Page last updated: 2024-10-16

carnitine and Brain Disorders

carnitine has been researched along with Brain Disorders in 30 studies

Research Excerpts

ExcerptRelevanceReference
"In systemic carnitine deficiency, an early phase of nonketotic hypoglycemia and liver dysfunction may precede a late phase of encephalopathy and myopathy."7.66Nonketotic hypoglycemia: an early indicator of systemic carnitine deficiency. ( Borum, PR; Diamond, MP; Mrak, RE; Najjar, J; Richardson, D; Slonim, AE, 1983)
"L-Carnitine is an essential cofactor in transfer of long-chain fatty acids across the inner mitochondrial membrane."6.36Carnitine metabolism and deficiency syndromes. ( Engel, AG; Rebouche, CJ, 1983)
"Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most prevalent encephalopathy in Japanese children."5.51Early administration of vitamins B1 and B6 and l-carnitine prevents a second attack of acute encephalopathy with biphasic seizures and late reduced diffusion: A case control study. ( Fukui, KO; Ishiguro, A; Kashii, H; Kubota, M; Terashima, H, 2019)
"Neurological injury because of transient cerebral ischemia is a potential complication of cardiovascular surgery."5.33Neuroprotective effects of L-carnitine and vitamin E alone or in combination against ischemia-reperfusion injury in rats. ( Aral, E; Aybek, H; Baltalarli, A; Baycu, C; Bir, LS; Coskun, E; Enli, Y; Oguz, EO; Onem, G; Ozcan, AV; Sacar, M, 2006)
"The association of macrocephaly, dystonia, and bilateral temporal arachnoid cysts, shown either by computed tomography or magnetic resonance imaging, seems to be diagnostic of glutaric aciduria type 1."5.29Macrocephaly, dystonia, and bilateral temporal arachnoid cysts: glutaric aciduria type 1. ( Casas, C; Fernández, MA; Martínez-Lage, JF; Poza, M; Puche, A; Rodriguez Costa, T, 1994)
"To evaluate the effects of L-carnitine on impaired brain function in patients with liver cirrhosis."3.96Impaired brain function improved by L-carnitine in patients with cirrhosis: evaluation using near-infrared spectroscopy. ( Hayakawa, Y; Higuchi, M; Inada, K; Itakura, J; Izumi, N; Kaneko, S; Kirino, S; Kubota, Y; Kurosaki, M; Maeyashiki, C; Nakanishi, H; Nakanishi, K; Noda, T; Okada, M; Osawa, L; Sekiguchi, S; Takahashi, Y; Takaura, K; Tamaki, N; Tsuchiya, K; Wan, W; Yamashita, K; Yasui, Y, 2020)
"We report the case of an aborted awake craniotomy for a left frontotemporoinsular glioma due to ammonia encephalopathy on a patient taking Levetiracetam, valproic acid and clobazam."3.81Ammonia encephalopathy and awake craniotomy for brain language mapping: cause of failed awake craniotomy. ( Arroyo Pérez, R; Fernández-Candil, JL; León Jorba, A; Pacreu Terradas, S; Villalba Martínez, G; Vivanco-Hidalgo, RM, 2015)
"A 24-years-old woman with epilepsy treated with valproic acid developed disorientation, acalculia, perseveration, slow responsiveness and loss of memory."3.70[A case report of valproate encephalopathy]. ( Hirata, K; Katayama, S; Miyamoto, M; Miyamoto, T, 1999)
"In systemic carnitine deficiency, an early phase of nonketotic hypoglycemia and liver dysfunction may precede a late phase of encephalopathy and myopathy."3.66Nonketotic hypoglycemia: an early indicator of systemic carnitine deficiency. ( Borum, PR; Diamond, MP; Mrak, RE; Najjar, J; Richardson, D; Slonim, AE, 1983)
"L-Carnitine is an essential cofactor in transfer of long-chain fatty acids across the inner mitochondrial membrane."2.36Carnitine metabolism and deficiency syndromes. ( Engel, AG; Rebouche, CJ, 1983)
