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HEPATOLENTICULAR DEGENERATION: REPORT OF TWO CASES WITH PREDOMINANTLY HEPATOGENIC SYMPTOMS, ONE ASSOCIATED WITH THE CRUVEILHIER-BAUMGARTEN SYNDROME

HEPATOLENTICULAR DEGENERATION: REPORT OF TWO CASES WITH PREDOMINANTLY HEPATOGENIC SYMPTOMS, ONE... Abstract Wilson1 in 1912, in an article entitled "Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver," first clearly defined the condition now most widely known as hepatolenticular degeneration. His accurate description included and amplified the poorly characterized so-called pseudosclerosis, described by Westphal2 and by Strumpell2 in the closing years of the last century. Since the publication of Wilson's article, considerably more than 100 cases of this disease have been reported in the literature. The essential features of the syndrome are cirrhosis of the liver and a bilateral softening and degeneration of the lenticular nuclei. Although not hereditary in the sense of being passed from one generation to the next, the disease has a pronounced familial tendency and frequently affects several children in the same family. As many as five of seven siblings have been known' to have the disease. It is a disease References 1. Wilson, S. A. K.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295-509 ( (March) ) 1912.Crossref 2. Cited by Wilson.3 3. Wilson, S. A. K.: Neurology , Baltimore, Williams & Wilkins Company, 1940, vol. 2, pp. 806-831. 4. Sweet, W. H.; Gray, S. J., and Allen, J. G.: Clinical Detection of Hepatic Disease in Hepatolenticular Degeneration: Report of Nine Cases , J. A. M. A. 117:1613-1619 ( (Nov. 8) ) 1941.Crossref 5. Walsh, M. N.: Hepatolenticular Degeneration , Proc. Staff Meet., Mayo Clin. 11:757-762 ( (Nov. 25) ) 1936. 6. Cusick, P. L., in discussion on Walsh,5 pp. 762-763. 7. Cited by Armstrong, Adams, Tragerman and Townsend.8 8. Armstrong, E. L.; Adams, W. L., Jr.; Tragerman, L. J., and Townsend, E. W.: The Cruveilhier-Buamgarten Syndrome: Review of the Literature and Report of Two Additional Cases , Ann. Int. Med. 16: 113-151 ( (Jan.) ) 1942.Crossref 9. Valk, H. L., and Horne, S. F.: Cruveilhier-Baumgarten Syndrome (Splenomegaly, Portal Hypertension and Patent Umbilical Vein): Case Report , Ann. Surg. 116:860-863 ( (Dec.) ) 1942.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

HEPATOLENTICULAR DEGENERATION: REPORT OF TWO CASES WITH PREDOMINANTLY HEPATOGENIC SYMPTOMS, ONE ASSOCIATED WITH THE CRUVEILHIER-BAUMGARTEN SYNDROME

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References (7)

Publisher
American Medical Association
Copyright
Copyright © 1945 American Medical Association. All Rights Reserved.
ISSN
0730-188X
DOI
10.1001/archinte.1945.00210270008002
Publisher site
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Abstract

Abstract Wilson1 in 1912, in an article entitled "Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver," first clearly defined the condition now most widely known as hepatolenticular degeneration. His accurate description included and amplified the poorly characterized so-called pseudosclerosis, described by Westphal2 and by Strumpell2 in the closing years of the last century. Since the publication of Wilson's article, considerably more than 100 cases of this disease have been reported in the literature. The essential features of the syndrome are cirrhosis of the liver and a bilateral softening and degeneration of the lenticular nuclei. Although not hereditary in the sense of being passed from one generation to the next, the disease has a pronounced familial tendency and frequently affects several children in the same family. As many as five of seven siblings have been known' to have the disease. It is a disease References 1. Wilson, S. A. K.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295-509 ( (March) ) 1912.Crossref 2. Cited by Wilson.3 3. Wilson, S. A. K.: Neurology , Baltimore, Williams & Wilkins Company, 1940, vol. 2, pp. 806-831. 4. Sweet, W. H.; Gray, S. J., and Allen, J. G.: Clinical Detection of Hepatic Disease in Hepatolenticular Degeneration: Report of Nine Cases , J. A. M. A. 117:1613-1619 ( (Nov. 8) ) 1941.Crossref 5. Walsh, M. N.: Hepatolenticular Degeneration , Proc. Staff Meet., Mayo Clin. 11:757-762 ( (Nov. 25) ) 1936. 6. Cusick, P. L., in discussion on Walsh,5 pp. 762-763. 7. Cited by Armstrong, Adams, Tragerman and Townsend.8 8. Armstrong, E. L.; Adams, W. L., Jr.; Tragerman, L. J., and Townsend, E. W.: The Cruveilhier-Buamgarten Syndrome: Review of the Literature and Report of Two Additional Cases , Ann. Int. Med. 16: 113-151 ( (Jan.) ) 1942.Crossref 9. Valk, H. L., and Horne, S. F.: Cruveilhier-Baumgarten Syndrome (Splenomegaly, Portal Hypertension and Patent Umbilical Vein): Case Report , Ann. Surg. 116:860-863 ( (Dec.) ) 1942.Crossref

Journal

Archives of Internal Medicine – American Medical Association

Published: Mar 1, 1945

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