Chronic Progressive External Ophthalmoplegia

A mitochondrial myopathy characterized by slowly progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. Ragged-red fibers and atrophy are found on muscle biopsy. Familial and sporadic forms may occur. Disease onset is usually in the first or second decade of life, and the illness slowly progresses until usually all ocular motility is lost. (From Adams et al., Principles of Neurology, 6th ed, p1422)

Also Known As:

Progressive External Ophthalmoplegia; CPEO; Ocular Muscular Dystrophy; Ophthalmoplegia, Chronic Progressive External; Graefe's Disease; Ocular Myopathy of Von Graefe-Fuchs; Ophthalmoplegia, Progressive External; Disease, Graefe; Dystrophy, Ocular Muscular; External Ophthalmoplegia, Progressive; Muscular Dystrophies, Ocular; Muscular Dystrophy, Ocular; Myopathy, Mitochondrial Ocular; Ocular Muscular Dystrophies; Ocular Myopathy of Von Graefe Fuchs; Ocular Myopathy, Mitochondrial; Graefe Disease; Mitochondrial Ocular Myopathy

Musculoskeletal Diseases: 719
- Muscular Diseases: 10128
  - Mitochondrial Myopathies: 872
    - Chronic Progressive External Ophthalmoplegia: 575
Nervous System Diseases: 14178
- Neurologic Manifestations: 7102
  - Paralysis: 14164
    - Ophthalmoplegia: 867
      - Chronic Progressive External Ophthalmoplegia: 575
- Neuromuscular Diseases: 2005
  - Muscular Diseases: 10128
    - Mitochondrial Myopathies: 872
      - Chronic Progressive External Ophthalmoplegia: 575
- Cranial Nerve Diseases: 554
  - Ocular Motility Disorders: 514
    - Ophthalmoplegia: 867
      - Chronic Progressive External Ophthalmoplegia: 575
Eye Diseases: 3522
- Ocular Motility Disorders: 514
  - Ophthalmoplegia: 867
    - Chronic Progressive External Ophthalmoplegia: 575
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Mitochondrial Diseases: 21919
    - Mitochondrial Myopathies: 872
      - Chronic Progressive External Ophthalmoplegia: 575
Signs and Symptoms Pathological Conditions
- Pathologic Processes: 10023
  - Disease Attributes: 18
    - Chronic Disease: 25762
      - Chronic Progressive External Ophthalmoplegia: 575
- Signs and Symptoms
  - Neurologic Manifestations: 7102
    - Paralysis: 14164
      - Ophthalmoplegia: 867
        Chronic Progressive External Ophthalmoplegia: 575

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Disease

Drug/Important Bio-Agent (IBA)