Page last updated: 2024-10-17

lactic acid and Encephalomyopathies, Mitochondrial

lactic acid has been researched along with Encephalomyopathies, Mitochondrial in 32 studies

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)
2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.

Research Excerpts

ExcerptRelevanceReference
"The purpose of this research was to report results on long-term administration of dichloroacetate in 36 children with congenital lactic acidosis who participated previously in a controlled trial of this drug."9.13Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate. ( Carney, PR; Gilbert, LR; Neiberger, RE; Shuster, JJ; Stacpoole, PW; Theriaque, DW; Valenstein, E, 2008)
"The purpose of this research was to report results on long-term administration of dichloroacetate in 36 children with congenital lactic acidosis who participated previously in a controlled trial of this drug."5.13Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate. ( Carney, PR; Gilbert, LR; Neiberger, RE; Shuster, JJ; Stacpoole, PW; Theriaque, DW; Valenstein, E, 2008)
" Among them, there were 19 cases of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS), 2 cases of encephalopathies which could not be classified into any specific type, 2 cases of floppy infants, one case of Kearns-Sayer syndrome (KSS) and one case of mitochondrial entero-myopathy."3.73[Phenotype heterogeneity associated with mitochondrial DNA A3243G mutation]. ( Niu, SL; Pei, P; Qi, Y; Wang, ZX; Xu, YF; Yang, YL; Yuan, Y; Zhang, Y, 2005)
"Characteristics of myoclonic epilepsy associated with ragged-red fibers include myoclonic epilepsy, generalized epilepsy, hearing loss, exercise intolerance, lactic acidosis, and ragged-red fibers."1.35Myoclonic epilepsy with ragged-red fibers without increased lactate levels. ( Kimura, S; Kosuge, H; Nakamura, K; Nomura, K; Ozasa, S, 2009)
"Five females with mitochondrial encephalomyopathies were treated for 3 to 7 years with a xanthine oxidase inhibitor (allopurinol, oral route, 20 mg/kg/day, in 2 or 3 doses daily)."1.33Treatment of mitochondrial encephalomyopathies with a xanthine oxidase inhibitor. ( Blanco-Barca, MO; Camiña, F; Castro-Gago, M; Eirís-Puñal, J; Gómez-Lado, C; Novo-Rodríguez, MI; Rodríguez-Segade, S, 2006)
"Mitochondrial diseases are a group of inherited disorders caused by a derangement of mitochondrial respiration."1.32Proton MR spectroscopy in the diagnostic evaluation of suspected mitochondrial disease. ( Barker, PB; Crawford, TO; Lin, DD, 2003)
"The mitochondrial encephalomyopathies are chronic progressive disorders affecting predominantly the neuromuscular system."1.32Effectiveness of creatine monohydrate in mitochondrial encephalomyopathies. ( Hanefeld, FA; Hobbiebrunken, E; Komura, K; Wilichowski, EK, 2003)

Research

Studies (32)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's15 (46.88)18.2507
2000's13 (40.63)29.6817
2010's2 (6.25)24.3611
2020's2 (6.25)2.80

