Diagnostic and Therapeutic Problems in Chronic Progressive External Ophthalmoplegia (CPEO)

¹Sommer F., ²Fötzsch R., ¹Wollensak G., ¹Pillunat L. E.,
¹Universitätsklinikum Carl Gustav Carus, Klinik und Poliklinik für Augenheilkunde (Dresden)
²Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Neurologie (Dresden)

Purpose: Strictly CPEO is defined as paralysis of the external ocular muscles combined with ptosis. In ophthalmoplegia-plus-syndrome or Kearns-Sayre-Syndrome additional neurological or muscular deficits can be observed may, however, sometimes even decades after the beginning of ocular symptoms.
Method: Three patients with classical CPEO and one patient with Kearns-Sayre-Syndrome where included in the present study. The clinical examinations included electromyography, various serological parameters and histological biopsies.
Results: The onset of ocular symptoms with divergent strabism and ptosis could be dated back to the age of 7, 27, 38 and 44 years starting on one eye and progressing slowly over years to both eyes. Almost complete restriction of ocular motility („freezing“) was reached after 10, 21, 29 and 31 years after the first symptoms. In the Kearns-Sayre-Syndrome patient general decrease in cardio-respiratory fitness and occasional collapses were noted. In three patients ptosis and strabism surgery led to the relief of the ocular symptoms.
Conclusions: In CPEO respectively Kearns-Sayre-Syndrome the onset of the disease is variable and often at an older age. The final correct diagnosis is often delayed due to the mild beginning of the symptoms and the slow progression of the paralyses . In suspicious cases, early EMG and muscle biopsy examination may facilitate the diagnosis. Because of the progressive character of the disease, corrective muscle surgery should be considered with caution.

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