Page last updated: 2024-10-16

carnitine and Nervous System Diseases

carnitine has been researched along with Nervous System Diseases in 21 studies

Nervous System Diseases: Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle.

Research Excerpts

ExcerptRelevanceReference
"Carnitine levels in CSF were 1/10 compared with serum in normal febrile children."5.30CSF levels of carnitine in children with meningitis, neurologic disorders, acute gastroenteritis, and seizure. ( Gruener, N; Lerner, A; Shinawi, M, 1998)
"These data on disease progression will be useful in future studies using the canine model for therapeutic intervention studies."1.72Characterization of neurological disease progression in a canine model of CLN5 neuronal ceroid lipofuscinosis. ( Coates, JR; Jensen, CA; Katz, ML; Kick, GR; Meiman, EJ, 2022)
"Carnitine levels in CSF were 1/10 compared with serum in normal febrile children."1.30CSF levels of carnitine in children with meningitis, neurologic disorders, acute gastroenteritis, and seizure. ( Gruener, N; Lerner, A; Shinawi, M, 1998)

Research

Studies (21)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's5 (23.81)18.2507
2000's6 (28.57)29.6817
2010's7 (33.33)24.3611
2020's3 (14.29)2.80

Authors

AuthorsStudies
Rücklová, K1
Hrubá, E1
Pavlíková, M1
Hanák, P1
Farolfi, M1
Chrastina, P1
Vlášková, H1
Kousal, B1
Smolka, V1
Foltenová, H1
Adam, T1
Friedecký, D1
Ješina, P1
Zeman, J1
Kožich, V1
Honzík, T1
Meiman, EJ1
Kick, GR1
Jensen, CA1
Coates, JR1
Katz, ML1
Praveenraj, SS1
Sonali, S1
Anand, N1
Tousif, HA1
Vichitra, C1
Kalyan, M1
Kanna, PV1
Chandana, KA1
Shasthara, P1
Mahalakshmi, AM1
Yang, J1
Pandi-Perumal, SR1
Sakharkar, MK1
Chidambaram, SB1
Nakamichi, N1
Kato, Y1
Gillingham, MB1
Heitner, SB1
Martin, J1
Rose, S1
Goldstein, A1
El-Gharbawy, AH1
Deward, S1
Lasarev, MR1
Pollaro, J1
DeLany, JP1
Burchill, LJ1
Goodpaster, B1
Shoemaker, J1
Matern, D1
Harding, CO1
Vockley, J1
Haglind, CB1
Nordenström, A1
Ask, S1
von Döbeln, U1
Gustafsson, J1
Stenlid, MH1
Schobersberger, W1
Dünnwald, T1
Gmeiner, G1
Blank, C1
Yamamoto, Y1
Matsui, N1
Hiramatsu, Y1
Miyazaki, Y1
Nodera, H1
Izumi, Y1
Takashima, H1
Kaji, R1
Jones, LL1
McDonald, DA1
Borum, PR1
Park, HD1
Kim, SR1
Ki, CS1
Lee, SY1
Chang, YS1
Jin, DK1
Park, WS1
Malaguarnera, M1
Bähr, O1
Mader, I1
Zschocke, J1
Dichgans, J1
Schulz, JB1
Mühlhausen, C1
Hoffmann, GF1
Strauss, KA1
Kölker, S1
Okun, JG1
Greenberg, CR1
Naughten, ER1
Ullrich, K1
Gordon, N1
Merinero, B1
Pérez-Cerdá, C1
Ruiz Sala, P1
Ferrer, I1
García, MJ1
Martínez Pardo, M1
Belanger-Quintana, A1
de la Mota, JL1
Martin-Hernández, E1
Vianey-Saban, C1
Bischoff, C1
Gregersen, N1
Ugarte, M1
López-Laso, E1
García-Villoria, J1
Martín, E1
Duque, P1
Cano, A1
Ribes, A1
Ozand, PT1
Rashed, M1
Gascon, GG1
al Odaib, A1
Shums, A1
Nester, M1
Brismar, J1
Walter, JH1
Wraith, JE1
Cleary, MA1
Shinawi, M1
Gruener, N1
Lerner, A1
Matsuoka, M1
Igisu, H1
Kohriyama, K1
Inoue, N1
Dionisi Vici, C1
Burlina, AB1
Bertini, E1
Bachmann, C1
Mazziotta, MR1
Zacchello, F1
Sabetta, G1
Hale, DE1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Phase 2 Study of Triheptanoin for Treatment of Long-Chain Fatty Acid Oxidation Disorders[NCT01379625]Phase 232 participants (Actual)Interventional2011-09-30Completed
A Phase 2A/2B Placebo-controlled Randomised Clinical Trial to Test the Ability of Triheptanoin to Protect Primary Airway Epithelial Cells Obtained From Participants With Ataxia-telangiectasia Against Death Induced by Glucose Deprivation[NCT04513002]Phase 230 participants (Actual)Interventional2022-03-15Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Ejection Fraction

Change in resting ejection fraction over 4 month treatment period (NCT01379625)
Timeframe: 4 months

Interventionpercent (Mean)
Medium Chain Triglyceride (MCT)-1.91
Triheptanoin2.14

Energy Expenditure

Total energy expenditure will be measured by doubly labeled water and resting energy expenditure will be measured by indirect calorimetry at baseline and again after 4 months of either MCT or trihpetanoin treatment. (NCT01379625)
Timeframe: change from baseline after 4 months of treatment

Interventionkcal/day (Mean)
Medium Chain Triglyceride (MCT)-73
Triheptanoin107

Exercise Heart Rate

Subjects will complete a submaximal treadmill exercise study at baseline. Exercise heart heart, ventilation and perceived exertion will be measured. Subjects will be randomized to MCT or triheptanoin supplementation for 4 months. At the end of treatment, the exercise test will be repeated keeping work performed constant. Change in exercise heart rate, ventilation and exertion will be compared between groups. (NCT01379625)
Timeframe: change from baseline to 4 months of treatment

Interventionbeats per minute (Mean)
Medium Chain Triglyceride (MCT)-0.1
Triheptanoin-12.6

Reviews

6 reviews available for carnitine and Nervous System Diseases

ArticleYear
The Role of a Gut Microbial-Derived Metabolite, Trimethylamine N-Oxide (TMAO), in Neurological Disorders.
    Molecular neurobiology, 2022, Volume: 59, Issue:11

    Topics: Betaine; Carnitine; Choline; Flavins; Gastrointestinal Microbiome; Humans; Inflammasomes; Lyases; Me

2022
Physiological Roles of Carnitine/Organic Cation Transporter OCTN1/SLC22A4 in Neural Cells.
    Biological & pharmaceutical bulletin, 2017, Volume: 40, Issue:8

    Topics: Animals; Carnitine; Humans; Nervous System Diseases; Neural Stem Cells; Neurons; Organic Cation Tran

2017
Story behind meldonium-from pharmacology to performance enhancement: a narrative review.
    British journal of sports medicine, 2017, Volume: 51, Issue:1

    Topics: Athletes; Athletic Performance; Cardiovascular Diseases; Carnitine; Doping in Sports; Humans; Metabo

2017
Acylcarnitines: role in brain.
    Progress in lipid research, 2010, Volume: 49, Issue:1

    Topics: Acetylcarnitine; Animals; Antioxidants; Brain; Carnitine; Energy Metabolism; Fatty Acids; Nerve Grow

2010
Carnitine derivatives: clinical usefulness.
    Current opinion in gastroenterology, 2012, Volume: 28, Issue:2

    Topics: Acetylcarnitine; Cardiotonic Agents; Cardiovascular Diseases; Carnitine; Cognition Disorders; Depres

2012
Maintenance treatment of glutaryl-CoA dehydrogenase deficiency.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Animals; Antioxidants; Carnitine; Glutaryl-CoA Dehydrogenase;

