Page last updated: 2024-10-17

lactic acid and Amyotonia Congenita

lactic acid has been researched along with Amyotonia Congenita in 28 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
"Sodium dichloroacetate (DCA) was administered orally at a dose of 50 mg per kg body weight twice or three times per day to a newborn infant with lactic acidosis of unknown cause (patient 1) and to a 15-year-old boy with mitochondrial encephalomyopathy associated with lactic acidosis (patient 2)."7.67Treatment of chronic congenital lactic acidosis by oral administration of dichloroacetate. ( Hashimoto, T; Hwang, TJ; Ito, M; Kuroda, Y; Masuda, M; Miyao, M; Naito, E; Takeda, E; Toshima, K; Yamashita, K, 1986)
"We report a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes who experienced visual and auditory hallucinations."3.68Therapeutic effect of sodium dichloroacetate on visual and auditory hallucinations in a patient with MELAS. ( Hashimoto, T; Ito, M; Kuroda, Y; Naito, E; Saijo, T; Takeda, E, 1991)
"Sodium dichloroacetate (DCA) was administered orally at a dose of 50 mg per kg body weight twice or three times per day to a newborn infant with lactic acidosis of unknown cause (patient 1) and to a 15-year-old boy with mitochondrial encephalomyopathy associated with lactic acidosis (patient 2)."3.67Treatment of chronic congenital lactic acidosis by oral administration of dichloroacetate. ( Hashimoto, T; Hwang, TJ; Ito, M; Kuroda, Y; Masuda, M; Miyao, M; Naito, E; Takeda, E; Toshima, K; Yamashita, K, 1986)
"Nine patients with mitochondrial myopathy, 10 patients with other neuromuscular diseases and 9 healthy but sedentary volunteers undertook the test."1.32Modified exercise test in screening for mitochondrial myopathies--adjustment of workload in relation to muscle strength. ( Hammarén, E; Kreuter, M; Lindberg, C; Rafsten, L, 2004)

Research

Studies (28)

TimeframeStudies, this research(%)All Research%
pre-199013 (46.43)18.7374
1990's7 (25.00)18.2507
2000's6 (21.43)29.6817
2010's2 (7.14)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Massimino, ML1
Peggion, C1
Loro, F1
Stella, R1
Megighian, A1
Scorzeto, M1
Blaauw, B1
Toniolo, L1
Sorgato, MC1
Reggiani, C1
Bertoli, A1
Laurin, J1
Dousset, E1
Mesure, S1
Decherchi, P1
Jakkani, R1
Jyoti, S1
Ahmed, M1
Thomas, MM1
Schulte-Mattler, WJ1
Müller, T1
Deschauer, M1
Gellerich, FN1
Iaizzo, PA1
Zierz, S3
Tarnopolsky, M1
Stevens, L1
MacDonald, JR1
Rodriguez, C1
Mahoney, D1
Rush, J1
Maguire, J1
Hammarén, E1
Rafsten, L1
Kreuter, M1
Lindberg, C1
Lössner, J1
Lehmann, W1
Kühn, HJ1
Ziegan, J1
Brehme, M1
LoPachin, RM1
Moore, RW1
Menahan, LA1
Peterson, RE1
Gabreëls, FJ2
Prick, MJ1
Trijbels, JM1
Renier, WO2
Jaspar, HH1
Janssen, AJ1
Slooff, JL1
Hanefeld, F1
Holzbach, U1
Kruse, B1
Wilichowski, E1
Christen, HJ1
Frahm, J1
Schmidt, M1
Kunkel, M1
Schuff-Werner, P1
Naumann, M1
Reichmann, H1
Reimers, CD2
Siciliano, G1
Pastorini, E1
Pasquali, L1
Manca, ML1
Iudice, A1
Murri, L1
Prelle, A1
Tancredi, L1
Sciacco, M1
Chiveri, L1
Comi, GP1
Battistel, A1
Bazzi, P1
Martinelli Boneschi, F1
Bagnardi, V1
Ciscato, P1
Bordoni, A1
Fortunato, F1
Strazzer, S1
Bresolin, N1
Scarlato, G1
Moggio, M1
Beyenburg, S1
von Wersebe, O1
Abe, K1
Fujimura, H1
Nishikawa, Y1
Yorifuji, S1
Mezaki, T1
Hirono, N1
Nishitani, N1
Kameyama, M1
van Hellenberg Hubar, JL1
Ruitenbeek, W1
Sengers, RC1
Thijssen, HO1
ter Laak, HJ1
Saijo, T1
Naito, E2
Ito, M2
Takeda, E2
Hashimoto, T2
Kuroda, Y2
Torbergsen, T1
Aasly, J1
Borud, O1
Lindal, S1
Mellgren, SI1
Mizuno, Y1
Ishiguro, K1
Okamoto, K2
Morimatsu, M1
Hirai, S2
Meessen, S1
Jerusalem, F1
Harigaya, Y1
Shoji, M1
Sato, T1
Toshima, K1
Hwang, TJ1
Miyao, M1
Masuda, M1
Yamashita, K1
Kobayashi, M1
Morishita, H1
Sugiyama, N1
Yokochi, K1
Nakano, M1
Wada, Y1
Hotta, Y1
Terauchi, A1
Nonaka, I1
Zöllner, N1
Reiter, S1
Gross, M1
Pongratz, D1
Gerbitz, K1
Paetzke, I1
Deufel, T1
Hübner, G1
Yase, Y1
Goebel, HH1
Bardosi, A1
Conrad, B1
Kuhlendahl, HD1
DiMauro, S1
Rumpf, KW1
Siemes, H1
Aspin, J1
Harrison, R1
Jehanli, A1
Lunt, G1
Campbell, M1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Phase 3 Trial of Coenzyme Q10 in Mitochondrial Disease[NCT00432744]Phase 324 participants (Actual)Interventional2007-01-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

