The pseudosclerotic form (“wing beating tremor”) of Wilson’s disease

Abstract

Wilson disease is a rare monogenic, autosomal recessive disorder of copper metabolism, leading to progressive accumulation of copper in different organs, essentially in the liver, brain and cornea. We report a case of a 25 years old man, Caucasian, with “wing-beating tremor” in the right arm that started with two month in advance of hospital admission, than evolved to the left arm, a week before hospitalization. The slit-lamp examination showed the presence of Kayser-Fleischer rings in both eyes. The laboratory tests and brain MRI confi rmed the diagnostic of Wilson’s disease.

Keywords

• wilson disease
• copper metabolism
• wing-beating tremor