MELAS Syndrome

A mitochondrial disorder characterized by focal or generalized seizures, episodes of transient or persistent neurologic dysfunction resembling strokes, and ragged-red fibers on muscle biopsy. Affected individuals tend to be normal at birth through early childhood, then experience growth failure, episodic vomiting, and recurrent cerebral insults resulting in visual loss and hemiparesis. The cortical lesions tend to occur in the parietal and occipital lobes and are not associated with vascular occlusion. VASCULAR HEADACHE is frequently associated and the disorder tends to be familial. (From Joynt, Clinical Neurology, 1992, Ch56, p117)

Also Known As:

Syndrome, MELAS; MELAS; Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes; Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-Like Episodes; Myopathy, Mitochondrial-Encephalopathy-Lactic Acidosis-Stroke; Mitochondrial Myopathy, Lactic Acidosis, Stroke-Like Episode

Musculoskeletal Diseases: 751
- Muscular Diseases: 10368
  - Mitochondrial Myopathies: 879
    - Mitochondrial Encephalomyopathies: 430
      - MELAS Syndrome: 1136
Nervous System Diseases: 14737
- Central Nervous System Diseases: 4371
  - Brain Diseases: 16141
    - Cerebrovascular Disorders: 7764
      - Cerebral Small Vessel Diseases: 676
        MELAS Syndrome: 1136
    - Metabolic Brain Diseases: 218
      - Inborn Metabolic Brain Diseases: 2
        MELAS Syndrome: 1136
- Neuromuscular Diseases: 2048
  - Muscular Diseases: 10368
    - Mitochondrial Myopathies: 879
      - Mitochondrial Encephalomyopathies: 430
        MELAS Syndrome: 1136
Cardiovascular Diseases: 94258
- Vascular Diseases: 15340
  - Cerebrovascular Disorders: 7764
    - Cerebral Small Vessel Diseases: 676
      - MELAS Syndrome: 1136
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Inborn Genetic Diseases: 12342
  - Inborn Errors Metabolism: 1183
    - Inborn Metabolic Brain Diseases: 2
      - MELAS Syndrome: 1136
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10634
  - Mitochondrial Diseases: 22985
    - Mitochondrial Myopathies: 879
      - Mitochondrial Encephalomyopathies: 430
        MELAS Syndrome: 1136
  - Inborn Errors Metabolism: 1183
    - Inborn Metabolic Brain Diseases: 2
      - MELAS Syndrome: 1136
  - Metabolic Brain Diseases: 218
    - Inborn Metabolic Brain Diseases: 2
      - MELAS Syndrome: 1136

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Disease

Drug/Important Bio-Agent (IBA)