H.; Gray, S. J., and Allen, J. G.: Clinical Detection of Hepatic Disease in Hepatolenticular Degeneration , J. A. M. A. 117:1613, 1941. 23. Franklin, E. C., and Bauman, A.: Liver Dysfunction in Hepatolenticular Degeneration , Am. J. Med. 15:450, 1953.

Journal

A.M.A. Archives of Neurology & PsychiatryAmerican Medical Association

Published: Jan 1, 1955

References