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methylmalonic acid and Nervous System Diseases

methylmalonic acid has been researched along with Nervous System Diseases in 21 studies

Methylmalonic Acid: A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA.
methylmalonic acid : A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group.

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
" Characteristic features in such patients included paresthesia, sensory loss, ataxia, dementia, and psychiatric disorders; longstanding neurologic symptoms without anemia; normal white-cell and platelet counts and serum bilirubin and lactate dehydrogenase levels; and markedly elevated serum concentrations of methylmalonic acid and total homocysteine."3.67Neuropsychiatric disorders caused by cobalamin deficiency in the absence of anemia or macrocytosis. ( Allen, RH; Brust, JC; Garrett, TJ; Healton, EB; Lindenbaum, J; Marcell, PD; Podell, ER; Savage, DG; Stabler, SP, 1988)
" We discuss the importance of methylmalonic acid dosage after bariatric surgery in patients who present suspect of cyanocobalamin deficiency with normal values of this vitamin and the role of proton pump inhibitor use and vitamin supplementation in patients with early neurological presentation."2.47The importance of methylmalonic acid dosage on the assessment of patients with neurological manifestations following bariatric surgery. ( Cosmo, C; Lucena, R; Vieira, C, 2011)
"Diagnosis of vitamin B12 deficiency is typically based on measurement of serum vitamin B12 levels; however, about 50 percent of patients with subclinical disease have normal B12 levels."2.42Vitamin B12 deficiency. ( Brown, DL; Oh, R, 2003)
"Neuropathies often accompany vitamin B12 deficiency."1.43Vitamin B12-responsive neuropathies: A case series. ( Solomon, LR, 2016)
"Neurological dysfunction due to vitamin B12 deficiency is most prominent in patients with mild to moderate anaemia."1.29Vitamin B12 deficiency is the primary cause of megaloblastic anaemia in Zimbabwe. ( Bennie, A; Gangaidzo, I; Gwanzura, C; Kiire, C; Lindenbaum, J; Moyo, A; Mudenge, B; Mukiibi, JM; Savage, D; Sitima, J, 1994)

Research

Studies (21)

TimeframeStudies, this research(%)All Research%
pre-19902 (9.52)18.7374
1990's10 (47.62)18.2507
2000's6 (28.57)29.6817
2010's2 (9.52)24.3611
2020's1 (4.76)2.80

Authors

AuthorsStudies
Ailliet, S1
Vandenberghe, R1
Schiemsky, T1
Van Overbeke, L1
Demaerel, P1
Meersseman, W1
Cassiman, D1
Vermeersch, P1
Solomon, LR1
Cosson, MA1
Benoist, JF2
Touati, G1
Déchaux, M1
Royer, N1
Grandin, L1
Jais, JP1
Boddaert, N1
Barbier, V1
Desguerre, I1
Campeau, PM1
Rabier, D1
Valayannopoulos, V1
Niaudet, P1
de Lonlay, P1
Vieira, C1
Cosmo, C1
Lucena, R1
Nyhan, WL1
Gargus, JJ1
Boyle, K1
Selby, R1
Koch, R1
Oh, R1
Brown, DL1
Roze, E1
Gervais, D1
Demeret, S1
Ogier de Baulny, H1
Zittoun, J1
Said, G1
Pierrot-Deseilligny, C1
Bolgert, F1
Hörster, F1
Baumgartner, MR1
Viardot, C1
Suormala, T1
Burgard, P1
Fowler, B1
Hoffmann, GF1
Garbade, SF1
Kölker, S1
Baumgartner, ER1
Shevell, MI2
Matiaszuk, N1
Ledley, FD1
Rosenblatt, DS3
Savage, D1
Gangaidzo, I1
Lindenbaum, J3
Kiire, C1
Mukiibi, JM1
Moyo, A1
Gwanzura, C1
Mudenge, B1
Bennie, A1
Sitima, J1
Allen, RH2
Stabler, SP2
Aspler, AL1
Pletcher, BA1
Fenton, WA1
Seashore, MR1
Snow, CF1
van't Hoff, W1
McKiernan, PJ1
Surtees, RA1
Leonard, JV1
de Jong, N1
Chin A Paw, MJ1
de Groot, LC1
Rutten, RA1
Swinkels, DW1
Kok, FJ1
van Staveren, WA1
Chanarin, I1
Deacon, R1
Lumb, M1
Perry, J1
Vieira-Makings, E1
Chetty, N1
Reavis, SC1
Metz, J1
Commodari, F1
Arnold, DL1
Sanctuary, BC1
Shoubridge, EA1
Kühne, T1
Bubl, R1
Baumgartner, R1
Carmel, R1
Watkins, D1
Goodman, SI1
Healton, EB1
Savage, DG1
Brust, JC1
Garrett, TJ1
Podell, ER1
Marcell, PD1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Effect of Oral Vitamin B12 Administration for Vitamin B12 Deficiency After Total Gastrectomy[NCT00699478]Phase 230 participants (Actual)Interventional2008-04-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

5 reviews available for methylmalonic acid and Nervous System Diseases

ArticleYear
The importance of methylmalonic acid dosage on the assessment of patients with neurological manifestations following bariatric surgery.
    Obesity surgery, 2011, Volume: 21, Issue:12

    Topics: Female; Gastric Bypass; Humans; Methylmalonic Acid; Nervous System Diseases; Obesity; Vitamin B 12 D

2011
Vitamin B12 deficiency.
    American family physician, 2003, Mar-01, Volume: 67, Issue:5

    Topics: Humans; Malabsorption Syndromes; Mental Disorders; Methylmalonic Acid; Nervous System Diseases; Prac

2003
High prevalence of cobalamin deficiency in the elderly.
    Transactions of the American Clinical and Climatological Association, 1996, Volume: 107

