Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Sep 5:7:463.
doi: 10.7916/D8TB1K44. eCollection 2017.

Recent Updates on Acquired Hepatocerebral Degeneration

Hae-Won Shin  1   2 , Hee Kyung Park  3   4
Affiliations
Review

Recent Updates on Acquired Hepatocerebral Degeneration

Hae-Won Shin et al. Tremor Other Hyperkinet Mov (N Y). .

Abstract

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.

Keywords: Acquired hepatocerebral degeneration; dopamine transporter imaging; liver cirrhosis; manganese; neuroinflammation; parkinsonism.

PubMed Disclaimer

Conflict of interest statement

Funding: This study was supported by a grant from Research year of Inje University in 20150606. Conflicts of interest: The authors report no conflict of interest. Ethics Statement: All patients that appear on video have provided written informed consent; authorization for the videotaping and publication of the videotape was provided.

Figures

Figure 1
Figure 1. Pathology of AHD. Alzheimer type II astrocyte showing large pale nuclei with basophilic nuclei. Reproduced with permission from Ferrara et al. AHD, Acquired Hepatocerebral Degeneration.
Figure 2
Figure 2. The Putative Pathomechanism in AHD. The exact pathomechanism in AHD remains unclear, but the putative pathomechanism includes complex actions between toxic substance accumulation, neuroinflammation, oxidative stress, and inducible nitric oxide (nitrosative stress). AHD, Acquired Hepatocerebral Degeneration.
Figure 3
Figure 3. Neuroimaging Studies in AHD with Parkinsonism. (A) Brain MRI in AHD. High signal intensities in the bilateral globus pallidus on T1-weighted images, (B-E) (18F FP-CIT PET findings. (B) Normal controls; (C) AHD patients with parkinsonism; (D) AHD patients with parkinsonism; (E) Idiopathic Parkinson diseases. AHD, Acquired Hepatocerebral Degeneration; 18F FP-CIT PET, 18F-N-3-Fluoropropyl-2β-Carboxymethoxy-3β-(4-iodophenyl)-Nortropane Positron Emission Tomography; MRI, Magnetic Resonance Imaging.
Figure 4
Figure 4. Brain MRI findings in AHD with Ataxia-Plus Syndrome. (A) High signal lesion on T2-weighted image and (B) low signal lesion on T1-weighted image in the middle cerebellar peduncles Reproduced with permission from Ishii K et al. AHD, Acquired Hepatocerebral Degeneration; MRI, Magnetic Resonance Imaging.
See this image and copyright information in PMC

References

    1. Victor M, Adams RD, Cole M. The acquired (non-Wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 1965;44:345–396. doi: http://dx.doi.org/10.1097/00005792-196509000-00001. - DOI - PubMed
    1. Pinarbasi B, Kalymakoglu S, Matur Z, Akyuz F, Demir K, Besisik F, et al. Are acquired hepatocerebral degeneration and hepatic myelopathy reversible? J Clin Gastroenterol. 2009;43:176–181. doi: http://dx.doi.org/10.1097/MCG.0b013e318150d399. - DOI - PubMed
    1. Fernandez-Rodriguez R, Contreras A, de Villoria JG, Grandas F. Acquired hepatocerebral degeneration: clinical characteristics and MRI findings. Eur J Neurol. 2010;17:1463–1470. doi: http://dx.doi.org/10.1111/j.1468-1331.2010.03076.x. - DOI - PubMed
    1. Qavi AH, Hammad S, Rana AI, Salih M, Shah NH, Dar FS, et al. Reversal of acquired hepatocerebral degeneration with living donor liver transplantation. Liver Transpl. 2016;22:125–129. doi: http://dx.doi.org/10.1002/lt.24306. - DOI - PubMed
    1. Burkhard PR, Delavelle J, Du Pasquier R, Spahr L. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol. 2003;60:521–528. doi: http://dx.doi.org/10.1001/archneur.60.4.521. - DOI - PubMed

MeSH terms