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Coenzyme Q 10 ‐responsive ataxia: 2‐year‐treatment follow‐up
Author(s) -
Pineda Merce,
Montero Raquel,
Aracil Asuncion,
O'Callaghan Mar M.,
Mas Ana,
Espinos Carmen,
MartinezRubio Dolores,
Palau Francesc,
Navas Placido,
Briones Paz,
Artuch Rafael
Publication year - 2010
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.23129
Subject(s) - ataxia , medicine , coenzyme q10 , rating scale , clinical trial , pediatrics , physical therapy , psychology , psychiatry , developmental psychology
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. © 2010 Movement Disorder Society