CoQ10 deficiency can present in infancy as a severe encephalomyopathy or multisystemic mitochondrial disease, with features such as hypotonia, developmental delay, intractable seizures, lactic acidosis, cardiomyopathy, and failure to thrive. Reports of infantile‑onset multisystem CoQ10 deficiency describe very early presentations, sometimes in the neonatal period, with rapid neurologic deterioration and involvement of brain, heart, kidney, and liver, and many affected children die in the first months or years of life despite intensive care. The important clinical point is that, although outcomes are often poor in the most severe cases, some infants and young children show neurologic improvement or stabilization when CoQ10 deficiency is recognized early and high‑dose CoQ10 supplementation is started promptly, which is why this diagnosis is considered a treatable cause of infantile encephalomyopathy This medication is commonly used for High Cholesterol.
Quinzii CM, Hirano M. Coenzyme Q and mitochondrial disease. Dev Disabil Res Rev. 2010;16(2):183-8. Chen RS, Huang CC, Chu NS. Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study. Eur Neurol. 1997;37(4):212-8. Boitier E, Degoul F, Desguerre I, Charpentier C, François D, Ponsot G, Diry M, Rustin P, Marsac C. A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency. J Neurol Sci. 1998;156(1):41-6. Sobreira C, Hirano M, Shanske S, Keller RK, Haller RG, Davidson E, Santorelli FM, Miranda AF, Bonilla E, Mojon DS, Barreira AA, King MP, DiMauro S. Mitochondrial encephalomyopathy with coenzyme Q10 deficiency. Neurology. 1997 May;48(5):1238-43.
Some side effects may be linked to nutrient depletion caused by this medication.
Fibrates are a class of medications used to lower cholesterol and triglycerides. They work by activating peroxisome proliferator-activated receptors (PPARs), which are transcription factors that regulate gene expression. Some studies have suggested that fibrates may interfere with the absorption of Coenzyme Q10 (CoQ10), a nutrient that is important for mitochondrial function. COQ10 supplementation should be considered to avoid a potential deficiency when taking this drug therapy.
Research demonstrates that fibrates, particularly fenofibrate, are known to increase homocysteine levels. Elevated levels of homocysteine is associated with increased risk of a range of health problems including atherosclerosis, stroke, and neurological diseases, and vitamin Bs including vitamin B12 helps facilitate the breakdown of homocysteine and may be helpful in reducing homocysteine levels.