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Manganese is an essential trace mineral that has a vital role in maintaining various body functions. Manganese toxicity, referred to as Manganism, causing parkinsonism is a well-known phenomenon that occurs secondary to chronic environmental and occupational exposure to manganese. Patients with underlying chronic liver disease are also susceptible to the toxic accumulation of manganese since it essentially undergoes biliary excretion.

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Recent Updates on Acquired Hepatocerebral Degeneration.

Tremor Other Hyperkinet Mov (N Y)

March 2018

Department of Neurology, Inje University Ilsan-Paik Hospital, Goyang, Republic of Korea.

Background: Acquired hepatocerebral degeneration (AHD) refers to a chronic neurological syndrome in patients with advanced hepatobiliary diseases. This comprehensive review focuses on the pathomechanism and neuroimaging findings in AHD.

Methods: A PubMed search was performed using the terms "acquired hepatocerebral degeneration," "chronic hepatocerebral degeneration," "Non-Wilsonian hepatocerebral degeneration," "cirrhosis-related parkinsonism," and "manganese and liver disease.

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Non-Wilsonian hepatolenticular degeneration (NWHD) is a heterogeneous neurological disorder occurring secondary to chronic acquired liver disease. Genetically determined familial NWHD is rare, poorly understood, and often mistaken for Wilson's disease (WD). We analysed clinical and MRI profiles of NWHD patients who did not have obvious cause for acquired liver disease, such as alcohol intake or hepatitis.

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Objective: Parkinsonism and other movement disorders have previously been reported in the acquired hepatocerebral degeneration associated with portosystemic shunting. However, there is no study to date about their prevalence as has been noted in general practice.

Methods: One hundred and forty-three patients with hepatic cirrhosis from the gastroenterology clinic and internal medicine wards were enrolled.

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Neurological manifestations are common in patients with decompensated cirrhosis. The majority of these patients show hepatic encephalopathy or chronic acquired (non-Wilsonian) hepatocerebral degeneration (CAHD). They characteristically present with dysarthria, ataxia, involuntary movements, and altered mental status.

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