Authors

AuthorsStudies
Baheerathan, A1
Pitceathly, RD1
Curtis, C1
Davies, NW1
Li, X1
Peng, B1
Hou, C1
Li, J1
Zeng, Y1
Wu, W1
Liao, Y1
Tian, Y1
Chen, WX1
Toi, T1
Nomura, Y1
Kishino, A1
Shigihara, S1
Oshima, T1
Ishikawa, H1
Kamei, S1
Miyazaki, H1
Nogueira, C1
Marques, JS1
Nesti, C1
Azevedo, L1
Di Lullo, M1
Meschini, MC1
Orlacchio, A1
Santorelli, FM1
Vilarinho, L1
Kimura, S1
Ozasa, S1
Nakamura, K1
Nomura, K1
Kosuge, H1
Schulte-Mattler, WJ1
Müller, T1
Deschauer, M1
Gellerich, FN1
Iaizzo, PA1
Zierz, S1
Lin, DD1
Crawford, TO1
Barker, PB1
Komura, K1
Hobbiebrunken, E1
Wilichowski, EK1
Hanefeld, FA1
Zhang, Y1
Wang, ZX1
Niu, SL1
Xu, YF1
Pei, P1
Yuan, Y1
Yang, YL1
Qi, Y1
Sperl, W1
Jesina, P1
Zeman, J1
Mayr, JA1
Demeirleir, L1
VanCoster, R1
Pícková, A1
Hansíková, H1
Houst'ková, H1
Krejcík, Z1
Koch, J1
Smet, J1
Muss, W1
Holme, E1
Houstek, J1
Castro-Gago, M2
Blanco-Barca, MO1
Novo-Rodríguez, MI2
Gómez-Lado, C1
Camiña, F1
Eirís-Puñal, J2
Rodríguez-Segade, S1
Walia, A1
Thapa, BR1
Kim, V1
Bianchi, MC1
Sgandurra, G1
Tosetti, M1
Battini, R1
Cioni, G1
José da Rocha, A1
Túlio Braga, F1
Carlos Martins Maia, A1
Jorge da Silva, C1
Toyama, C1
Pereira Pinto Gama, H1
Kok, F1
Rodrigues Gomes, H1
Stacpoole, PW1
Gilbert, LR1
Neiberger, RE1
Carney, PR1
Valenstein, E1
Theriaque, DW1
Shuster, JJ1
Jackson, MJ1
Schaefer, JA1
Johnson, MA1
Morris, AA1
Turnbull, DM1
Bindoff, LA1
Agsteribbe, E1
Huckriede, A1
Veenhuis, M1
Ruiters, MH1
Niezen-Koning, KE1
Skjeldal, OH1
Skullerud, K1
Gupta, RS1
Hallberg, R1
van Diggelen, OP1
Ozawa, M1
Goto, Y1
Sakuta, R1
Tanno, Y1
Tsuji, S1
Nonaka, I1
Lincke, CR1
van den Bogert, C1
Nijtmans, LG1
Wanders, RJ2
Tamminga, P1
Barth, PG1
Chen, RS1
Huang, CC1
Chu, NS1
Dionisi-Vici, C1
Ruitenbeek, W1
Fariello, G1
Bentlage, H1
Schägger, H1
Bosman, C1
Piantadosi, C1
Sabetta, G1
Bertini, E1
Schmidt, M1
Kunkel, M1
Schuff-Werner, P1
Naumann, M1
Reichmann, H1
Reimers, CD1
Bowen, J1
Richards, T1
Maravilla, K1
Argov, Z1
Boitier, E1
Degoul, F1
Desguerre, I1
Charpentier, C1
François, D1
Ponsot, G1
Diry, M1
Rustin, P1
Marsac, C1
Haas, RH1
Barshop, BA1
Artuch, R1
Vilaseca, MA1
Pineda, M1
Sperfeld, A1
Vietzke, G1
Kleber, FX1
Ludolph, AC1
DiMauro, S1
Bonilla, E1
De Vivo, DC1
Siciliano, G1
Mancuso, M1
Pasquali, L1
Manca, ML1
Tessa, A1
Iudice, A1
Imataka, G1
Yamanouchi, H1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
In Vivo Investigation on Mitochondrial Dysfunction in Post-COVID Fatigue and Cancer Fatigue.[NCT05753228]90 participants (Anticipated)Interventional2022-12-09Recruiting
Phase 3 Trial of Dichloroacetate in Pyruvate Dehydrogenase Complex Deficiency:[NCT02616484]Phase 334 participants (Actual)Interventional2020-07-14Active, not recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

6 reviews available for lactic acid and Encephalomyopathies, Mitochondrial

ArticleYear
CSF lactate.
    Practical neurology, 2020, Volume: 20, Issue:4

    Topics: Adult; Biomarkers; Brain Diseases; Central Nervous System Infections; Enterovirus; Female; Humans; L

2020
Brain magnetic resonance in the diagnostic evaluation of mitochondrial encephalopathies.
    Bioscience reports, 2007, Volume: 27, Issue:1-3

    Topics: Brain; Humans; Lactic Acid; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Mitochondri

2007
Lactate detection by MRS in mitochondrial encephalopathy: optimization of technical parameters.
    Journal of neuroimaging : official journal of the American Society of Neuroimaging, 2008, Volume: 18, Issue:1

    Topics: Brain Diseases, Metabolic, Inborn; Humans; Lactic Acid; Magnetic Resonance Spectroscopy; Mitochondri

2008
Functional evaluation techniques in mitochondrial disorders.
    European neurology, 1998, Volume: 39, Issue:2

    Topics: Evaluation Studies as Topic; Exercise Test; Humans; Lactic Acid; Magnetic Resonance Spectroscopy; Mi

