MERRF Syndrome

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

Musculoskeletal Diseases: 719
- Muscular Diseases: 10128
  - Mitochondrial Myopathies: 872
    - Mitochondrial Encephalomyopathies: 427
      - MERRF Syndrome: 339
Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Epilepsy: 37791
      - Generalized Epilepsy: 896
        Myoclonic Epilepsies: 1199
        Progressive Myoclonic Epilepsies: 434
        MERRF Syndrome: 339
      - Epileptic Syndromes: 883
        Myoclonic Epilepsies: 1199
        Progressive Myoclonic Epilepsies: 434
        MERRF Syndrome: 339
    - Metabolic Brain Diseases: 215
      - Inborn Metabolic Brain Diseases: 2
        MERRF Syndrome: 339
- Neuromuscular Diseases: 2005
  - Muscular Diseases: 10128
    - Mitochondrial Myopathies: 872
      - Mitochondrial Encephalomyopathies: 427
        MERRF Syndrome: 339
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Inborn Errors Metabolism: 1162
    - Inborn Metabolic Brain Diseases: 2
      - MERRF Syndrome: 339
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Mitochondrial Diseases: 21919
    - Mitochondrial Myopathies: 872
      - Mitochondrial Encephalomyopathies: 427
        MERRF Syndrome: 339
  - Inborn Errors Metabolism: 1162
    - Inborn Metabolic Brain Diseases: 2
      - MERRF Syndrome: 339
  - Metabolic Brain Diseases: 215
    - Inborn Metabolic Brain Diseases: 2
      - MERRF Syndrome: 339

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