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J. Zelson, MD; A. S. Dekaban, MD

Arch Neurol. 1969;20(4):358-361.

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

HUNTER-HURLER'S disease is an born error of metabolism characterized by excessive deposition of acid mucopolysaccharides (chondroitin sulfate B and heparitin sulfate) in the liver, spleen, skin, and other organs whereas the nerve cells accumulate an abnormal amount of ganglioside containing lipids.^1,2 The main clinical features consist of dwarfism, hepatosplenomegaly, bone and joint deformities, mental and physical deterioration, corneal clouding and abnormal and coarse facial features: bushy eyebrows, sunken and upturned nose, thick lips, and hirsutism. The onset is commonly between a few months and 5 years of age and these patients rarely survive past the age of adolescence. Dorfman and Lorincz³ and subsequently many others demonstrated that the affected children excrete an excessive amount of acid mucopolysaccharide (AMPS) in their urine, while Danes and Bearn⁴ and Matalon and Dorfman⁵ showed that skin fibroblasts from these patients grown in tissue culture accumulate a large quantity of . . . [Full Text PDF of this Article]

Author Affiliations
Bethesda, Md

From the Section on Child Neurology, Surgical Neurology Branch, National Institute of Neurological Diseases and Blindness, National Institute of Health, Bethesda, Md. Dr. Zelson is now at Yale New Haven Hospital, New Haven, Conn.

Footnotes
Submitted for publication Aug 27, 1968; accepted Nov 5.

Read before the 93rd annual meeting of the American Neurological Association, Washington, DC, June 17, 1968.

Reprint requests to Section on Child Neurology, Surgical Neurology Branch, National Institute of Neurological Diseases and Blindness, National Institutes of Health, Bethesda, Md 20014 (Dr. Zelson).

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