title: Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients.
abstract: We studied plasma and cerebrospinal fluid (CSF) concentrations of idebenone in five Friedreich ataxia patients on treatment with this antioxidant, and plasma and CSF ubiquinone-10 (Q (10)) concentrations in 15 controls. CSF idebenone concentrations were below the detection limit in 3 Friedreich ataxia patients and no association could be demonstrated between plasma and CSF idebenone values. Q (10) CSF concentrations (median: 2.25 nmol/L) were approximately 300 times lower than those of plasma (median: 0.77 micro mol/L). No correlation was observed between plasma and CSF Q (10) concentrations. A significantly positive correlation was observed between CSF total protein values (range 8.1 - 107.5 mg/dL; median: 29.5) and CSF Q (10) concentrations (Spearman test: r = 0.664; p = 0.01). Our findings suggest that less idebenone is distributed to the brain than to other tissues, although CSF does not appear to be an appropriate material for treatment monitoring of idebenone and other quinoid compounds.
authors.affiliation: Biochemistry Department, Hospital Sant Joan de Dèu, University of Barcelona, Esplugues, Spain. rartuch@hsjdbcn.org
authors.forename: R
authors.initials: R
authors.lastname: Artuch
authors.forename: A
authors.initials: A
authors.lastname: Aracil
authors.forename: A
authors.initials: A
authors.lastname: Mas
authors.forename: E
authors.initials: E
authors.lastname: Monrós
authors.forename: M A
authors.initials: MA
authors.lastname: Vilaseca
authors.forename: M
authors.initials: M
authors.lastname: Pineda
mesh_terms: D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000378:metabolism* / Q000627:therapeutic use; D016227:Benzoquinones / Q000134:cerebrospinal fluid / Q000378:metabolism* / Q000627:therapeutic use; D001812:Blood-Brain Barrier / Q000378:metabolism*; D016022:Case-Control Studies; D002648:Child; D003067:Coenzymes; D005621:Friedreich Ataxia / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000378:metabolism*; D006801:Humans; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000134:cerebrospinal fluid / Q000378:metabolism
chemical_list: D000975:Antioxidants; D016227:Benzoquinones; D003067:Coenzymes; D014451:Ubiquinone; C024989:coenzyme Q10; C036619:idebenone