The efficacy of coenzyme Q₁₀treatment in alleviating the symptoms of primary coenzyme Q₁₀deficiency: a systematic review

Abstract

Coenzyme Q10 (CoQ10) is necessary for mitochondrial electron transport. Mutations in CoQ10 biosynthetic genes cause primary CoQ10 deficiency (PCoQD) and manifest as mitochondrial disorders. It is often stated that PCoQD patients can be treated by oral CoQ10 supplementation. To test this, we compiled all studies describing PCoQD patients up to May 2022. We excluded studies with no data on CoQ10 treatment, or with insufficient description of effectiveness. Out of 303 PCoQD patients identified, we retained 89 cases, of which 24 reported improvements after CoQ10 treatment (27.0%). In five cases, the patient’s condition was reported to deteriorate after halting of CoQ10 treatment. 12 cases reported improvement in the severity of ataxia, and 5 cases in the severity of proteinuria. Only a subjective description of improvement was reported for four patients described as responding. All reported responses were partial improvements of only some symptoms. For PCoQD patients, CoQ10 supplementation is replacement therapy. Yet, there is only very weak evidence for the efficacy of the treatment. Our findings thus suggest a need for caution when seeking to justify the widespread use of CoQ10 for the treatment of any disease or as dietary supplement.HighlightsOnly 27% of primary CoQ10 deficiency patients benefited from CoQ10 supplementation.Studies of the effects of supplementation necessarily lacked controls and blinding.All reported positive responses to treatment only partially improved few symptoms.CoQ10 supplementation for the treatment of any disease should be questioned.Firm evidence of benefits requires randomize, controlled trials of CoQ10 therapy.Graphic Abstract

Combined Supplementation of Coenzyme Q10 and Other Nutrients in Specific Medical Conditions

“…A systematic review of the intervention in patients with primary CoQ 10 deficiencies, a total of 89 cases, reported symptom improvements in 27% of patients. Five cases even deteriorated after discontinuing CoQ 10 supplementation [ 69 ]. The intervention on this genetic condition generally required a high dosage, ranging from 5 to 50 mg/kg/day, of oral CoQ 10 supplementation to achieve favorable responses [ 70 ].…”