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Metabolic Brain Disease

, Volume 29, Issue 1, pp 207–209 | Cite as

Parkinsonian syndrome and ataxia as a presenting finding of acquired hepatocerebral degeneration

  • Vladimir Miletić
  • David Ozretić
  • Maja Relja
Short Communication

Abstract

The term “acquired hepatocerebral degeneration” (AHD) was coined to describe clinical entity distinct from genetically defined Wilson’s disease. AHD is chronic neurological disorder, characterized by extrapyramidal and neuropsychiatric symptoms accompanied with advanced liver disease with portosystemic shunts. In majority of AHD cases, extrapyramidal symptoms appear in the presence of known liver disease. Here we present a patient with subacute onset of bilateral, asymmetric, hypokinetic-rigid syndrome and ataxia as initial presentation of liver cirrhosis. Manganese toxicity have major role in AHD pathogenesis. Failure of liver detoxification and presence of portosystemic shunting enables neurotoxic substance of manganese to avoid hepatic metabolism and to enter and accumulate in central nervous system. Predilection brain regions for manganese deposits are globus pallidum (GP) and substantia nigra (SN). Characteristic MRI findings of bilateral, symmetrical hyperintensities of GP and SN on T1-weighted sequences supported the diagnosis of AHD in our patient. In addition, increased T2 signal in dendate nuclei seen in our patient is rare radiological finding. So far, no consensus guidelines regarding medical treatment of AHD exist. We initiated low-dose levodopa treatment, but failed to provide beneficial effect. In conclusion, AHD is distinct clinical entity that should be included in differential diagnosis of both typical and atypical parkinsonian syndromes. Furthermore, our case highlights the importance of performing MRI in patients with atypical parkinsonism.

Keywords

Acquired hepatocerebral degeneration Parkinsonism in cirrhosis Magnetic resonance imaging Manganese 

Notes

Acknowledgments

Supported from Croatian Ministry of Science, Education and Sports

Conflict of interest

The authors declare that they have no conflict of interest

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of NeurologyUniversity of Zagreb, School of Medicine and University Hospital Centre ZagrebZagrebCroatia
  2. 2.Department of RadiologyUniversity of Zagreb, School of Medicine and University Hospital Centre ZagrebZagrebCroatia

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