Mesh term MERRF Syndrome
Browse to parent terms:Brain Diseases, Metabolic, Inborn
Mitochondrial Encephalomyopathies
Myoclonic Epilepsies, Progressive
Description
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)Search for this term in our Faculty Database
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