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Atypical neurological disorders in an elderly man with liver cirrhosis

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Atypical neurological disorders in an elderly man with liver cirrhosis

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Guerino Recinella, Giovanni Serafini, Donatella Magalotti & Marco Zoli 

Introduction

Acquired hepatocerebral degeneration (AHD) is a rare and often unrecognized chronic neurologic disorder that occurs in patients with liver cirrhosis and seems related to portosystemic shunts. The exact prevalence of this disorder is unknown, but it seems to occur in 1–2% of patients with liver cirrhosis and portosystemic shunts [1].

The clinical manifestations of AHD are heterogeneous but movement disorders are the most common features. About 20% of AHD patients have parkinsonism and also ataxia is commonly reported.

The pathogenesis of AHD remains not completely clear; however, heavy metals have an important role in pathophysiology. The accumulation of manganese plays a key role in the alteration of dopaminergic neurotransmission in AHD. In patients with AHD, toxic substances are not sufficiently removed by the hepatobiliary system because of the presence of portosystemic shunts; so, these neuroactive agents are able to enter in the brain via the systemic circulation.

Several drugs have been tried for symptomatic management of AHD such as levodopa, dopamine receptor antagonists and manganese chelators but the only effective therapy is the liver transplantation.

We present typical clinical and instrumental findings of AHD in a patient with chronic liver disease due to HBV infection.

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Year 2020
Language English
Format PDF
DOI 10.1007/s40520-019-01275-7