Mitochondrial myopathy

File:562px-Diagram of a human mitochondrion.png
Simplified structure of a typical mitochondrion
ICD-10 | G71.3
MeSH | D017240

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

## Overview

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Mitochondrial myopathy are a type of myopathy associated with mitochondrial disease.

Examples include:

### Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like syndrome (MELAS)

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*     * varying degrees of cognitive impairment and dementia
* lactic acidosis
* strokes
* transient ischemic attacks
* hearing loss
* dysmotility
* weight loss

### Myoclonic epilepsy and ragged-red fibers (MERRF)

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*     * progressive myoclonic epilepsy
* clumps of diseased mitochondria accumulate in the subsarcolemmal region of the muscle fiber and appear as "ragged-red fibers" when muscle is stained with modified Gomori trichrome stain
* short stature

### Kearns-Sayre syndrome (KSS)

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*     * external ophthalmoplegia
* cardiac conduction defects
* sensory-neural hearing loss

### Progressive external ophthalmoplegia (PEO)

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*     * progressive ophthalmoparesis is the cardinal feature
* symptomatic overlap with many other mitochondrial myopathies

Template:PNS diseases of the nervous system Template:Mitochondrial diseases

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