Ubidecarenone (coenzyme Q10)

High-dose CoQ10 (10–30 mg/kg/day, in adults up to 1,200–2,400 mg daily) is the only disease-modifying approach to the rare inherited primary CoQ10 deficiency. Early therapy in children prevents progression of encephalopathy and nephrotic syndrome; in adults it stabilises cerebellar ataxia. GeneReviews and international mitochondrial disease guidelines support this use.

Used in specialised centres with confirmed genetic diagnosis; not for self-prescription.

Coenzyme Q10 is considered as preventive therapy in episodic migraine, particularly in patients intolerant to topiramate, β-blockers, or amitriptyline. RCTs Sandor 2005 (n=42), Shoeibi 2017 (n=80), and the Zeng 2018 meta-analysis (5 RCTs) show 30-50% reduction in attack frequency at 300 mg/day (100 mg three times). Effect develops over 3 months. AAN/AHS 2012 and European Headache Federation guidelines classify CoQ10 as category C («possibly effective») – an acceptable option when first-line prophylaxis is contraindicated. EHF guidelines also list CoQ10 as an option in children.

In chronic heart failure, coenzyme Q10 has been studied as add-on therapy to guideline-directed treatment (ACE inhibitors/ARNI, β-blockers, mineralocorticoid receptor antagonists). The Q-SYMBIO trial (Mortensen 2014, n=420, 2 years) showed 43% reduction in the composite endpoint (mortality + adverse cardiovascular events) at 300 mg/day. The result is criticized for small sample size and design issues. ESC 2021 HF guidelines do not include CoQ10 as mandatory therapy but describe it as an «acceptable add-on option». AHA/ACC 2022 guidelines do not mention CoQ10.