Neuropediatrics 2004; 35(2): 95-98
DOI: 10.1055/s-2004-815830
Original Article

Georg Thieme Verlag KG Stuttgart · New York

Cerebrospinal Fluid Concentrations of Idebenone in Friedreich Ataxia Patients

R. Artuch1 , A. Aracil2 , A. Mas3 , E. Monrós4 , M. A. Vilaseca1 , M. Pineda2
  • 1Biochemistry Department, Hospital Sant Joan de Dèu, University of Barcelona, Esplugues, Spain
  • 2Neurology Department, Hospital Sant Joan de Dèu, University of Barcelona, Esplugues, Spain
  • 3Pharmacy Department, Hospital Sant Joan de Dèu, University of Barcelona, Esplugues, Spain
  • 4Genetics Department, Hospital Sant Joan de Dèu, University of Barcelona, Esplugues, Spain
Further Information

Publication History

Received: August 11, 2003

Accepted after Revision: November 30, 2003

Publication Date:
04 May 2004 (online)

Preview

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 (Q10) 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. Q10 CSF concentrations (median: 2.25 nmol/L) were approximately 300 times lower than those of plasma (median: 0.77 µmol/L). No correlation was observed between plasma and CSF Q10 concentrations. A significantly positive correlation was observed between CSF total protein values (range 8.1 - 107.5 mg/dL; median: 29.5) and CSF Q10 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.

References

Rafael Artuch

Biochemistry Department, Hospital Sant Joan de Deu

Passeig Sant Joan de Déu 2

08950 Esplugues Barcelona

Spain

Email: rartuch@hsjdbcn.org