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Elevated manganese levels in blood and CNS in human prion disease

Shirley Hesketh1, Judyth Sassoon, Robert Knight

  • 1Department of Biology and Biochemistry, University of Bath, Bath, BA2 7AY, UK.

Molecular and Cellular Neurosciences
|February 1, 2008
PubMed

Insights

Elevated manganese and copper in the blood may help diagnose Creutzfeldt-Jakob disease (CJD), a fatal neurodegenerative prion disease. This finding offers a specific biomarker for CJD, distinguishing it from other neurological conditions.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Pathology

Background:

  • Prion diseases, or transmissible spongiform encephalopathies, are fatal neurodegenerative disorders.
  • Previous research suggests a link between prion diseases and elevated manganese levels in the brain and blood.

Purpose of the Study:

  • To investigate if elevated manganese is a specific biomarker for Creutzfeldt-Jakob disease (CJD), the most common human prion disease.
  • To differentiate manganese levels in CJD patients from those in healthy individuals and patients with other neurological disorders.

Main Methods:

  • Analysis of manganese concentrations in blood and brain samples from human subjects.
  • Comparison of manganese levels in CJD patients, individuals with other neurodegenerative diseases, and healthy controls.
  • Evaluation of copper levels in conjunction with manganese.

Main Results:

  • Blood manganese concentrations showed minimal variation with age or sex in normal individuals.
  • Creutzfeldt-Jakob disease (CJD) uniquely exhibited elevated levels of both manganese and copper compared to other neurodegenerative diseases.
  • While other conditions like blood-brain barrier disorders and haemochromatosis also showed elevated manganese, CJD could be readily distinguished.

Conclusions:

  • Increased blood manganese is a highly specific characteristic of prion disease, particularly CJD.
  • Manganese and copper elevation may serve as a specific diagnostic marker for CJD.
  • This finding aids in distinguishing CJD from other neurological and systemic diseases.

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