Uncommon Movement Disorders in Chronic Hepatic Disease with Response to Rifaximin

Ana L Sousa,Sara Silva,José E Alves,Sofia Ferreira,Paula Salgado,Marina Magalhães

doi:10.5334/tohm.475

Ana L Sousa, Sara Silva + Show 4 more

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https://doi.org/10.5334/tohm.475

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Abstract

Background: Chronic hepatic disease can present with extrapyramidal symptoms. We describe two cases that presented with highly unusual movement disorders: ballism and gait freezing. Case report: Patient 1 is a 42-year-old man with previous episodes of hepatic encephalopathy (HE) who presented with upper limb dystonia and generalized chorea that progressed to ballism. Patient 2 is a 55-year-old woman who presented with pronounced gait freezing. In both patients, features of HE and acquired hepatocerebral degeneration coexisted. They improved markedly, though transiently, with rifaximin. Discussion: Ammonia-reducing treatments should be considered in patients presenting with movement disorders due to chronic liver disease.

Highlights

Compromised liver function as a consequence of either acute hepatic failure or advanced chronic liver disease leads to insufficient detoxification of neurotoxic substances, which accumulate in the blood and are responsible for a broad range of neurological manifestations
We report two patients with chronic hepatic disease presenting with unusual movement disorders, in which features of both hepatic encephalopathy (HE) and acquired hepatocerebral degeneration (AHD) are present (Table 1)
Ammonia interferes in the communication between astrocytes and neurons, leading to widespread neurological manifestations.[1]

Summary

Introduction

Compromised liver function as a consequence of either acute hepatic failure or advanced chronic liver disease leads to insufficient detoxification of neurotoxic substances, which accumulate in the blood and are responsible for a broad range of neurological manifestations. These include hepatic encephalopathy (HE) and acquired hepatocerebral degeneration (AHD).[1] Clinical manifestations usually involve movement disorders and cognitive features. We present two cases of advanced liver disease with uncommon movement disorders (freezing of gait and ballismus) that reversed with rifaximin.

Results

Conclusion