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April 1980

'Ophthalmoplegia Plus' or Kearns-Sayre Syndrome?

Author Affiliations

Neurological Institute 710 W 168th St New York, NY 10032

Arch Neurol. 1980;37(4):256. doi:10.1001/archneur.1980.00500530094031

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To the Editor.—  "Ophthalmoplegia plus" is a popular term, but some of us are convinced that one form of ophthalmoplegia is distinct from all others. The Kearns-Sayre syndrome can be recognized when atypical pigmentary degeneration of the retina and external ophthalmoplegia occur before age 15 years. When these three criteria are met, CSF protein level greater than 100 mg/dL and heart block are almost always found. Concomitant abnormalities include short stature, hearing loss, cerebellar syndromes, limb weakness, and mitochondrial abnormalities in skeletal muscle. In the few postmortem examinations, spongy degeneration of the brain has been a constant finding.Goodhue et al (Archives 36:481-484, 1979) described a patient with almost all of these abnormalities, but did not mention the Kearns-Sayre syndrome in discussing the differential diagnosis. That their patient actually had the Kearns-Sayre disorder, as I believe, could be documented by answers to the following simple questions: (1) Was the "retinitis

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