Page last updated: 2024-10-18

glutaric acid and Leigh Disease

glutaric acid has been researched along with Leigh Disease in 3 studies

glutaric acid: RN given refers to parent cpd
glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.

Leigh Disease: A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include X-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the PYRUVATE DEHYDROGENASE COMPLEX; CYTOCHROME-C OXIDASE; ATP synthase subunit 6; and subunits of mitochondrial complex I. (From Menkes, Textbook of Child Neurology, 5th ed, p850).

Research Excerpts

ExcerptRelevanceReference
"The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3)."3.80Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome. ( Arvinda, HR; Bharath, MM; Bindu, PS; Gayathri, N; Govindaraju, C; Khan, NA; Nagappa, M; Ranjith, D; Sinha, S; Sonam, K; Taly, AB; Thangaraj, K, 2014)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19902 (66.67)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's1 (33.33)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Bindu, PS1
Taly, AB1
Sonam, K1
Govindaraju, C1
Arvinda, HR1
Gayathri, N1
Bharath, MM1
Ranjith, D1
Nagappa, M1
Sinha, S1
Khan, NA1
Thangaraj, K1
Rutledge, JN1
Hilal, SK1
Silver, AJ1
Defendini, R1
Fahn, S1
Stutchfield, P1
Edwards, MA1
Gray, RG1
Crawley, P1
Green, A1

Other Studies

3 other studies available for glutaric acid and Leigh Disease

ArticleYear
Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.
    The British journal of radiology, 2014, Volume: 87, Issue:1034

    Topics: Child; Cohort Studies; Diagnosis, Differential; Early Diagnosis; Female; Giant Axonal Neuropathy; Gl

2014
Magnetic resonance imaging of dystonic states.
    Advances in neurology, 1988, Volume: 50

    Topics: Adolescent; Adult; Brain; Child; Child, Preschool; Dystonia; Ferric Compounds; Glutarates; Humans; I

1988
Glutaric aciduria type I misdiagnosed as Leigh's encephalopathy and cerebral palsy.
    Developmental medicine and child neurology, 1985, Volume: 27, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Cerebral Palsy; Child; Child, Presc

1985