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MERRF Syndrome (Myoclonic Epilepsy and Ragged Red Fibers)

A mitochondrial encephalomyopathy characterized clinically by a mixed seizure disorder, myoclonus, progressive ataxia, spasticity, and a mild myopathy. Dysarthria, optic atrophy, growth retardation, deafness, and dementia may also occur. This condition tends to present in childhood and to be transmitted via maternal lineage. Muscle biopsies reveal ragged-red fibers and respiratory chain enzymatic defects. (From Adams et al., Principles of Neurology, 6th ed, p986)
Also Known As:
Myoclonic Epilepsy and Ragged Red Fibers; Fukuhara Syndrome; MERRF; Myoclonic Epilepsy Associated with Ragged-Red Fibers; Myoclonic Epilepsy with Ragged-Red Fibers; Myoclonus with Epilepsy with Ragged Red Fibers; Myoencephalopathy Ragged-Red Fiber Disease; Myoclonic Epilepsy Associated with Ragged Red Fibers; Myoencephalopathy Ragged Red Fiber Disease; Syndrome, Fukuhara; Syndrome, MERRF; Fukuhara Disease; Myoclonic Epilepsy with Ragged Red Fibers
Networked: 339 relevant articles (4 outcomes, 30 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. MELAS Syndrome (Syndrome, MELAS)
2. MERRF Syndrome (Myoclonic Epilepsy and Ragged Red Fibers)
3. Myoclonic Epilepsies (Myoclonic Encephalopathy)
4. Mitochondrial Diseases (Mitochondrial Disease)
5. Lactic Acidosis

Experts

1. Wei, Yau-Huei: 18 articles (01/2018 - 01/2002)
2. Finsterer, Josef: 11 articles (01/2020 - 07/2009)
3. Ma, Yi-Shing: 8 articles (01/2018 - 01/2002)
4. Suzuki, Tsutomu: 7 articles (01/2018 - 11/2002)
5. Mancuso, Michelangelo: 6 articles (01/2019 - 03/2005)
6. Filosto, Massimiliano: 5 articles (01/2019 - 03/2002)
7. Siciliano, Gabriele: 5 articles (01/2019 - 04/2006)
8. Zeviani, Massimo: 5 articles (01/2019 - 01/2006)
9. Hsieh, Mingli: 5 articles (01/2018 - 09/2011)
10. Liu, Chin-San: 5 articles (12/2013 - 05/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to MERRF Syndrome:
1. Mitochondrial DNA (mtDNA)IBA
2. Lys Transfer RNAIBA
3. Mitochondrial Proteins (Mitochondrial Protein)IBA
4. coenzyme Q10 (CoQ10)IBA
5. Oxidants (Oxidizing Agents)IBA
6. Cyclosporine (Ciclosporin)FDA LinkGeneric
7. Proteins (Proteins, Gene)FDA Link
8. NucleotidesIBA
9. Electron Transport Complex IV (Cytochrome c Oxidase)IBA
10. DNA (Deoxyribonucleic Acid)IBA

Therapies and Procedures

1. Therapeutics
2. Gastric Bypass (Roux-en-Y Gastric Bypass)
3. Prostatectomy (Retropubic Prostatectomy)
4. Gastrostomy
5. Cataract Extraction