McMaster Gross Motor Function (GMFM 88)

The McMaster Gross Motor Function is a validated scale ranging from 0 to 100 (the higher the better). Since there was the possibility of a subject becoming totally disabled our FDA peer reviewed design called for its use as follows: If the subject completed both periods, the score was calculated as the difference in scores between the end of Period 2 (at 12 months) minus that at the end of Period 1 (6 months). If a subject became totally disabled, this difference was considered as plus infinity if it occurred in period 1 (Penalizes period 1), and minus infinity if it occurred in Period 2 (Penalizes period 2). The two treatments were compared via the Wilcoxon test, and the effect size was estimated using Kendall's Tau-B. This is interpreted in a similar manner to correlation with positive values favoring COQenzyme10 and negative values favoring placebo. One of the links in this report is to the the GMFM scale and how it is scored. A link to the instrument is included. (NCT00432744)
Timeframe: Taken at 6 and 12 Months

Interventionunits on a scale (Median)
Placebo First-0.002
CoenzymeQ10 Frist-0.12

Non-parametric Hotelling T-square Bivariate Analysis of GMGF 88 and OPeds QOL.

This is a multivariate analysis of the first two outcomes: Period 2 minus Period 1 GMFM88 and Peds Quality of Life, analyzed as follows: First, to be in the analysis, subjects must contribute at least one of these endpoints. Second, if the subject became totally disabled during period 1, the difference was defined as + infinity, (highest possible evidence favoring period 2), and if the subject became totally disabled in period 2, the subject was scored as - infinity (highest possible evidence favoring period 1). Period 2 minus period 1 differences were ranked form low to high with missing values scores at the mid-rank. The Hotelling T-square was computed on these ranks and the P-value was obtained from 100,000 rerandomizations as the fraction of rerandomizations with T-sq at least as large as that observed. (NCT00432744)
Timeframe: end of 12 month minus end of 6 month difference.

Interventionparticipants (Number)
Placebo First7
CoenzymeQ10 Frist8

Pediatric Quality of Life Scale

"The Pediatric Quality of Life Scale is a validated scale ranging from 0 to 100 (the higher the better). Since there was the possibility of a subject becoming totally disabled our FDA peer reviewed design called for its use as follows: If the subject completed both periods, the score was calculated as the difference in scores between the end of Period 2 (at 12 months) minus that at the end of Period 1 (6 months). If a subject became totally disabled, this difference was considered as plus infinity if it occurred in period 1 (Penalizes period 1), and minus infinity if it occurred in Period 2 (Penalizes period 2). The two treatments were compared via the Wilcoxon test, and the effect size was estimated using Kendall's Tau-B. This is interpreted in a similar manner to correlation with positive values favoring COQenzyme10 and negative values favoring placebo. Goggle pedsQL and Mapi to browse the copyrighted manual. A link to the instrument is included." (NCT00432744)
Timeframe: At 6 and 12 Months

