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Abstract Details

Elevated Cerebral Lactate Levels on Multi-Voxel Proton MR Spectroscopy in Metachromatic Leukodystrophy
Child Neurology/Developmental Neurobiology
P03 - (-)
016
BACKGROUND: 1H-MRS allows for studying selected brain metabolites including N-acetyl aspartate (NAA), Creatine (Cr), myoinositol (mI), Choline (Cho) and lactate (Lac). While NAA is widely accepted as a neuronal marker, mI is thought to represent the activity of the glial cells. Glucose crosses the blood-brain barrier and enters the astrocytes to produce lactate via glycolysis. Lactate is then transported to the neurons where it enters the Krebs cycle in order to generate ATP. Cerebral Lac level has not been studied in the past in MLD.
DESIGN/METHODS: Four patients with clinical, laboratory and genetic confirmation of MLD underwent long echo-time multi-voxel 1H-MRS of brain. Studies were conducted with a Siemens Magnetom Avanto 1.5 Tesla MRI system. Consecutive areas of interest in the bilateral basal ganglia, thalami, white matter, parts of the insular and occipital cortices were sampled.
RESULTS: We documented a significant drop in the NAA/Cr ratio and diffuse elevation in the Cho/Cr and mI/Cr ratios. Also, elevation of Lac /Cr ratio was noted in the peri-ventricular white matter.
CONCLUSIONS: The drop in the NAA/Cr ratio is related to diffuse neuronal loss and therefore, not unexpected in MLD. On the other hand, the elevation of Lac/Cr ratio in the peri-ventricular white matter is a novel finding. We propose that this may be the result of astrocytic gliosis causing over-production of lactate and neuronal loss resulting in reduced utilization of lactate. Both abnormalities of elevated Lac/Cr and mI/Cr ratios in our cohort are attributable to the astrocytic gliosis characteristic of MLD. We propose that elevated Lac/Cr and mI/Cr ratios on brain 1H-MRS may be used as markers of disease progression in MLD.
Authors/Disclosures
Yadira Velazquez-Rodriguez, MD
PRESENTER 		No disclosure on file
Mitra Assadi Khansare, MD, FAAN (Christiana Care) No disclosure on file
No disclosure on file
Howard L. Weiner, MD (Brigham and Women'S Hospital) Dr. Weiner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Weiner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medday Pharmaceuticals. Dr. Weiner has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for vTv Therapeutics. Dr. Weiner has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Tiziana Life Sciences. Dr. Weiner has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for vTv Therapeutics. Dr. Weiner has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Medday Pharmaceuticals. Dr. Weiner has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for vTv Therapeutics. Dr. Weiner has stock in vTv Therapeutics. The institution of Dr. Weiner has received research support from National Institute of Health. The institution of Dr. Weiner has received research support from National MS Society. The institution of Dr. Weiner has received research support from Genzyme Corp. The institution of Dr. Weiner has received research support from Genentech, Inc. . The institution of Dr. Weiner has received research support from Verily Life Sciences LLC. The institution of Dr. Weiner has received research support from EMD Serono, Inc..