Journal of Postgraduate Medicine
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CASE REPORT
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Year : 2015  |  Volume : 61  |  Issue : 3  |  Page : 200-202  

A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned

SR Chandra1, TG Issac2, N Gayathri3, N Gupta1, MM Abbas1 
1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
2 Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
3 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India

Correspondence Address:
S R Chandra
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka
India

Mitochondrial diseases have a special predilection to involve the brain in view of its high metabolic demand and the tendency for the formation of excitatory neurotransmitters when there is deficiency of intracellular ATP. These diseases have a great phenotypic variation and need a high degree of suspicion. However, some specific syndromes are well defined, both genotypically and phenotypically. Some of the drugs are potentially fatal mitochondrial poisons and an insight into that may be lifesaving as well as prevent serious morbidities.We report a typical case of myoclonic epilepsy with ragged red fibers (MERRF) with classical phenotype and genotype. There was rapid multiaxial deterioration with the introduction of sodium valproate which partly reversed on introducing mitochondrial cocktail and withdrawal of the offending drug.Sodium valproate, phenobarbitone, chloramphenicol and many anti-viral agents are mitochondrial poisons that increase the morbidity and mortality in patients with mitochondrial disease. More harm to the patient can be avoided with insight into this information.


How to cite this article:
Chandra S R, Issac T G, Gayathri N, Gupta N, Abbas M M. A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned.J Postgrad Med 2015;61:200-202


How to cite this URL:
Chandra S R, Issac T G, Gayathri N, Gupta N, Abbas M M. A typical case of myoclonic epilepsy with ragged red fibers (MERRF) and the lessons learned. J Postgrad Med [serial online] 2015 [cited 2021 May 11 ];61:200-202
Available from: https://www.jpgmonline.com/article.asp?issn=0022-3859;year=2015;volume=61;issue=3;spage=200;epage=202;aulast=Chandra;type=0


 
Tuesday, May 11, 2021
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