2004

Trials

1 trial available for carnitine and Nervous System Diseases

ArticleYear
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
    Journal of inherited metabolic disease, 2017, Volume: 40, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil

2017
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
    Journal of inherited metabolic disease, 2017, Volume: 40, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil

2017
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
    Journal of inherited metabolic disease, 2017, Volume: 40, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil

2017
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
    Journal of inherited metabolic disease, 2017, Volume: 40, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Caprylates; Cardiomyopathies; Carnitine; Chil

2017

Other Studies

14 other studies available for carnitine and Nervous System Diseases

ArticleYear
Impact of Newborn Screening and Early Dietary Management on Clinical Outcome of Patients with Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Medium Chain Acyl-CoA Dehydrogenase Deficiency-A Retrospective Nationwide Study.
    Nutrients, 2021, Aug-24, Volume: 13, Issue:9

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; Child; Child,

2021
Characterization of neurological disease progression in a canine model of CLN5 neuronal ceroid lipofuscinosis.
    Developmental neurobiology, 2022, Volume: 82, Issue:4

    Topics: Animals; Atrophy; Carnitine; Disease Progression; Dogs; Homozygote; Nervous System Diseases; Neurona

2022
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast.
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:2

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Age Factors; Biomarkers; Blood Glucose; Calorimetry, Indirect; Car

2015
Mitochondrial trifunctional protein deficiency: an adult patient with similar progress to Charcot-Marie-Tooth disease.
    Rinsho shinkeigaku = Clinical neurology, 2017, 02-25, Volume: 57, Issue:2

    Topics: Biomarkers; Cardiomyopathies; Carnitine; Charcot-Marie-Tooth Disease; Diagnosis, Differential; Disea

2017
Two novel HADHB gene mutations in a Korean patient with mitochondrial trifunctional protein deficiency.
    Annals of clinical and laboratory science, 2009,Fall, Volume: 39, Issue:4

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathies; Carnitine; DNA Mutational Analysis; Humans; Infa

2009
Adult onset glutaric aciduria type I presenting with a leukoencephalopathy.
    Neurology, 2002, Dec-10, Volume: 59, Issue:11

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Brain; Carnitine; Electroencephalography; Electrophores

2002
Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement.
    Developmental medicine and child neurology, 2005, Volume: 47, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Child; Child, Preschool; Chromosome Aberrations; Developmental Di

2005
Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:5

    Topics: Brain Diseases; Butyryl-CoA Dehydrogenase; Carnitine; Humans; Malonates; Mitochondrial Proteins; Ner

2006
Classic and late-onset neurological disease in two siblings with glutaryl-CoA dehydrogenase deficiency.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:6

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Carnitine; Female; Glutaryl-CoA Dehydrogenase; Hum

2007
3-Ketothiolase deficiency: a review and four new patients with neurologic symptoms.
    Brain & development, 1994, Volume: 16 Suppl

    Topics: Acetyl-CoA C-Acyltransferase; Brain; Carnitine; Child, Preschool; Electroencephalography; Female; Fi

1994
Absence of acidosis in the initial presentation of propionic acidaemia.
    Archives of disease in childhood. Fetal and neonatal edition, 1995, Volume: 72, Issue:3

    Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Food Pr

1995
CSF levels of carnitine in children with meningitis, neurologic disorders, acute gastroenteritis, and seizure.
    Neurology, 1998, Volume: 50, Issue:6

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Female; Fever; Gastroenteritis; Humans; Infant; Infa

1998
Suppression of neurotoxicity of ammonia by L-carnitine.
    Brain research, 1991, Dec-20, Volume: 567, Issue:2

    Topics: Ammonia; Animals; Behavior, Animal; Brain Chemistry; Carnitine; Energy Metabolism; Male; Mice; Mice,

1991
Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
    The Journal of pediatrics, 1991, Volume: 118, Issue:5

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Biopsy; Carnitine; Child; Combined Modality Therapy; Dietary Fats;

1991