"A 9-month-old patient was admitted with encephalopathy and acute loss of acquired motor skills during the course of COVID-19 disease."1.62COVID-19 triggered encephalopathic crisis in a patient with glutaric aciduria type 1. ( Ahmadzada, S; Aktuglu-Zeybek, C; Kiykim, E; Yalcinkaya, C; Zubarioglu, T, 2021)
"Levocarnitine is the treatment of choice in valproic acid toxicity-related hyperammonaemia."1.62Managing valproic acid toxicity-related hyperammonaemia: an unpredicted course. ( Edquist, C; O'Rourke, N; Pagali, S, 2021)
"Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most prevalent encephalopathy in Japanese children."1.51Early administration of vitamins B1 and B6 and l-carnitine prevents a second attack of acute encephalopathy with biphasic seizures and late reduced diffusion: A case control study. ( Fukui, KO; Ishiguro, A; Kashii, H; Kubota, M; Terashima, H, 2019)
"Neurodevelopmental delay and encephalopathy are complications of both HIV and GA1."1.48Favourable outcome in a child with symptomatic diagnosis of Glutaric aciduria type 1 despite vertical HIV infection and minor head trauma. ( Ackermann, C; Cotton, MF; Dobbels, EFM; Laughton, B; Springer, PE; Thomas, A, 2018)
"To examine the clinical features and risk factors of secondary carnitine deficiency due to long-term use of pivalate-conjugated antibiotics (PCAs)."1.43Clinical Features of Carnitine Deficiency Secondary to Pivalate-Conjugated Antibiotic Therapy. ( Fukuda, S; Hasegawa, Y; Kobayashi, H; Purevsuren, J; Takahashi, T; Yamada, K; Yamaguchi, S, 2016)
"We report the first case of acute encephalopathy induced by vaccination in an infant with methylmalonic aciduria cblA in China."1.42[Acute encephalopathy induced by vaccination in an infant with methylmalonic aciduria cblA]. ( Ding, Y; Li, X; Liu, Y; Song, J; Wang, H; Wang, Q; Wu, T; Yang, Y; Zhang, Y, 2015)
" The vast knowledge on the carnitine/acylcarnitine translocase is essential both as a progress in basic science and as instrument to foresee therapeutic or toxic effects of xenobiotics and drugs."1.42Mitochondrial carnitine/acylcarnitine translocase: insights in structure/ function relationships. Basis for drug therapy and side effects prediction. ( Console, L; Giangregorio, N; Indiveri, C; Tonazzi, A, 2015)
"Carnitine supplement was administrated in two patients, which resulted in resolution of symptoms and normalized ammonium levels."1.40Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease). ( Larsen, EP; Ostergaard, JR, 2014)
"Carnitine is an essential co-factor in fatty acid metabolism."1.35Hyperammonemic encephalopathy caused by carnitine deficiency. ( Limketkai, BN; Zucker, SD, 2008)
"Neurological injury because of transient cerebral ischemia is a potential complication of cardiovascular surgery."1.33Neuroprotective effects of L-carnitine and vitamin E alone or in combination against ischemia-reperfusion injury in rats. ( Aral, E; Aybek, H; Baltalarli, A; Baycu, C; Bir, LS; Coskun, E; Enli, Y; Oguz, EO; Onem, G; Ozcan, AV; Sacar, M, 2006)
"The association of macrocephaly, dystonia, and bilateral temporal arachnoid cysts, shown either by computed tomography or magnetic resonance imaging, seems to be diagnostic of glutaric aciduria type 1."1.29Macrocephaly, dystonia, and bilateral temporal arachnoid cysts: glutaric aciduria type 1. ( Casas, C; Fernández, MA; Martínez-Lage, JF; Poza, M; Puche, A; Rodriguez Costa, T, 1994)
"Of the nine patients with encephalopathy, eight showed improvement in their mental status."1.27Plasma carnitine deficiency. Clinical observations in 51 pediatric patients. ( Curry, CJ; Hogue, R; Hutchinson, HT; Shug, A; Szabo-Aczel, S; Winter, SC, 1987)

