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)
| Name | H7_PROGEXTOPHTHALMOPLEGIA |
|---|---|
| Long name | Progressive external ophthalmoplegia |
| Hospital Discharge registry | ICD-10: H494 |
| Cause of Death registry | ICD-10: H494 |
| Level in the ICD-hierarchy | 4 |
| First defined in version | DF2 |
| Latin name | Ophthalmoplegia externa progressiva |
| All | Female | Male | |
|---|---|---|---|
| Number of individuals | 10 | 7 | - |
| Unadjusted prevalence (%) | 0.00 | 0.01 | - |
| Mean age at first event (years) | 45.97 | 50.45 | - |
| Case fatality at 5-years (%) | 0.00 | 0.00 | - |
| All | Female | Male | |
|---|---|---|---|
| Median nb. of events per indiv. | 2.0 | 2.0 | - |
| Recurrence at 6 months (%) | 30.00 | 14.29 | - |
before Progressive external ophthalmoplegia
after Progressive external ophthalmoplegia
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