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Recent Updates on Acquired Hepatocerebral Degeneration
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 “acquire...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Columbia University Libraries/Information Services
2017
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5623760/ https://www.ncbi.nlm.nih.gov/pubmed/28975044 http://dx.doi.org/10.7916/D8TB1K44 |
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author | Shin, Hae-Won Park, Hee Kyung |
author_facet | Shin, Hae-Won Park, Hee Kyung |
author_sort | Shin, Hae-Won |
collection | PubMed |
description | 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.” RESULTS: Multiple mechanisms involving the accumulation of toxic substances such as ammonia or manganese and neuroinflammation may lead to widespread neurodegeneration in AHD. Clinical characteristics include movement disorders, mainly parkinsonism and ataxia-plus syndrome, as well as cognitive impairment with psychiatric features. Neuroimaging studies of AHD with parkinsonism show hyperintensity in the bilateral globus pallidus on T1-weighted magnetic resonance images, whereas molecular imaging of the presynaptic dopaminergic system shows variable findings. Ataxia-plus syndrome in AHD may demonstrate high-signal lesions in the middle cerebellar peduncles on T2-weighted images. DISCUSSION: Future studies are needed to elucidate the exact pathomechanism and neuroimaging findings of this heterogeneous syndrome. |
format | Online Article Text |
id | pubmed-5623760 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Columbia University Libraries/Information Services |
record_format | MEDLINE/PubMed |
spelling | pubmed-56237602017-10-03 Recent Updates on Acquired Hepatocerebral Degeneration Shin, Hae-Won Park, Hee Kyung Tremor Other Hyperkinet Mov (N Y) Reviews 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.” RESULTS: Multiple mechanisms involving the accumulation of toxic substances such as ammonia or manganese and neuroinflammation may lead to widespread neurodegeneration in AHD. Clinical characteristics include movement disorders, mainly parkinsonism and ataxia-plus syndrome, as well as cognitive impairment with psychiatric features. Neuroimaging studies of AHD with parkinsonism show hyperintensity in the bilateral globus pallidus on T1-weighted magnetic resonance images, whereas molecular imaging of the presynaptic dopaminergic system shows variable findings. Ataxia-plus syndrome in AHD may demonstrate high-signal lesions in the middle cerebellar peduncles on T2-weighted images. DISCUSSION: Future studies are needed to elucidate the exact pathomechanism and neuroimaging findings of this heterogeneous syndrome. Columbia University Libraries/Information Services 2017-09-05 /pmc/articles/PMC5623760/ /pubmed/28975044 http://dx.doi.org/10.7916/D8TB1K44 Text en © 2017 Shin et al. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommerical–No Derivatives License, which permits the user to copy, distribute, and transmit the work provided that the original author and source are credited; that no commercial use is made of the work; and that the work is not altered or transformed. |
spellingShingle | Reviews Shin, Hae-Won Park, Hee Kyung Recent Updates on Acquired Hepatocerebral Degeneration |
title | Recent Updates on Acquired Hepatocerebral Degeneration |
title_full | Recent Updates on Acquired Hepatocerebral Degeneration |
title_fullStr | Recent Updates on Acquired Hepatocerebral Degeneration |
title_full_unstemmed | Recent Updates on Acquired Hepatocerebral Degeneration |
title_short | Recent Updates on Acquired Hepatocerebral Degeneration |
title_sort | recent updates on acquired hepatocerebral degeneration |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5623760/ https://www.ncbi.nlm.nih.gov/pubmed/28975044 http://dx.doi.org/10.7916/D8TB1K44 |
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