Coenzyme Q 10 improves lactic acidosis, strokelike episodes, and epilepsy in a patient with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes)

Clinical Neuropharmacology

Volumn 27, Issue 4, 2004, Pages 187-191

  Berbel Garcia, Angel ^a   Barbera Farre, Jose Ramon ^a   Porta Etessam, Jesús ^b   Martinez Salio, Antonio ^b   Cabello, Ana ^b   Gutierrez Rivas, Eduardo ^b   Campos, Yolanda ^b  

^a Hospital Mancha Centro   (Spain)

^b HOSPITAL UNIVERSITARIO 12 DE OCTUBRE   (Spain)

Author keywords

Coenzyme Q 10, efficacy, mitochondrial encephalomyopathies, MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes)

Indexed keywords

BECOZYME C FORTE; BICARBONATE; BICARBONATO; CARNICOR; CARNITINE; CREATINE KINASE; LACTIC ACID; LAMOTRIGINE; MULTIVITAMIN; UBIDECARENONE;

ADULT; AEROBIC METABOLISM; ARTICLE; BICARBONATE BLOOD LEVEL; BLOOD PH; CASE REPORT; CREATINE KINASE BLOOD LEVEL; DRUG EFFICACY; EPILEPSY; EXERCISE TOLERANCE; FEMALE; GENOTYPE PHENOTYPE CORRELATION; HUMAN; KEARNS SAYRE SYNDROME; LACTIC ACIDOSIS; LEIGH DISEASE; MELAS SYNDROME; MITOCHONDRIAL MYOPATHY; PRIORITY JOURNAL; STROKE;

ACIDOSIS, LACTIC; ADULT; ANTIOXIDANTS; BICARBONATES; CEREBROVASCULAR ACCIDENT; CREATINE; EPILEPSY; FEMALE; HUMANS; HYDROGEN-ION CONCENTRATION; LACTIC ACID; MELAS SYNDROME; META-ANALYSIS; UBIQUINONE;

EID: 4344684797     PISSN: 03625664     EISSN: None     Source Type: Journal    
DOI: 10.1097/01.wnf.0000137862.67131.bf     Document Type: Article

References (28)

1. Di Mauro S, Bonilla E, Zeviani M, et al. Mitochondrial myopathies. Ann Neurol. 1985;17:521-538.
2. Fukuhara N, Tokiguchi S, Shirakawa S, et al. Myoclonus epilepsy associated with red-ragged fibers (mitochondrial abnormalities): disease or syndrome? Light and electron-microscopic studies of two cases and review of the literature. J Neurol Sci. 1980;47:117-133.
3. Pavlakis SG, Phillips PC, Di Mauro S, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: a distinctive clinical syndrome associated with red-ragged fibers. Ann Neurol. 1984:481-488.
4. Hyman BK, Patten BM, Dosdon RF, et al. Mitochondrial abnormalities in progressive external ophthalmoplegia. Am J Ophthalmol. 1977;83:362-371.
5. Remes AM, Liimatta EV, Winqvist S, et al. Ubiquinone and nicotinamide treatment of patients with the 3243A->G mtDNA mutation. Neurology. 2002;59:1275-1277.
6. Barbiroli B, Frassineti C, Martinelli P, et al. Q 10 improves mitochondrial respiration in patients with mitochondrial cytopathies: an in vivo study on brain and skeletal muscle by phosphorous magnetic resonance spectroscopy. Cell Mol Biol(Noisy-le-grand) 1997;43:741-749.
7. Shinkai T, Nakashima M, Ohmori O, et al. Coenzyme Q10 improves psychiatric symptoms in adult-onset mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: a case report. Aust N Z J Psychiatry. 2000;34:1034-1035.
8. Abe K, Matsuo Y, Kadekawa J, et al. Effect of coenzyme Q10 in patients with mitochondrial, myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): evaluation by noninvasive tissue oximetry. J Neurol Sci. 1999;162:65-68.
9. Goda S, Hamada T, Ishimoto S, et al. Clinical improvement after administration of coenzyme Q10 in a patient with mitochondrial encephalomyopathy. J Neurol. 1987;234:62-63.
10. Chen RS, Huang CC, Chu NS, et al. Coenzyme Q10 treatment in mitochondrial encephalomyopathy: short-term, double-blind, crossover study. Eur Neurol. 1997;37:212-218.
11. Abe K, Fujimura H, Nishikawa Y, et al. 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 Neurol Scand. 1991;83:356-359.
12. Yamamoto M, Sato T, Anno M, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes with recurrent abdominal symptoms and coenzyme Q10 administration. J Neurol Neurosurg Psychiatry. 1987;50:1475-1481.
13. Berio A, Piazzi A. Improvement of Kearns-Sayre syndrome with controlled carbohydrate intake and coenzyme Q10 therapy. Ophthalmologica. 1994;208:342-343.
14. Calatayud MT, Diaz-Castroverde AG, Solar M, et al. Treatment with ubiquinone in Kearns-Sayre syndrome: improvement in ocular motility and visual evoked potentials. Neurologia. 1992;7:266-269.
15. Ihara Y, Namba R, Kuroda S, et al. Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone. J Neurol Sci. 1989;90:263-271.
16. Desnuelle C, Pellissier JF, Serratrice G, et al. Kearns-Sayre syndrome: mitochondrial encephalomyopathy caused by deficiency of the respiratory chain. Rev Neurol (Paris). 1989;145:842-850.
17. Bresolin N, Bet L, Binda A, et al. Clinical and biochemical correlations in mitochondrial myopathy treated with coenzyme Q10. Neurology. 1988;38:892-899.
18. Ogasahara S, Nishikawa Y, Yorifuji S, et al. Treatment of Kearns-Sayre syndrome with coenzyme Q10. Neurology. 1986;36:45-53.
19. Ogasahara S, Yorifuji S, Nishikawa Y, et al. Improvement of abnormal pyruvate metabolism and cardiac conduction defect with coenzyme Q10 in Kearns-Sayre syndrome. Neurology. 1985;35:372-377.
20. Nishikawa Y, Takahashi M, Yorifuji S, et al. Long-term coenzyme Q10 therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency: a 3D NMR study. Neurology. 1989;39:399-403.
21. Maldergem LV, Trijbels F, Di Mauro S, et al. Coenzyme Q-responsive Leigh's encephalopathy in two sisters. Ann Neurol. 2002;52:750-754.
22. Chan A, Reichmann H, Kögel A, et al. Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q-10 Therapy. J Neurol. 1998;245:681-685.
23. Matthews PM, Ford B, Dandurand RJ, et al. Coenzyme Q10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease. Neurology. 1993;43:884-890.
24. Bresolin N, Doriguzzi C, Ponzetto C. Ubidecarenone in the treatment of mitochondrial myopathies: a multi-center double-blind trial. J Neurol Sci. 1990;100:77-78.
25. Zierz S, von Wersebe O, Bleistein J, et al. Exogenous coenzyme Q fails to increase CoQ in skeletal muscle of two patients with mitochondrial cytopathies. J Neurol Sci. 1990;95:283-290.
26. Campos Y, Garcia A, del Hoyo P, et al. Two pathogenic mutations in the mitochondrial DNA tRNA Leu (UUR) gene (T3258C and A3280G) resulting in variable clinical phenotypes. Neuromuscul Disord. 2003;13:416-420.
27. Geromel V, Darin N, Chretien D, et al. Coenzyme Q10 and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits. Mol Genet Metab. 2002;77:21-30.
28. Castro-Gago M, Novo-Rodriguez MI, Pintosmartinez E, et al. Mitochondrial myopathies. Rev Neurol. 2000;31:263-282.