Interventionunits on a scale (Median)
Placebo First-1.1
CoenzymeQ10 Frist-11.9

Reviews

1 review available for lactic acid and Amyotonia Congenita

ArticleYear
MELAS syndrome. Report of two patients, and comparison with data of 24 patients derived from the literature.
    Neuropediatrics, 1991, Volume: 22, Issue:1

    Topics: Biopsy; Child; Child, Preschool; Electroencephalography; Electromyography; Energy Metabolism; Enzyme

1991

Trials

1 trial available for lactic acid and Amyotonia Congenita

ArticleYear
Impaired oxidative metabolism in exercising muscle from ALS patients.
    Journal of the neurological sciences, 2001, Oct-15, Volume: 191, Issue:1-2

    Topics: Adult; Aged; Amyotrophic Lateral Sclerosis; Electromyography; Exercise Test; Female; Heart Rate; Hum

2001

Other Studies

26 other studies available for lactic acid and Amyotonia Congenita

ArticleYear
Age-dependent neuromuscular impairment in prion protein knockout mice.
    Muscle & nerve, 2016, Volume: 53, Issue:2

    Topics: Action Potentials; Adenosine Triphosphatases; Aging; Animals; Citrate (si)-Synthase; Disease Models,

2016
Neuromuscular recovery pattern after medial collateral ligament disruption in rats.
    Journal of applied physiology (Bethesda, Md. : 1985), 2009, Volume: 107, Issue:1

    Topics: Animals; Disease Models, Animal; Female; Femoral Nerve; Hindlimb; Knee Injuries; Knee Joint; Lactic

2009
Magnetic resonance imaging findings in adult-form myotonic dystrophy type 1.
    Singapore medical journal, 2012, Volume: 53, Issue:7

    Topics: Adult; Atrophy; Brain; Cataract; Central Nervous System; Electromyography; Hearing Disorders; Humans

2012
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
Diagnostic utility of a modified forearm ischemic exercise test and technical issues relevant to exercise testing.
    Muscle & nerve, 2003, Volume: 27, Issue:3

    Topics: Adult; Ammonia; Catheterization, Peripheral; Eating; Exercise Test; Forearm; Glycogen Storage Diseas

2003
Modified exercise test in screening for mitochondrial myopathies--adjustment of workload in relation to muscle strength.
    European neurology, 2004, Volume: 51, Issue:1

    Topics: Adult; Aged; Case-Control Studies; Exercise Test; Exercise Tolerance; Female; Humans; Lactic Acid; M

2004
[Ophthalmoplegia plus. On nosologic, biochemical, morphologic and computer tomographic aspects of the oculocraniosomatic syndrome].
    Psychiatrie, Neurologie und medizinische Psychologie. Beihefte, 1983, Volume: 29

    Topics: Adult; Biopsy; Blepharoptosis; Brain Stem; Enzymes; Humans; Lactates; Lactic Acid; Male; Muscles; Ne

1983
Glucose-dependent lactate production by homogenates of neuronal tissues prepared from rats treated with 2,4-dithiobiuret, acrylamide, p-bromophenylacetylurea and 2,5-hexanedione.
    Neurotoxicology, 1984,Summer, Volume: 5, Issue:2

    Topics: Acrylamide; Acrylamides; Animals; Central Nervous System; Glucose; Glycolysis; Hexanones; In Vitro T

1984
Defects in citric acid cycle and the electron transport chain in progressive poliodystrophy.
    Acta neurologica Scandinavica, 1984, Volume: 70, Issue:3

    Topics: Adolescent; Brain; Carbon Dioxide; Child; Child, Preschool; Citric Acid Cycle; Electron Transport; F

1984
Diffuse white matter disease in three children: an encephalopathy with unique features on magnetic resonance imaging and proton magnetic resonance spectroscopy.
    Neuropediatrics, 1993, Volume: 24, Issue:5

    Topics: Aspartic Acid; Blood Glucose; Brain; Brain Diseases, Metabolic; Child; Child, Preschool; Choline; Ch

1993
[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
Retrospective study of a large population of patients with asymptomatic or minimally symptomatic raised serum creatine kinase levels.
    Journal of neurology, 2002, Volume: 249, Issue:3

    Topics: Adolescent; Adult; Aged; Amino Acids; Biopsy; Child; Child, Preschool; Creatine Kinase; Electromyogr

2002
[Delayed manifestation of mitochondrial myopathy--complex I and IV deficiency of the mitochondrial respiratory chain with progressive paresis].
    Der Nervenarzt, 1991, Volume: 62, Issue:8