1998
[Mitochondrial encephalomyopathies starting in childhood and adolescence].
    Revista de neurologia, 1998, Volume: 26 Suppl 1

    Topics: Adolescent; Child; DNA, Mitochondrial; Humans; Infant; Lactic Acid; Male; Mitochondrial Encephalomyo

1998
[Coenzyme Q10 deficiency].
    Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4

    Topics: Coenzymes; Diagnosis, Differential; Humans; Lactic Acid; Mitochondria; Mitochondrial Diseases; Mitoc

2002

Trials

2 trials available for lactic acid and Encephalomyopathies, Mitochondrial

ArticleYear
Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate.
    Pediatrics, 2008, Volume: 121, Issue:5

    Topics: Acidosis, Lactic; Administration, Oral; Adolescent; Adult; Child; Child, Preschool; Dichloroacetic A

2008
Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study.
    European neurology, 1997, Volume: 37, Issue:4

    Topics: Activities of Daily Living; Adolescent; Adult; Aged; Coenzymes; Cross-Over Studies; Double-Blind Met

1997

Other Studies

24 other studies available for lactic acid and Encephalomyopathies, Mitochondrial

ArticleYear
Novel compound heterozygous TARS2 variants in a Chinese family with mitochondrial encephalomyopathy: a case report.
    BMC medical genetics, 2020, 11-05, Volume: 21, Issue:1

    Topics: Asian People; Developmental Disabilities; Epilepsy; Family; Gene Expression; Heterozygote; High-Thro

2020
Repeated Attacks of Dizziness Caused by a Rare Mitochondrial Encephalomyopathy.
    The journal of international advanced otology, 2018, Volume: 14, Issue:1

    Topics: Administration, Oral; Adult; Biopsy; Caloric Tests; Diagnosis, Differential; Dizziness; Female; Huma

2018
Identification of maternal uniparental isodisomy of chromosome 10 in a patient with mitochondrial DNA depletion syndrome.
    Molecular genetics and metabolism, 2013, Volume: 110, Issue:4

    Topics: Brain; Chromosome Aberrations; Chromosomes, Human, Pair 10; DNA, Mitochondrial; Humans; Intestinal P

2013
Myoclonic epilepsy with ragged-red fibers without increased lactate levels.
    Pediatric neurology, 2009, Volume: 41, Issue:1

    Topics: Adolescent; Anticonvulsants; Diagnosis, Differential; DNA Mutational Analysis; DNA, Mitochondrial; E

2009
Increased metabolic muscle fatigue is caused by some but not all mitochondrial mutations.
    Archives of neurology, 2003, Volume: 60, Issue:1

    Topics: Action Potentials; Adolescent; Adult; Enzymes; Female; Humans; Lactic Acid; Male; Middle Aged; Mitoc

2003
Proton MR spectroscopy in the diagnostic evaluation of suspected mitochondrial disease.
    AJNR. American journal of neuroradiology, 2003, Volume: 24, Issue:1

    Topics: Adolescent; Adult; Brain; Brain Diseases, Metabolic, Inborn; Child; Child, Preschool; Female; Fourie

2003
Effectiveness of creatine monohydrate in mitochondrial encephalomyopathies.
    Pediatric neurology, 2003, Volume: 28, Issue:1

    Topics: Adolescent; Adult; Child; Creatine; Exercise Test; Female; Humans; Lactic Acid; Male; MELAS Syndrome

2003
[Phenotype heterogeneity associated with mitochondrial DNA A3243G mutation].
    Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae, 2005, Volume: 27, Issue:1

    Topics: Adolescent; Adult; Child; Child, Preschool; DNA, Mitochondrial; Female; Humans; Infant; Kearns-Sayre

2005
Deficiency of mitochondrial ATP synthase of nuclear genetic origin.
    Neuromuscular disorders : NMD, 2006, Volume: 16, Issue:12

    Topics: Adenosine Triphosphate; Adolescent; Age of Onset; Cardiomyopathy, Hypertrophic, Familial; Cell Nucle

2006
Treatment of mitochondrial encephalomyopathies with a xanthine oxidase inhibitor.
    Pediatric neurology, 2006, Volume: 35, Issue:5

    Topics: Adenine Nucleotides; Allopurinol; Child; Child, Preschool; Drug Administration Schedule; Energy Meta

