MERRF Syndrome

Overview

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Is a:

Disease or Syndrome

Definition:

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)

Synonyms:

Fukuhara Disease
Fukuhara Syndrome
Myoclonic Epilepsy and Ragged Red Fibers
Myoclonic Epilepsy with Ragged Red Fibers
Syndrome, Fukuhara
Syndrome, MERRF

Broader Topics:

Narrower Topics:

None

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