Mitochondrial disorders in children: Co-enzyme Q10

Study reference

Reason for exclusion

Abe K, Fujimura H, Nishikawa Y et al. (1991) Marked reduction in CSF lactate and pyruvate levels after CoQ therapy in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Acta Neurologica Scandinavica (6): 356–9

Study not prioritised (not the best available evidence: case report in an adult)

Abe K, Matsuo Y, Kadekawa J et al. (1999) Effect of coenzyme Q10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): evaluation by noninvasive tissue oximetry. Journal of the Neurological Sciences (1): 65–8

Study not prioritised (not the best available evidence: case reports in adults)

Apetauerova D, Scala S, Standaert D et al. (2015) Effects of coenzyme Q10 in PSP, A multicenter, randomized, placebo controlled, double-blind study. European journal of neurology (22): 292–

Poor relevance against search terms (not mitochondrial disorders)

Bendahan D, Desnuelle C, Vanuxem D et al. (1992) 31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies. Neurology (6): 1203–8

Study not prioritised (not the best available evidence: case reports in adults)

Butler MG, Dasouki M, Bittel D et al. (2003) Coenzyme Q10 levels in Prader-Willi syndrome: comparison with obese and non-obese subjects. American journal of medical genetics. Part A 119A (2): 168–71

Poor relevance against search terms (not mitochondrial disorders)

Chan A, Reichmann H, Kogel A et al. (1998) Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy. Journal of neurology (10): 681–685

Study not prioritised (not the best available evidence: non-comparative study in 9 adults)

Costeff H, Apter N, Elpeleg ON et al. (1998) Ineffectiveness of oral coenzyme Q10 supplementation in 3-methylglutaconic aciduria, type 3. Brain and Development (1): 33–35

Poor relevance against search terms (not mitochondrial disorders)

Emmanuele V, Lopez LC, Lopez L et al. (2012) Heterogeneity of coenzyme Q10 deficiency: patient study and literature review. Archives of neurology (8): 978–83

Not a relevant study (narrative review)

Gempel K, Topaloglu H, Talim B et al. (2007) The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring-flavoprotein dehydrogenase (ETFDH) gene. Brain: A journal of neurology 130 (Pt 8): 2037–44

Study not prioritised (not the best available evidence: case reports of 7 adults and children)

Gold R, Seibel P, Reinelt G et al. (1996) Phosphorus magnetic resonance spectroscopy in the evaluation of mitochondrial myopathies: results of a 6-month therapy study with coenzyme Q. European Neurology (4): 191–6

Study not prioritised (not the best available evidence: non-comparative study in 8 adults)

Ihara Y, Namba R, Kuroda S et al. (1989) Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone. Journal of the Neurological Sciences (3): 263–71

Study not prioritised (not the best available evidence: case reports in adults and young people)

Li CW, Zhuang ZY, Zhang SK (2009) [CIinical study on treatment of Leber hereditary optic neuropathy]. Chinese journal of integrated traditional and Western (12): 1078–80

Non- English language study

Muller W, Reimers CD, Berninger T et al. (1990) Coenzyme Q10 in ophthalmoplegia plus - a double blind, cross over therapeutic trial. Journal of the Neurological Sciences 98 Supplement: 442

Abstract only

Neustadt J (2006) Mitochondrial dysfunction and disease. Integrative Medicine (3): 14–20

Unable to source study

Newman NJ (2011) Treatment of Leber hereditary optic neuropathy. Brain: A journal of neurology 134 (Pt 9): 2447–50

Not a relevant study (a narrative review)

Nishikawa K, Takahashi M, Yorifuji S et al. (1989) Long-term coenzyme Q10 therapy for a mitochondrial encephalomyopathy with cytochrome coxidase deficiency: a 31P NMR study. Neurology (3): 399–403

Study not prioritised (not the best available evidence: case report in an adult)

Ogasahara S, Engel AG, Frens D et al. (1989) Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy. Proceedings of the National Academy of Sciences of the United States of America (7): 2379–82

Poor relevance against search terms (study assessing co-enzyme Q10 levels in muscle not assessing treatment with co-enzyme Q10)

Ogasahara S, Nishikawa Y, Yorifuji S et al. (1986) Treatment of Kearns-Sayre syndrome with coenzyme Q10. Neurology (1): 45–53

Study not prioritised (not the best available evidence: non-comparative study in 5 adults)

Omata T, Fujii K, Takanashi J-I et al. (2016) Drugs indicated for mitochondrial dysfunction as treatments for acute encephalopathy with onset of febrile convulsive status epileptics. Journal of the Neurological Sciences (360): 57–60

Poor relevance against search terms (not mitochondrial disorders)

Panetta J, Smith LJ, Boneh A (2004) Effect of high-dose vitamins, coenzyme Q and high-fat diet in paediatric patients with mitochondrial diseases. Journal of Inherited Metabolic Disease (4): 487–98

Study not prioritised (not the best available evidence: study assessing combination of treatments)

Peterson PL (1995) The treatment of mitochondrial myopathies and encephalomyopathies. Biochimica et biophysica acta (1): 275–80

Study not prioritised (not the best available evidence: study assessing combination of treatments)

Pineda M, Montero R, Aracil A et al. (2010) Coenzyme Q (10)-responsive ataxia: 2-year-treatment follow-up. Movement disorders : Official journal of the Movement Disorder Society (9): 1262–68

Poor relevance against search terms (not mitochondrial disorders)

Rodriguez MC, MacDonald JR, Mahoney DJ et al. (2007) Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders. Muscle and Nerve (2): 235–42

Study not prioritised (not the best available evidence: study assessing combination treatment)

Sacconi S, Trevisson E, Salviati L et al. (2010) Coenzyme Q10 is frequently reduced in muscle of patients with mitochondrial myopathy. Neuromuscular disorders: NMD (1): 44–48

Poor relevance against search terms (study assessing co-enzyme Q10 levels in muscle not assessing treatment with co-enzyme Q10)

Scarlato G, Bresolin N, Moroni I et al. (1991) Multicenter trial with ubidecarenone: treatment of 44 patients with mitochondrial myopathies. Revue neurologique (6–7): 542–48

Duplicate study

Shults CW, Haas R (2005) Clinical trials of coenzyme Q10 in neurological disorders. BioFactors (1–4): 117–26

Not a relevant study (a narrative review)

Sobreira C, Hirano M, Shanske S et al. (1997) Mitochondrial encephalomyopathy with coenzyme Q10 deficiency. Neurology (5): 1238–43

Study not prioritised (not the best available evidence: case report in adult)

Sommerville RB, Zaidman CM, Pestronk A (2013) Coenzyme Q10 deficiency in children: frequent type 2C muscle fibers with normal morphology. Muscle & nerve (5): 722–726

Poor relevance against search terms (study assessing co-enzyme Q10 deficiency)

Suzuki S, Hinokio Y, Ohtomo M et al. (1998) The effects of coenzyme Q10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation. Diabetologia 5:584–8

Study not prioritised (not the best available evidence: non-comparative study in adults)