    Topics: Biopsy; Cytochrome-c Oxidase Deficiency; Electromyography; Humans; Lactates; Lactic Acid; Male; Midd

1991
Marked reduction in CSF lactate and pyruvate levels after CoQ therapy in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS).
    Acta neurologica Scandinavica, 1991, Volume: 83, Issue:6

    Topics: Acidosis, Lactic; Adult; Agnosia; Cerebrovascular Disorders; Delirium; Dose-Response Relationship, D

1991
Therapeutic effect of sodium dichloroacetate on visual and auditory hallucinations in a patient with MELAS.
    Neuropediatrics, 1991, Volume: 22, Issue:3

    Topics: Acidosis, Lactic; Administration, Oral; Adult; Auditory Perception; Dichloroacetic Acid; Hallucinati

1991
Mitochondrial myopathy in Marinesco-Sjögren syndrome.
    Journal of mental deficiency research, 1991, Volume: 35 ( Pt 2)

    Topics: Adolescent; Adult; Biopsy; Cerebellum; Chromosome Aberrations; Chromosome Disorders; Exercise Test;

1991
[A case of mitochondrial encephalomyopathy characterized by repeated stroke-like episodes].
    Rinsho shinkeigaku = Clinical neurology, 1989, Volume: 29, Issue:3

    Topics: Adult; Brain Diseases; Cerebrovascular Disorders; Electroencephalography; Epilepsies, Myoclonic; Hum

1989
[Lactate and pyruvate blood levels in the diagnosis of mitochondrial myopathies].
    Der Nervenarzt, 1989, Volume: 60, Issue:9

    Topics: Adolescent; Adult; Aged; Exercise Test; Female; Humans; Lactates; Lactic Acid; Male; Middle Aged; Mi

1989
[A case of mitochondrial encephalomyopathy with myoclonic attacks, hyper-lactic-pyruvic acidemia, and decreased activities of complex II and cytochrome c oxidase].
    Rinsho shinkeigaku = Clinical neurology, 1988, Volume: 28, Issue:1

    Topics: Adult; Brain Diseases, Metabolic; Electron Transport Complex II; Electron Transport Complex IV; Huma

1988
Treatment of chronic congenital lactic acidosis by oral administration of dichloroacetate.
    Journal of inherited metabolic disease, 1986, Volume: 9, Issue:3

    Topics: Acetates; Acidosis; Adolescent; Brain; Dichloroacetic Acid; Humans; Infant, Newborn; Kidney; Lactate

1986
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.
    Journal of inherited metabolic disease, 1986, Volume: 9, Issue:3

    Topics: Acidosis; Adolescent; Cerebrovascular Disorders; Child, Preschool; Humans; Intellectual Disability;

1986
Myoadenylate deaminase deficiency: successful symptomatic therapy by high dose oral administration of ribose.
    Klinische Wochenschrift, 1986, Dec-15, Volume: 64, Issue:24

    Topics: Ammonia; AMP Deaminase; Drug Therapy, Combination; Humans; Hypoxanthine; Hypoxanthines; Inosine; Lac

1986
The pathogenetic role of metals in motor neuron disease--the participation of aluminum.
    Advances in experimental medicine and biology, 1987, Volume: 209

    Topics: Amyotrophic Lateral Sclerosis; Animals; Anterior Horn Cells; Calcium; Central Nervous System; Guam;

1987
Myoadenylate deaminase deficiency.
    Klinische Wochenschrift, 1986, Apr-01, Volume: 64, Issue:7

    Topics: Adult; Ammonia; AMP Deaminase; Biopsy; Electromyography; Exercise Test; Humans; Lactates; Lactic Aci

1986
[Mitochondrial myopathies and encephalomyopathies. Neuromuscular and central nervous system diseases caused by defects in mitochondrial oxidative metabolism].
    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde, 1985, Volume: 133, Issue:11

    Topics: Brain; Brain Diseases, Metabolic; Carnitine O-Acetyltransferase; Child; Citric Acid Cycle; Cytochrom

1985
Stimulation by mitogens and neuronal membranes of lymphocytes from patients with motor neurone disease.
    Journal of neuroimmunology, 1986, Volume: 11, Issue:1

    Topics: Adult; Aged; Animals; Cell Membrane; Cells, Cultured; Concanavalin A; Dose-Response Relationship, Dr

1986