    Topics: Aged; Hematologic Diseases; Homocysteine; Humans; Mental Disorders; Methylmalonic Acid; Nervous Syst

1996
Laboratory diagnosis of vitamin B12 and folate deficiency: a guide for the primary care physician.
    Archives of internal medicine, 1999, Jun-28, Volume: 159, Issue:12

    Topics: Antibodies; Clinical Laboratory Techniques; Clinical Trials as Topic; Decision Trees; Diagnosis, Dif

1999
Cobalamin and folate: recent developments.
    Journal of clinical pathology, 1992, Volume: 45, Issue:4

    Topics: Anemia, Megaloblastic; Female; Folic Acid; Humans; Methylation; Methylmalonic Acid; Nervous System D

1992

Trials

2 trials available for methylmalonic acid and Nervous System Diseases

ArticleYear
Neuropsychiatric disturbances in presumed late-onset cobalamin C disease.
    Archives of neurology, 2003, Volume: 60, Issue:10

    Topics: Adolescent; Adult; Brain; Cobamides; Female; Fibroblasts; Homocysteine; Humans; Mental Disorders; Me

2003
Nutrient-dense foods and exercise in frail elderly: effects on B vitamins, homocysteine, methylmalonic acid, and neuropsychological functioning.
    The American journal of clinical nutrition, 2001, Volume: 73, Issue:2

    Topics: Aged; Aged, 80 and over; Cohort Studies; Cross-Sectional Studies; Dietary Supplements; Exercise; Fem

2001

Other Studies

14 other studies available for methylmalonic acid and Nervous System Diseases

ArticleYear
A case of vitamin B12 deficiency neurological syndrome in a young adult due to late-onset cobalamin C (CblC) deficiency: a diagnostic challenge.
    Biochemia medica, 2022, Jun-15, Volume: 32, Issue:2

    Topics: Adult; Female; Folic Acid; Humans; Methylmalonic Acid; Nervous System Diseases; Vitamin B 12; Vitami

2022
Vitamin B12-responsive neuropathies: A case series.
    Nutritional neuroscience, 2016, Volume: 19, Issue:4

    Topics: Administration, Oral; Adult; Aged; Aged, 80 and over; Antioxidants; Biotransformation; Female; Homoc

2016
Long-term outcome in methylmalonic aciduria: a series of 30 French patients.
    Molecular genetics and metabolism, 2009, Volume: 97, Issue:3

    Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Disease Progressio

2009
Progressive neurologic disability in methylmalonic acidemia despite transplantation of the liver.
    European journal of pediatrics, 2002, Volume: 161, Issue:7

    Topics: Acute Kidney Injury; Adult; Disease Progression; Female; Humans; Liver Transplantation; Metabolism,

2002
Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut0, mut-, cblA, cblB).
    Pediatric research, 2007, Volume: 62, Issue:2

    Topics: Adolescent; Adult; Age of Onset; Alkyl and Aryl Transferases; Amino Acid Metabolism, Inborn Errors;

2007
Varying neurological phenotypes among muto and mut- patients with methylmalonylCoA mutase deficiency.
    American journal of medical genetics, 1993, Mar-01, Volume: 45, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Cell Line; Child; Child, Preschool; Developmental Disabilities

1993
Vitamin B12 deficiency is the primary cause of megaloblastic anaemia in Zimbabwe.
    British journal of haematology, 1994, Volume: 86, Issue:4

    Topics: Anemia, Megaloblastic; Anemia, Pernicious; Erythrocyte Indices; Female; Folic Acid; Folic Acid Defic

1994
Clinical heterogeneity and prognosis in combined methylmalonic aciduria and homocystinuria (cblC).
    Journal of inherited metabolic disease, 1997, Volume: 20, Issue:4

    Topics: Adolescent; Age of Onset; Amino Acid Metabolism, Inborn Errors; Cause of Death; Child; Child, Presch

1997
Liver transplantation for methylmalonic acidaemia.
    European journal of pediatrics, 1999, Volume: 158 Suppl 2

    Topics: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Kidney Transplantation; Liver Transplan

1999
Methylmalonic acid metabolism and nervous-system fatty acids in cobalamin-deficient fruit bats receiving supplements of methionine, valine and isoleucine.
    The Biochemical journal, 1991, May-01, Volume: 275 ( Pt 3)

    Topics: Amino Acids; Animals; Brain; Chiroptera; Fatty Acids; Isoleucine; Liver; Methionine; Methylmalonic A

1991
1H NMR characterization of normal human cerebrospinal fluid and the detection of methylmalonic acid in a vitamin B12 deficient patient.
    NMR in biomedicine, 1991, Volume: 4, Issue:4

    Topics: Adult; Aged; Female; Humans; Magnetic Resonance Spectroscopy; Male; Methylmalonic Acid; Middle Aged;

1991
Maternal vegan diet causing a serious infantile neurological disorder due to vitamin B12 deficiency.
    European journal of pediatrics, 1991, Volume: 150, Issue:3

    Topics: Adult; Breast Feeding; Diet, Vegetarian; Female; Homocysteine; Humans; Infant; Methionine; Methylmal

1991
Hereditary defect of cobalamin metabolism (cblG mutation) presenting as a neurologic disorder in adulthood.
    The New England journal of medicine, 1988, Jun-30, Volume: 318, Issue:26

    Topics: 5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase; Adult; Diagnosis, Differential; Female; H

1988
Neuropsychiatric disorders caused by cobalamin deficiency in the absence of anemia or macrocytosis.
    The New England journal of medicine, 1988, Jun-30, Volume: 318, Issue:26

    Topics: Adolescent; Adult; Aged; Anemia; Anemia, Megaloblastic; Ataxia; Erythrocyte Indices; Erythrocytes; F

1988