Structured Summary
Abstract
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)
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Classification
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Synonyms
13 entry terms
- Fukuhara Disease
- Fukuhara Syndrome
- MERRF
- Myoclonic Epilepsy Associated with Ragged-Red Fibers
- Myoclonic Epilepsy and Ragged Red Fibers
- Myoclonic Epilepsy 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
MeSH Record
Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
1993
MeSH Record
Previous Indexing
- Epilepsy, Myoclonic (1980-1992)
- Mitochondria, Muscle (1980-1992)
MeSH Hierarchy
Tree Numbers
AMA Style
References
- National Library of Medicine. MERRF Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D017243. http://id.nlm.nih.gov/mesh/2026/D017243
- MERRF Syndrome. In: Wikipedia. https://en.wikipedia.org/wiki/MERRF_syndrome
- MERRF Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q1881388