Metabolic Brain Disease

, Volume 10, Issue 4, pp 259–267 | Cite as

Manganese toxicity, dopaminergic dysfunction and hepatic encephalopathy

  • Roger F. Butterworth
  • Laurent Spahr
  • Suzanne Fontaine
  • Gilles Pomier Layrargues
Review Article


Patients with chronic liver disease manifest a high incidence (>75%) of pallidal signal hyperintensity on T1-weighted Magnetic Resonance Imaging (MRI), the intensity of which correlates with blood manganese levels and the presence of extrapyramidal symptoms. A major cause of pallidal hyperintensity on T1-weighted MRI is manganese deposition; chronic manganese intoxication in the absence of liver disease results in pallidal MR signal hyperintensity, in extrapyramidal symptoms and in selective effects on the dopaminergic neurotransmitter system in basal ganglia. Direct measurements in globus pallidus obtained at autopsy from patients with chronic liver disease who died in hepatic coma reveal 2 to 7-fold increases of pallidal manganese and a concomitant loss of dopamine D2 binding sites. Liver transplantation results in normalization of pallidal MR signals and of blood manganese levels. These findings suggest that (1) pallidal MR signal hyperintensity in patients with chronic liver disease is the result of manganese deposition and (2) alterations of dopaminergic function due to the toxic effects of manganese may contribute to the extrapyramidal symptoms in these patients.

Key words

manganese hepatic encephalopathy MR signal hyperintensity globus pallidus dopamine D2 receptors MAO extrapyramidal symptoms 


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Copyright information

© Plenum Publishing Corporation 1995

Authors and Affiliations

  • Roger F. Butterworth
    • 1
  • Laurent Spahr
    • 2
  • Suzanne Fontaine
    • 3
  • Gilles Pomier Layrargues
    • 2
  1. 1.Neuroscience Research UnitHôpital Saint-Luc (University of Montreal)MontrealCanada
  2. 2.Liver UnitHôpital Saint-Luc (University of Montreal)Montreal
  3. 3.Radiology DepartmentHôpital Saint-Luc (University of Montreal)Montreal

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