title: Encephalopathy in Wilson disease: copper toxicity or liver failure?
abstract: Hepatic encephalopathy (HE) is a complex syndrome of neurological and psychiatric signs and symptoms that is caused by portosystemic venous shunting with or without liver disease irrespective of its etiology. The most common presentation of Wilson disease (WD) is liver disease and is frequently associated with a wide spectrum of neurological and psychiatric symptoms. The genetic defect in WD leads to copper accumulation in the liver and later in other organs including the brain. In a patient presenting with Wilsonian cirrhosis neuropsychiatric symptoms may be caused either by the metabolic consequences of liver failure or by copper toxicity. Thus, in clinical practice a precise diagnosis is a great challenge. Contrary to HE in neurological WD consciousness, is very rarely disturbed and pyramidal signs, myoclonus dominate. Asterixis and many other clinical symptoms may be present in both disease conditions and are quite similar. However details of neurological assessment as well as additional examinations could help in differential diagnosis.
authors.affiliation: Internal Medicine 3, Department of Gastroenterology and Hepatology, Medical University of Vienna, Austria.
authors.forename: Peter
authors.initials: P
authors.lastname: Ferenci
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
authors.forename: Tomasz
authors.initials: T
authors.lastname: Litwin
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Seniow
authors.affiliation: 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland ; Department of Experimental and Clinical Pharmacology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Czlonkowska
keywords: AHD, acquired hepatocerebral-degeneration; Cho, choline; EEG, electroencephalography; Glx, glutamine and glutamate; HE, hepatic encephalopathy; MHE, minimal hepatic encephalopathy; MI, myoinositol; MRI, magnetic resonance imaging; MRS, magnet resonance spectroscopy; NAA, N-acetyl-aspartate; WD, Wilson disease; Wilson disease; ammonia; copper; hepatic encephalopathy