[Skip to Navigation]
Article
October 1941

KAYSER-FLEISCHER RING ASSOCIATED WITH HEPATOLENTICULAR DEGENERATION: REPORT OF SIX CASES

Author Affiliations

NEW YORK
From the Ophthalmologic Service of Dr. Robert K. Lambert, Montefiore Hospital for Chronic Diseases.

Arch Ophthalmol. 1941;26(4):595-605. doi:10.1001/archopht.1941.00870160069006
Abstract

Hepatolenticular degeneration was defined as an entity by Wilson1 in 1912. However, typical examples had been described under other names by Frerichs2 in 1860 and by Gowers3 in 1888. The corneal ring was first described by Kayser4 in 1902, in a case in which the diagnosis was multiple sclerosis. Fleischer5 reported the ring in 1903 in a case in which the condition was called pseudosclerosis. Later it was appreciated that the Kayser-Fleischer ring is pathognomonic of hepatolenticular degeneration. The neurologic signs of this disease are not always diagnostic. The hepatic cirrhosis usually causes no symptoms; so the diagnosis of hepatolenticular degeneration often depends on finding the Kayser-Fleischer ring in the cornea.

Patients with this condition show a remarkable similarity in their family history and in the development of their symptoms. Consanguinity of the parents is often disclosed. The childhood and early development of the patients

Add or change institution
×