Document Detail


Coenzyme Q(10)-responsive ataxia: 2-year-treatment follow-up.
MedLine Citation:
PMID:  20629161     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We assessed the clinical outcome after coenzyme Q(10) (CoQ(10)) therapy in 14 patients presenting ataxia classified into two groups according to CoQ(10) values in muscle (deficient or not). We performed an open-label prospective study: patients were evaluated clinically (international cooperative ataxia rating scale [ICARS] scale, MRI, and videotape registration) at baseline and every 6 months during a period of 2 years after CoQ(10) treatment (30 mg/kg/day). Patients with CoQ(10) deficiency showed a statistically significant reduction of ICARS scores (Wilcoxon test: P = 0.018) after 2 years of CoQ(10) treatment when compared with baseline conditions. In patients without CoQ(10) deficiency, no statistically significant differences were observed in total ICARS scores after therapy, although 1 patient from this group showed a remarkable clinical amelioration. Biochemical diagnosis of CoQ(10) deficiency was a useful tool for the selection of patients who are good candidates for treatment as all of them responded to therapy. However, the remarkable clinical response in 1 case without CoQ(10) deficiency highlights the importance of treatment trials for identification of patients with CoQ(10)-responsive ataxia.
Authors:
Merce Pineda; Raquel Montero; Asuncion Aracil; Mar M O'Callaghan; Ana Mas; Carmen Espinos; Dolores Martinez-Rubio; Francesc Palau; Placido Navas; Paz Briones; Rafael Artuch
Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  25     ISSN:  1531-8257     ISO Abbreviation:  Mov. Disord.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-07-26     Completed Date:  2010-11-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1262-8     Citation Subset:  IM    
Affiliation:
Department of Pediatric Neurology, Hospital Sant Joan de Déu, Barcelona, Spain.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Ataxia / drug therapy*,  metabolism
Child
Child, Preschool
Female
Humans
Longitudinal Studies
Male
Neurologic Examination / methods
Statistics, Nonparametric
Time Factors
Treatment Outcome
Ubiquinone / analogs & derivatives*,  deficiency,  therapeutic use
Vitamins / therapeutic use*
Young Adult
Chemical
Reg. No./Substance:
0/Vitamins; 1339-63-5/Ubiquinone; 303-98-0/coenzyme Q10

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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