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Article
April 1960

Essential Hypercholesteremic Xanthomatosis with Neurological Involvement

Author Affiliations

Colombo, Ceylon
Department of Medicine, University of Ceylon.

AMA Arch Neurol. 1960;2(4):458-462. doi:10.1001/archneur.1960.03840100096013
Abstract

Thannhauser and Magendantz8 were the first to describe fully the syndrome of essential hypercholesteremic xanthomatosis. According to Thannhauser,9 this syndrome involves singly or in various combinations the skin, with tuberous or plain xanthomatous lesions; the tendons; the intima of the blood vessels and endocardium, and, in rare instances, the large bile ducts. There is an increase in the total cholesterol, which may be small, and a slight elevation of lecithin in the serum; the neutral fat is usually normal, but may be slightly raised. The color of the serum is yellow, unlike that in the cases of hyperlipemia, in which it is milky, due to an increase in the neutral fat. Thannhauser recommends use of the terms hypercholesteremia or hyperlecithemia when the lipids other than neutral fats are increased.

The involvement of the nervous system in association with essential hypercholesteremic xanthomatosis with tendon xanthomata appears to be a

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