Hepatolenticular Degeneration


Cerebral Pseudoscleroses

Cerebral Pseudosclerosis

Copper Storage Disease

Degeneration, Hepatocerebral

Degeneration, Hepatolenticular

Degeneration, Neurohepatic

Degeneration, Progressive Lenticular

Degenerations, Hepatocerebral

Degenerations, Neurohepatic

Diseases, Hepato-Neurologic Wilson

Diseases, Kinnier-Wilson

Hepatic Form of Wilson Disease

Hepato Neurologic Wilson Disease

Hepato-Neurologic Wilson Disease

Hepato-Neurologic Wilson Diseases

Hepatocerebral Degeneration

Hepatocerebral Degenerations

Hepatolenticular Degeneration Syndrome

Kinnier Wilson Disease

Kinnier-Wilson Disease

Kinnier-Wilson Diseases

Lenticular Degeneration, Progressive

Neurohepatic Degeneration

Neurohepatic Degenerations

Progressive Lenticular Degeneration

Pseudoscleroses, Cerebral


Pseudosclerosis, Cerebral

Westphal Strumpell Syndrome

Westphal-Strumpell Syndrome

Westphal-Strumpell Syndromes

Wilson Disease

Wilson Disease, Hepatic Form

Wilson Disease, Hepato-Neurologic

Wilson Diseases, Hepato-Neurologic

Wilson's Disease

Wilsons Disease

A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC, also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.