Skip to main content Accessibility help
×
Home

Regain of visuospatial capacity after coenzyme Q10 in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: a case report

  • Che-Sheng Chu (a1), Chin-Liang Chu (a1), Hong-Ern Liu (a1) and Ti Lu (a1)

Abstract

Objective: MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a neurodegenerative disorder caused by mitochondrial dysfunction. Multiple systems of the body, including cognitive function and heart conduction, can be affected by this disorder. We report a case with global cognitive impairment.

Method: A single-case report.

Results: The patient got improved cognitive function, especially visuospatial function, under coenzyme Q10 treatment.

Conclusion: First, coenzyme Q10 may give some benefit to control MELAS. Second, cognitive functions and intellectual abilities decline with disease progression. Routine neuropsychological tests should be performed.

Copyright

Corresponding author

Ti Lu, Department of Psychiatry, Kaohsiung Veterans General Hospital, No. 386, Ta-Chung1st Road, Kaohsiung 813, Taiwan. Tel: +88673422121x7093; Fax: +88673468346; E-mail: youngtzuchi@hotmail.com

References

Hide All
1.Pavlakis, SG, Phillips, PC, DiMauro, S et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol 1984;16:481488.
2.Shapira, Y.Clinical aspects of mitochondrial encephalomyopathies. International Pediatrics 1993;8:225232.
3.Goto, Y, Nonaka, I, Horai, S.A mutation in the tRNALeu (UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature 1990;348:651653.
4.Finsterer, J.Mitochondrial disorders, cognitive impairment and dementia. J Neurol Sci 2009;283:143148.
5.Turconi, AC, Benti, R, Castelli, E et al. Focal cognitive impairment in mitochondrial encephalomyopathies: a neuropsychological and neuroimaging study. J Neurol Sci 1999;170:5763.
6.Remes, AM, Liimatta, EV, Winqvist, S et al. Ubiquinone and nicotinamide treatment of patients with the 3243A->G mtDNA mutation. Neurology 2002;59:12751277.
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;34:10341035.
8.Goda, S, Hamada, T, Ishimoto, S et al. Clinical improvement after administration of coenzyme Q10 in a patient with mitochondrial encephalomyopathy. J Neurol 1987;234:6263.
9.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;83:356359.
10.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;90:263271.
11.Staub, F, Mackert, B, Kempski, O, Peters, J, Baethmann, A.Swelling and death of neuronal cells by lactic acid. J Neurol Sci 1993;119:7984.
12.Kaufmann, P, Shungu, DC, Sano, MC et al. Cerebral lactic acidosis correlates with neurological impairment in MELAS. Neurology 2004;62:12971302.
13.Berbel-Garcia, A, Barbera-Farre, JR, Etessam, JP et al. Coenzyme Q 10 improves lactic acidosis, strokelike episodes, and epilepsy in a patient with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes). Clin Neuropharmacol 2004;27:187191.
14.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;83:356359.
15.Finsterer, J.Overview on visceral manifestations of mitochondrial disorders. Neth J Med 2006;64:6171.
16.Neustadt, J, Pieczenik, SR.Medication-induced mitochondrial damage and disease. Mol Nutr Food Res 2008;52:780788.
17.Baik, R, Chae, JH, Lee, YM, Kang, HC, Lee, JS, Kim, HD.Electrocardiography as an early cardiac screening test in children with mitochondrial disease. Korean J Pediatr 2010;53:644647.
18.Matthews, PM, Ford, B, Dandurand, RJ et al. Coenzyme Q10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease. Neurology 1993;43:884890.

Keywords

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed