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Decreased CSF valine concentration

MedGen UID:
1687649
•Concept ID:
C5139573
•
Finding
Synonym: Low valine levels in cerebrospinal fluid
 
HPO: HP:0500188

Definition

Any decreased amount from normal of valine in the cerebrospinal fluid. [from HPO]

Conditions with this feature

Branched-chain keto acid dehydrogenase kinase deficiency
MedGen UID:
766992
•Concept ID:
C3554078
•
Disease or Syndrome
Branched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD) is a neurodevelopmental disorder characterized by autism, impaired intellectual development, and microcephaly (Tangeraas et al., 2023).
See: Condition Record
Branched-chain keto acid dehydrogenase kinase deficiency

Recent clinical studies

Etiology

Leukocyte glutamate dehydrogenase and CSF amino acids in late onset ataxias.
Kaakkola S, Marnela KM, Oja SS, Icén A, Palo J
Acta Neurol Scand 1990 Nov;82(5):292-6. doi: 10.1111/j.1600-0404.1990.tb03306.x. PMID: 2281745
Leukocyte glutamate dehydrogenase and CSF amino acids in late onset ataxias.
Kaakkola S, Marnela KM, Oja SS, Icén A, Palo J
Acta Neurol Scand 1990 Oct;82(4):225-9. doi: 10.1111/j.1600-0404.1990.tb01610.x. PMID: 2270751
Amino acid concentrations in cerebrospinal fluid and plasma in Alzheimer's disease and healthy control subjects.
Basun H, Forssell LG, Almkvist O, Cowburn RF, Eklöf R, Winblad B, Wetterberg L
J Neural Transm Park Dis Dement Sect 1990;2(4):295-304. doi: 10.1007/BF02252924. PMID: 2078309
Changes in free amino acids of cerebrospinal fluid and plasma in various neurological diseases.
Iijima K, Takase S, Tsumuraya K, Endo M, Itahara K
Tohoku J Exp Med 1978 Oct;126(2):133-50. doi: 10.1620/tjem.126.133. PMID: 715764

Diagnosis

Amino acid metabolism in patients with propionic acidaemia.
Scholl-Bürgi S, Sass JO, Zschocke J, Karall D
J Inherit Metab Dis 2012 Jan;35(1):65-70. Epub 2010 Nov 27 doi: 10.1007/s10545-010-9245-9. PMID: 21113738
Cerebrospinal fluid metabolic profiles in multiple sclerosis and degenerative dementias obtained by high resolution proton magnetic resonance spectroscopy.
Nicoli F, Vion-Dury J, Confort-Gouny S, Maillet S, Gastaut JL, Cozzone PJ
C R Acad Sci III 1996 Jul;319(7):623-31. PMID: 9011324
Leukocyte glutamate dehydrogenase and CSF amino acids in late onset ataxias.
Kaakkola S, Marnela KM, Oja SS, Icén A, Palo J
Acta Neurol Scand 1990 Nov;82(5):292-6. doi: 10.1111/j.1600-0404.1990.tb03306.x. PMID: 2281745
Leukocyte glutamate dehydrogenase and CSF amino acids in late onset ataxias.
Kaakkola S, Marnela KM, Oja SS, Icén A, Palo J
Acta Neurol Scand 1990 Oct;82(4):225-9. doi: 10.1111/j.1600-0404.1990.tb01610.x. PMID: 2270751

Therapy

The effects of ammonia tolerance tests on the cerebrospinal fluid concentrations of amino acids and indoleamines in patients with liver cirrhosis.
Jonung T, Jeppsson B, Herlin P, Nobin A, Hultberg B
Scand J Gastroenterol 1990 May;25(5):422-8. doi: 10.3109/00365529009095510. PMID: 1694297
Clinical and biochemical effects of citalopram, a selective 5-HT reuptake inhibitor--a dose-response study in depressed patients.
Bjerkenstedt L, Edman G, Flyckt L, Hagenfeldt L, Sedvall G, Wiesel FA
Psychopharmacology (Berl) 1985;87(3):253-9. doi: 10.1007/BF00432703. PMID: 3936081

Clinical prediction guides

Amino acid metabolism in patients with propionic acidaemia.
Scholl-Bürgi S, Sass JO, Zschocke J, Karall D
J Inherit Metab Dis 2012 Jan;35(1):65-70. Epub 2010 Nov 27 doi: 10.1007/s10545-010-9245-9. PMID: 21113738
Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crises.
Wajner M, Coelho DM, Barschak AG, Araújo PR, Pires RF, Lulhier FL, Vargas CR
J Inherit Metab Dis 2000 Jul;23(5):505-12. doi: 10.1023/a:1005668431926. PMID: 10947205
Peptide histidine methionine and vasoactive intestinal peptide levels in human cerebrospinal fluid: age-related changes and absence of a correlation with serum prolactin.
Yasuda M, Minamitani N, Maeda K, Chihara K, Fujita K, Kobayashi I
Regul Pept 1993 Jul 23;46(3):565-73. doi: 10.1016/0167-0115(93)90258-a. PMID: 8210516
Amino acid concentrations in cerebrospinal fluid and plasma in Alzheimer's disease and healthy control subjects.
Basun H, Forssell LG, Almkvist O, Cowburn RF, Eklöf R, Winblad B, Wetterberg L
J Neural Transm Park Dis Dement Sect 1990;2(4):295-304. doi: 10.1007/BF02252924. PMID: 2078309

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