Imaging Neurodegenerative Metabolism in Amyotrophic Lateral Sclerosis with Hyperpolarized [1-13C]pyruvate MRI

Nikolaj Bøgh*, Christoffer Laustsen, Esben S. S. Hansen, Hatice Tankisi, Lotte B. Bertelsen, Jakob U. Blicher

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

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The cause of amyotrophic lateral sclerosis (ALS) is still unknown, and consequently, early diagnosis of the disease can be difficult and effective treatment is lacking. The pathology of ALS seems to involve specific disturbances in carbohydrate metabolism, which may be diagnostic and therapeutic targets. Magnetic resonance imaging (MRI) with hyperpolarized [1-13C]pyruvate is emerging as a technology for the evaluation of pathway-specific changes in the brain's metabolism. By imaging pyruvate and the lactate and bicarbonate it is metabolized into, the technology is sensitive to the metabolic changes of inflammation and mitochondrial dysfunction. In this study, we performed hyperpolarized MRI of a patient with newly diagnosed ALS. We found a lateralized difference in [1-13C]pyruvate-to-[1-13C]lactate exchange with no changes in exchange from [1-13C]pyruvate to 13C-bicarbonate. The 40% increase in [1-13C]pyruvate-to-[1-13C]lactate exchange corresponded with the patient's symptoms and presentation with upper-motor neuron affection and cortical hyperexcitability. The data presented here demonstrate the feasibility of performing hyperpolarized MRI in ALS. They indicate potential in pathway-specific imaging of dysfunctional carbohydrate metabolism in ALS, an enigmatic neurodegenerative disease.

Original languageEnglish
Issue number3
Pages (from-to)1570-1577
Number of pages8
Publication statusPublished - 14 Jun 2022


  • Amyotrophic Lateral Sclerosis/diagnostic imaging
  • Bicarbonates
  • Humans
  • Lactic Acid/metabolism
  • Magnetic Resonance Imaging/methods
  • Neurodegenerative Diseases
  • Pyruvic Acid/metabolism
  • magnetic resonance imaging
  • hyperpolarized
  • neurodegeneration
  • amyotrophic lateral sclerosis
  • pyruvate
  • metabolic


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