Research

Studies (30)

TimeframeStudies, this research(%)All Research%
pre-19906 (20.00)18.7374
1990's4 (13.33)18.2507
2000's5 (16.67)29.6817
2010's12 (40.00)24.3611
2020's3 (10.00)2.80

Authors

AuthorsStudies
Zubarioglu, T1
Ahmadzada, S1
Yalcinkaya, C1
Kiykim, E1
Aktuglu-Zeybek, C1
Nakanishi, H1
Hayakawa, Y1
Kubota, Y1
Kurosaki, M1
Osawa, L1
Inada, K1
Kirino, S1
Yamashita, K1
Sekiguchi, S1
Okada, M1
Wan, W1
Higuchi, M1
Takaura, K1
Maeyashiki, C1
Kaneko, S1
Tamaki, N1
Yasui, Y1
Noda, T1
Nakanishi, K1
Tsuchiya, K1
Itakura, J1
Takahashi, Y1
Izumi, N1
Pagali, S1
Edquist, C1
O'Rourke, N1
Thomas, A1
Dobbels, EFM1
Springer, PE1
Ackermann, C1
Cotton, MF1
Laughton, B1
Camilleri, L1
Fukui, KO1
Kubota, M1
Terashima, H1
Ishiguro, A1
Kashii, H1
Boruczkowski, D1
Pujal, JM1
Zdolińska-Malinowska, I1
Larsen, EP1
Ostergaard, JR1
Villalba Martínez, G1
Fernández-Candil, JL1
Vivanco-Hidalgo, RM1
Pacreu Terradas, S1
León Jorba, A1
Arroyo Pérez, R1
Liu, Y1
Wu, T1
Wang, H1
Ding, Y1
Song, J1
Li, X1
Zhang, Y1
Wang, Q1
Yang, Y1
Tonazzi, A1
Giangregorio, N1
Console, L1
Indiveri, C1
Al-sharefi, A1
Bilous, R1
Kobayashi, H1
Fukuda, S1
Yamada, K1
Hasegawa, Y1
Takahashi, T1
Purevsuren, J1
Yamaguchi, S1
Ozsoy, SY1
Ozsoy, B1
Ozyildiz, Z1
Aytekin, I1
Gokmen-Ozel, H1
MacDonald, A1
Daly, A1
Ashmore, C1
Preece, MA1
Hendriksz, C1
Vijay, S1
Chakrapani, A1
Spiekerkoetter, U1
Huener, G1
Baykal, T1
Demirkol, M1
Duran, M1
Wanders, R1
Nezu, J1
Mayatepek, E1
Bischof, F1
Nägele, T1
Wanders, RJ1
Trefz, FK1
Melms, A1
Onem, G1
Aral, E1
Enli, Y1
Oguz, EO1
Coskun, E1
Aybek, H1
Ozcan, AV1
Sacar, M1
Bir, LS1
Baltalarli, A1
Baycu, C1
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
Limketkai, BN1
Zucker, SD1
Rebouche, CJ1
Engel, AG1
Kim, CS1
Dorgan, DR1
Roe, CR1
Slonim, AE1
Borum, PR1
Mrak, RE1
Najjar, J1
Richardson, D1
Diamond, MP1
Di Donato, S1
Rimoldi, M1
Cornelio, F1
Bottacchi, E1
Giunta, A1
Averbuch-Heller, L1
Ben-Hur, T1
Reches, A1
Martínez-Lage, JF1
Casas, C1
Fernández, MA1
Puche, A1
Rodriguez Costa, T1
Poza, M1
Miyamoto, T1
Miyamoto, M1
Hirata, K1
Katayama, S1
Murakami, K1
Sugimoto, T2
Nishida, N2
Kobayashi, Y1
Kuhara, T1
Matsumoto, I1
Winter, SC1
Szabo-Aczel, S1
Curry, CJ1
Hutchinson, HT1
Hogue, R1
Shug, A1
Woo, M1
Takeuchi, T1
Yasuhara, A1

Clinical Trials (3)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
In Vivo Study of Safety, Tolerability and Dosing Effect on SMN mRNA and Protein Levels of Valproic Acid in Patients With Spinal Muscular Atrophy[NCT00374075]Phase 142 participants Interventional2003-09-30Completed
Phase I/II Trial of Valproic Acid and Carnitine in Infants With Spinal Muscular Atrophy Type I (CARNI-VAL Type I)[NCT00661453]Phase 1/Phase 240 participants (Actual)Interventional2008-04-30Completed
Multi-center Phase II Trial of Valproic Acid and Carnitine in Patients With Spinal Muscular Atrophy (SMA CARNI-VAL Trial)[NCT00227266]Phase 294 participants (Actual)Interventional2005-09-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Anthropometric Measures of Nutritional Status (Body Mass Index [BMI] Z-scores, Weight for Length Ratios, Lean/Fat Mass Via DEXA, Growth Parameters, and Triceps Skinfold Measures)

