Magnetic resonance spectroscopy in patients with MELAS.
Localized magnetic resonance spectroscopy (MRS) yields sensitive metabolic markers to provide insight into the pathophysiology of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) in vivo. Findings in full MELAS syndrome at 1H MRS of the brain typically include severely elevated lactate and reduced N-acetylaspartate, glutamate, myo-inositol, and total creatine concentrations in stroke-like lesions. Similar but less extreme alterations are also common in gray matter (GM) regions that appear normal at magnetic resonance imaging. Phosphorus spectroscopy of peripheral muscle permits investigation of the bioenergetic status. A decline of the phosphorylation potential indicates a low energy reserve at rest. Phosphocreatine resynthesis during post-exercise recovery is delayed pointing to reduced mitochondrial capacity. As MRS is inherently non-invasive, follow-up studies can be performed to assess treatment response quantitatively.
Möller
Harald E
HE
Max Planck Institute for Human Cognitive and Brain Sciences, Stephanstrasse 1A, 04103 Leipzig, Germany. moeller@cbs.mpg.de
Kurlemann
Gerhard
G
Pützler
Manfred
M
Wiedermann
Dirk
D
Hilbich
Thorsten
T
Fiedler
Barbara
B
D000255:Adenosine Triphosphate / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D002648:Child; D015444:Exercise / Q000502:physiology; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000378:metabolism* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D018482:Muscle, Skeletal / Q000737:chemistry / Q000378:metabolism; D010725:Phosphocreatine / Q000378:metabolism; D012189:Retrospective Studies; D020521:Stroke / Q000473:pathology
D010725:Phosphocreatine; D000255:Adenosine Triphosphate