This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on HighWire  • Citing articles on Web of Science (12)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

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

ERIC E. WOLLAEGER, M.D.; HARLEY C. SHANDS, M.D.

Arch Intern Med. 1945;75(3):151-154.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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 . . . [Full Text PDF of this Article]

Author Affiliations
Fellow in Medicine, Mayo Foundation ROCHESTER, MINN.

From the Division of Medicine, Mayo Clinic.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?