(NCT00661453)
Timeframe: -2 weeks, time 0, 3 months, 6 months

Interventiong (Mean)
Lean Mass BaselineLean Mass 3 monthsLean Mass 6 monthsFat Mass BaselineFat Mass 3 monthsFat Mass 6 months
SMA Type 14317.154993.925133.833011.373618.254316.08

Max CMAP Amplitude (Mean)

The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)

,,
InterventionmV (Mean)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment2.282.32
Cohort 1b Sitters Treatment2.932.37
Cohort 2 Standers and Walkers - Treatment5.526.56

Max CMAP Amplitude Median

The maximum Compound Motor Action Potential (CMAP) is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This is done multiple times, the outcome used is the highest peak, or response observed. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)

,,
InterventionmV (Median)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment1.911.44
Cohort 1b Sitters Treatment2.21.8
Cohort 2 Standers and Walkers - Treatment5.35.85

Max CMAP Area (Mean)

The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)

,,
InterventionmVms (Mean)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment5.465.28
Cohort 1b Sitters Treatment5.455.26
Cohort 2 Standers and Walkers - Treatment14.8516.26

Max CMAP Area (Median)

The maximum Compound Motor Action Potential (CMAP) area is a measurement obtained through EMG testing that is associated with disease progression. In this study, we measure the maximum CMAP by stimulating one nerve in the hand and measuring the response of the muscle. This procedure is repeated multiple times. The maximum area is the response that results in the largest area under the response curve. (NCT00227266)
Timeframe: 1 month prior to official enrollment, beginning of study (0 months), 6 months, 12 months (data point not available)

,,
InterventionmVms (Median)
Baseline6 months
Cohort 1a Sitters Placebo Then Treatment3.63.74
Cohort 1b Sitters Treatment4.63.4
Cohort 2 Standers and Walkers - Treatment13.6516.85

Modified Hammersmith Change From Baseline to 6 Months

Comparison of Modified Hammersmith Change from baseline to 6 months. Scores range from 0 to 40. A higher score indicates a better outcome. This scale is used to assess gross motor abilities of non-ambulant children with SMA in multiple research trials as well as in clinical settings. (NCT00227266)
Timeframe: 0 months, 6 months

,
InterventionScore (Mean)
Baseline visit (0 weeks)6 Month visit (V2)Change from Baseline
Cohort 1a Sitters Placebo Then Treatment20.020.60.6
Cohort 1b Sitters Treatment16.616.80.2

Modified Hammersmith Extend Baseline

"Baseline Modified Hammersmith Extend testing. The baseline test is the score they receive during their screening visits. This scale ranges from 0 to 56. A higher score indicates a better outcome.~This scale is used to assess gross motor abilities of children with SMA in multiple research trials as well as in clinical settings." (NCT00227266)
Timeframe: 1 month prior to enrollment, at enrollment (0 months)

InterventionScore (Mean)
Modified Hammersmith Extend at S1 (-4 weeks)Modified Hammersmith Extend at S2 (0 weeks)
Cohort 2 Experimental47.048.3

Reviews

3 reviews available for carnitine and Brain Disorders

ArticleYear
Lesson of the month 1: Sodium valproate-induced encephalopathy.
    Clinical medicine (London, England), 2018, Volume: 18, Issue:5

    Topics: Aged; Ammonia; Anticonvulsants; Brain Diseases; Carnitine; Consciousness Disorders; Epilepsy; Humans

2018
Autologous cord blood in children with cerebral palsy: a review.
    International journal of molecular sciences, 2019, May-16, Volume: 20, Issue:10

    Topics: 3-Hydroxybutyric Acid; Amino Acids; Animals; Brain Diseases; Brain Injuries; Carnitine; Cerebral Pal

2019
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983
Carnitine metabolism and deficiency syndromes.
    Mayo Clinic proceedings, 1983, Volume: 58, Issue:8