2006
Mitochondrial neuro-gastrointestinal encephalopathy syndrome.
    Indian journal of pediatrics, 2006, Volume: 73, Issue:12

    Topics: Biopsy; Child; Diagnosis, Differential; Gastrointestinal Diseases; Humans; Intestinal Pseudo-Obstruc

2006
Presentation and clinical investigation of mitochondrial respiratory chain disease. A study of 51 patients.
    Brain : a journal of neurology, 1995, Volume: 118 ( Pt 2)

    Topics: Adolescent; Adult; Alanine; Brain; Child; Creatine Kinase; DNA, Mitochondrial; Electroencephalograph

1995
A fatal, systemic mitochondrial disease with decreased mitochondrial enzyme activities, abnormal ultrastructure of the mitochondria and deficiency of heat shock protein 60.
    Biochemical and biophysical research communications, 1993, May-28, Volume: 193, Issue:1

    Topics: Cells, Cultured; Female; Fibroblasts; Glucose; Heat-Shock Proteins; Humans; Infant, Newborn; Lactate

1993
The 8,344 mutation in mitochondrial DNA: a comparison between the proportion of mutant DNA and clinico-pathologic findings.
    Neuromuscular disorders : NMD, 1995, Volume: 5, Issue:6

    Topics: Adolescent; Adult; Base Sequence; Child; Child, Preschool; DNA Mutational Analysis; DNA, Mitochondri

1995
Cerebellar hypoplasia in respiratory chain dysfunction.
    Neuropediatrics, 1996, Volume: 27, Issue:4

    Topics: Cerebellum; Consanguinity; Cytochrome-c Oxidase Deficiency; Fatal Outcome; Female; Humans; Infant, N

1996
New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency.
    Annals of neurology, 1997, Volume: 42, Issue:4

    Topics: Cardiomyopathies; Electrophoresis, Gel, Two-Dimensional; Family Health; Fatal Outcome; Fibroblasts;

1997
[Standardized aerobic walking on the treadmill by healthy probands and patients with mitochondrial and non-mitochondrial myopathies].
    Der Nervenarzt, 1997, Volume: 68, Issue:10

    Topics: Adult; Energy Metabolism; Exercise Test; Female; Humans; Lactic Acid; Male; Middle Aged; Mitochondri

1997
MR imaging and proton MR spectroscopy in A-to-G substitution at nucleotide position 3243 of leucine transfer RNA.
    AJNR. American journal of neuroradiology, 1998, Volume: 19, Issue:2

    Topics: Adult; Amino Acid Substitution; Aspartic Acid; Basal Ganglia; Base Sequence; Brain; Cerebral Cortex;

1998
A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency.
    Journal of the neurological sciences, 1998, Volume: 156, Issue:1

    Topics: Cerebellar Ataxia; Child, Preschool; Coenzymes; Electron Transport; Epilepsy; Humans; Kinetics; Lact

1998
Diet change in the management of metabolic encephalomyopathies.
    BioFactors (Oxford, England), 1998, Volume: 7, Issue:3

    Topics: Adolescent; Adult; Child; Dietary Carbohydrates; Fasting; Fructose; Glucose Tolerance Test; Humans;

1998
Biochemical monitoring of the treatment in paediatric patients with mitochondrial disease.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:8

    Topics: Adolescent; Alanine; Ascorbic Acid; Carnitine; Child; Child, Preschool; Erythrocytes; Female; Humans

1998
[Spiroergometry in diagnosis of mitochondrial diseases].
    Der Nervenarzt, 1999, Volume: 70, Issue:2

    Topics: Adolescent; Adult; Anaerobic Threshold; Diagnosis, Differential; Exercise Test; Female; Humans; Lact

1999
Does the patient have a mitochondrial encephalomyopathy?
    Journal of child neurology, 1999, Volume: 14 Suppl 1

    Topics: Diagnosis, Differential; DNA Mutational Analysis; DNA, Mitochondrial; Electron Transport; Humans; La

1999
Abnormal levels of human mitochondrial transcription factor A in skeletal muscle in mitochondrial encephalomyopathies.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2000, Volume: 21, Issue:5 Suppl

    Topics: Adult; Aged; DNA Mutational Analysis; DNA-Binding Proteins; DNA, Mitochondrial; Energy Metabolism; F

2000