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Regional variation in brain lactate in Leigh syndrome by localized 1H magnetic resonance spectroscopy. 1991

J A Detre, and Z Y Wang, and A R Bogdan, and D A Gusnard, and C A Bay, and P M Bingham, and R A Zimmerman
Department of Radiology, Children's Hospital of Philadelphia, PA.

Localized water-suppressed 1H magnetic resonance spectroscopy was performed in an 11-month-old infant with Leigh syndrome. Spectra obtained from the basal ganglia, occipital cortex, and brainstem showed elevations in lactate, which were most pronounced in regions where abnormalities were seen with routine T2-weighted magnetic resonance imaging. This approach has allowed us to examine metabolism in brain tissue directly and noninvasively, and may provide a sensitive means for evaluating metabolic disease and the response to therapy in the brain.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007773 Lactates Salts or esters of LACTIC ACID containing the general formula CH3CHOHCOOR.
D007888 Leigh Disease A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include X-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the PYRUVATE DEHYDROGENASE COMPLEX; CYTOCHROME-C OXIDASE; ATP synthase subunit 6; and subunits of mitochondrial complex I. (From Menkes, Textbook of Child Neurology, 5th ed, p850). Encephalomyelitis, Subacute Necrotizing,Encephalopathy, Subacute Necrotizing,Encephalomyelopathy, Subacute Necrotizing,Encephalopathy, Subacute Necrotizing, Infantile,Encephalopathy, Subacute Necrotizing, Juvenile,Infantile Leigh Disease,Infantile Subacute Necrotizing Encephalopathy,Juvenile Leigh Disease,Juvenile Subacute Necrotizing Encephalopathy,Leigh Disease, Infantile,Leigh Disease, Juvenile,Leigh Syndrome,Leigh's Disease,Subacute Necrotizing Encephalomyelitis, Infantile,Subacute Necrotizing Encephalomyelopathy,Subacute Necrotizing Encephalopathy,Subacute Necrotizing Encephalopathy, Infantile,Subacute Necrotizing Encephalopathy, Juvenile,Disease, Leigh's,Encephalomyelitides, Subacute Necrotizing,Encephalomyelopathies, Subacute Necrotizing,Encephalopathies, Subacute Necrotizing,Leighs Disease,Necrotizing Encephalomyelitides, Subacute,Necrotizing Encephalomyelitis, Subacute,Necrotizing Encephalomyelopathies, Subacute,Necrotizing Encephalomyelopathy, Subacute,Necrotizing Encephalopathies, Subacute,Necrotizing Encephalopathy, Subacute,Subacute Necrotizing Encephalomyelitides,Subacute Necrotizing Encephalomyelitis,Subacute Necrotizing Encephalomyelopathies,Subacute Necrotizing Encephalopathies
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D009682 Magnetic Resonance Spectroscopy Spectroscopic method of measuring the magnetic moment of elementary particles such as atomic nuclei, protons or electrons. It is employed in clinical applications such as NMR Tomography (MAGNETIC RESONANCE IMAGING). In Vivo NMR Spectroscopy,MR Spectroscopy,Magnetic Resonance,NMR Spectroscopy,NMR Spectroscopy, In Vivo,Nuclear Magnetic Resonance,Spectroscopy, Magnetic Resonance,Spectroscopy, NMR,Spectroscopy, Nuclear Magnetic Resonance,Magnetic Resonance Spectroscopies,Magnetic Resonance, Nuclear,NMR Spectroscopies,Resonance Spectroscopy, Magnetic,Resonance, Magnetic,Resonance, Nuclear Magnetic,Spectroscopies, NMR,Spectroscopy, MR
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006859 Hydrogen The first chemical element in the periodic table with atomic symbol H, and atomic number 1. Protium (atomic weight 1) is by far the most common hydrogen isotope. Hydrogen also exists as the stable isotope DEUTERIUM (atomic weight 2) and the radioactive isotope TRITIUM (atomic weight 3). Hydrogen forms into a diatomic molecule at room temperature and appears as a highly flammable colorless and odorless gas. Protium,Hydrogen-1
D019344 Lactic Acid A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed) Lactate,2-Hydroxypropanoic Acid,2-Hydroxypropionic Acid,Ammonium Lactate,D-Lactic Acid,L-Lactic Acid,Propanoic Acid, 2-Hydroxy-, (2R)-,Propanoic Acid, 2-Hydroxy-, (2S)-,Sarcolactic Acid,2 Hydroxypropanoic Acid,2 Hydroxypropionic Acid,D Lactic Acid,L Lactic Acid,Lactate, Ammonium

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