Neurochemistry of Hypomyelination Investigated with MR Spectroscopy.

Abstract

Proton magnetic resonance spectroscopy (MRS) allows the noninvasive exploration of tissue metabolism in vivo, providing neurophysiological and neurochemical information. N-acetylaspartate (NAA) is generally considered to be a marker of neurons and axons, and many neurodegenerative disorders, including demyelinating disorders, exhibit a decrease in total NAA (tNAA). MRS in human hypomyelination disorders, such as Pelizaeus-Merzbacher disease (PMD), is characterized by normal to elevated tNAA, elevated myo-inositol and creatine (Cr), and normal to decreased choline (Cho). MRS in the thalamus of a hypomyelinating mouse model, a myelin synthesis-deficient (msd) mouse, a model of connatal PMD with mutation of the Plp1 gene, revealed increased tNAA and Cr and decreased Cho. That of a shiverer mouse with an autosomal recessive mutation of the Mbp gene showed decreased Cho with normal tNAA and Cr. Accordingly, the reduction of Cho on MRS might be a common marker for hypomyelinating disorders. tNAA concentrations range from normal to increased, probably depending upon the underlying pathology of oligodendrocytes. tNAA may be increased in hypomyelination with a reduced number of mature oligodendrocytes, such as PMD.

• Authors: Jun-ichi Takanashi
• Journal: Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine (Magn Reson Med Sci) Vol. 14 Issue 2 Pg. 85-91 ( 2015) ISSN: 1880-2206 [Electronic] Japan
• PMID: 26185828 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Aspartic Acid
• N-acetylaspartate
• Creatine
• Choline

Topics

• Animals
• Aspartic Acid (analogs & derivatives, analysis)
• Brain Chemistry
• Choline (analysis)
• Creatine (analysis)
• Demyelinating Diseases (diagnosis)
• Disease Models, Animal
• Humans
• Mice
• Pelizaeus-Merzbacher Disease (diagnosis)
• Proton Magnetic Resonance Spectroscopy (methods)