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Wendy M. Robertson, MD

Arch Neurol. 2000;57:276-277.

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

INTRODUCTION

Wilson's disease, or hepatolenticular degeneration, is now known to result from defective biliary copper excretion with copper accumulation in several tissues, including the liver, brain, and cornea. An inevitably fatal disease at the time of Wilson's description, several treatment options are now available to control the disease manifestations. This article cites several seminal works published before the use of penicillamine in the treatment of Wilson's disease.¹

CLINICAL RECOGNITION

The first published description of the disease that later became known as Wilson's disease, was that of Westphal² in 1883. He described 2 young patients with a progressive neurologic disease that differed from multiple sclerosis; hence, he called the disease "pseudosclerosis." The association of hepatic cirrhosis was noted in cases reported by Strümpell.³ In 1888, Gowers described 2 brothers with "tetanoid chorea," later noted to be associated with cirrhosis of the liver:

A case was recently under my care that . . . [Full Text of this Article]