    Topics: Adolescent; Adult; Aged; Brain Diseases; Carnitine; Chemical Phenomena; Chemistry; Child; Child, Pre

1983

Other Studies

27 other studies available for carnitine and Brain Disorders

ArticleYear
COVID-19 triggered encephalopathic crisis in a patient with glutaric aciduria type 1.
    Journal of pediatric endocrinology & metabolism : JPEM, 2021, Dec-20, Volume: 34, Issue:12

    Topics: Amino Acid Metabolism, Inborn Errors; Brain; Brain Diseases; Brain Diseases, Metabolic; Carnitine; C

2021
Impaired brain function improved by L-carnitine in patients with cirrhosis: evaluation using near-infrared spectroscopy.
    Scientific reports, 2020, 08-11, Volume: 10, Issue:1

    Topics: Aged; Brain Diseases; Carnitine; Female; Humans; Liver Cirrhosis; Male; Prognosis; Retrospective Stu

2020
Managing valproic acid toxicity-related hyperammonaemia: an unpredicted course.
    BMJ case reports, 2021, Apr-19, Volume: 14, Issue:4

    Topics: Adult; Brain Diseases; Carnitine; Drug Overdose; Female; Humans; Hyperammonemia; Valproic Acid; Youn

2021
Favourable outcome in a child with symptomatic diagnosis of Glutaric aciduria type 1 despite vertical HIV infection and minor head trauma.
    Metabolic brain disease, 2018, Volume: 33, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Atrophy; Brain; Brain Diseases; Brain Diseases, Metabolic; Car

2018
Early administration of vitamins B1 and B6 and l-carnitine prevents a second attack of acute encephalopathy with biphasic seizures and late reduced diffusion: A case control study.
    Brain & development, 2019, Volume: 41, Issue:7

    Topics: Brain Diseases; Carnitine; Case-Control Studies; Child, Preschool; Diagnosis, Differential; Disease

2019
Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease).
    Seizure, 2014, Volume: 23, Issue:6

    Topics: Adolescent; Anticonvulsants; Brain Diseases; Carnitine; Drug Therapy, Combination; Female; Humans; H

2014
Ammonia encephalopathy and awake craniotomy for brain language mapping: cause of failed awake craniotomy.
    Revista espanola de anestesiologia y reanimacion, 2015, Volume: 62, Issue:5

    Topics: Anesthesia, General; Anesthesia, Local; Anticonvulsants; Aphasia; Benzodiazepines; Brain Diseases; B

2015
[Acute encephalopathy induced by vaccination in an infant with methylmalonic aciduria cblA].
    Zhonghua er ke za zhi = Chinese journal of pediatrics, 2015, Volume: 53, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases; Carnitine; Diet, Protein-Restricted; Hepatitis

2015
Mitochondrial carnitine/acylcarnitine translocase: insights in structure/ function relationships. Basis for drug therapy and side effects prediction.
    Mini reviews in medicinal chemistry, 2015, Volume: 15, Issue:5

    Topics: Amino Acid Sequence; Animals; Anti-Bacterial Agents; Bacterial Infections; Brain Diseases; Cardiovas

2015
Reversible weakness and encephalopathy while on long-term valproate treatment due to carnitine deficiency.
    BMJ case reports, 2015, Sep-02, Volume: 2015

    Topics: Adult; Antimanic Agents; Brain Diseases; Cardiomyopathies; Carnitine; Female; Humans; Hyperammonemia

2015
Clinical Features of Carnitine Deficiency Secondary to Pivalate-Conjugated Antibiotic Therapy.
    The Journal of pediatrics, 2016, Volume: 173

    Topics: Adolescent; Adult; Anti-Bacterial Agents; Brain Diseases; Carnitine; Child; Child, Preschool; Female

2016
Protective effect of L-carnitine on experimental lead toxicity in rats: a clinical, histopathological and immunohistochemical study.
    Biotechnic & histochemistry : official publication of the Biological Stain Commission, 2011, Volume: 86, Issue:6

    Topics: Animals; Blood Cells; Brain Diseases; Carnitine; Chemical and Drug Induced Liver Injury; Enzymes; He

2011
Dietary practices in glutaric aciduria type 1 over 16 years.
    Journal of human nutrition and dietetics : the official journal of the British Dietetic Association, 2012, Volume: 25, Issue:6

    Topics: Adolescent; Adult; Age Factors; Amino Acid Metabolism, Inborn Errors; Biomarkers; Brain Diseases; Br

2012
Silent and symptomatic primary carnitine deficiency within the same family due to identical mutations in the organic cation/carnitine transporter OCTN2.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:6

    Topics: Adult; Brain Diseases; Carnitine; Carrier Proteins; Child, Preschool; Fibroblasts; Homozygote; Human

2003
3-hydroxy-3-methylglutaryl-CoA lyase deficiency in an adult with leukoencephalopathy.
    Annals of neurology, 2004, Volume: 56, Issue:5

    Topics: Adult; Brain Diseases; Carnitine; Female; Fibroblasts; Glutarates; Humans; Hypoglycemia; Leukocytes;

2004
Neuroprotective effects of L-carnitine and vitamin E alone or in combination against ischemia-reperfusion injury in rats.
    The Journal of surgical research, 2006, Volume: 131, Issue:1

    Topics: Animals; Antioxidants; Brain; Brain Diseases; Brain Ischemia; Carnitine; Drug Therapy, Combination;

2006
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
Hyperammonemic encephalopathy caused by carnitine deficiency.
    Journal of general internal medicine, 2008, Volume: 23, Issue:2

    Topics: Adult; Ammonia; Brain Diseases; Carnitine; Fatty Acids; Female; Gastric Bypass; Humans; Lipid Metabo

2008
L-carnitine: therapeutic strategy for metabolic encephalopathy.
    Brain research, 1984, Sep-17, Volume: 310, Issue:1

    Topics: Animals; Biological Transport, Active; Brain Diseases; Caprylates; Carnitine; Choroid Plexus; In Vit

1984
Nonketotic hypoglycemia: an early indicator of systemic carnitine deficiency.
    Neurology, 1983, Volume: 33, Issue:1

    Topics: Brain Diseases; Carnitine; Child; Child, Preschool; Female; Humans; Hypoglycemia; Liver; Male; Muscl

1983
Evidence for autosomal recessive inheritance in systemic carnitine deficiency.
    Annals of neurology, 1982, Volume: 11, Issue:2

    Topics: Brain Diseases; Carnitine; Child, Preschool; Female; Humans; Muscular Diseases

1982
Valproate encephalopathy and hypocarnitinaemia in diabetic patients.
    Journal of neurology, 1994, Volume: 241, Issue:9

    Topics: Adolescent; Adult; Brain Diseases; Carnitine; Diabetes Mellitus, Type 1; Electroencephalography; Epi

1994
Macrocephaly, dystonia, and bilateral temporal arachnoid cysts: glutaric aciduria type 1.
    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 1994, Volume: 10, Issue:3

    Topics: Arachnoid Cysts; Brain; Brain Diseases; Carnitine; Dystonia; Female; gamma-Aminobutyric Acid; Glutar

1994
[A case report of valproate encephalopathy].
    Rinsho shinkeigaku = Clinical neurology, 1999, Volume: 39, Issue:10

    Topics: Adult; Ammonia; Anticonvulsants; Biomarkers; Brain; Brain Diseases; Carnitine; Epilepsy; Female; Hum

1999
Abnormal metabolism of carnitine and valproate in a case of acute encephalopathy during chronic valproate therapy.
    Brain & development, 1992, Volume: 14, Issue:3

    Topics: Brain Diseases; Carnitine; Cerebral Palsy; Child; Chronic Disease; Female; Gas Chromatography-Mass S

1992
Plasma carnitine deficiency. Clinical observations in 51 pediatric patients.
    American journal of diseases of children (1960), 1987, Volume: 141, Issue:6

    Topics: Adolescent; Brain Diseases; Cardiomyopathies; Carnitine; Child; Child, Preschool; Failure to Thrive;

1987
[Significance of serum carnitine in acute encephalopathy].
    No to hattatsu = Brain and development, 1985, Volume: 17, Issue:2

    Topics: Acute Disease; Adolescent; Brain Diseases; Carnitine; Child; Child, Preschool; Diagnosis, Differenti

1985