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August 1958

Retinitis Pigmentosa, External Ophthalmoplegia, and Complete Heart BlockUnusual Syndrome with Histologic Study in One of Two Cases

Author Affiliations

Rochester, Minn.
Section of Ophthalmology and Section of Pathologic Anatomy, Mayo Clinic and Mayo Foundation. The Mayo Foundation, Rochester, Minn., is a part of the Graduate School of the University of Minnesota.

AMA Arch Ophthalmol. 1958;60(2):280-289. doi:10.1001/archopht.1958.00940080296016

Pigmentary degeneration of the retina (retinitis pigmentosa) may appear as an isolated finding unassociated with other systemic abnormalities. Often, however, the patient may present other abnormalities, a number of which occur frequently enough in association with retinitis pigmentosa to be recognized as a syndrome. The Lawrence-Moon-Biedl syndrome, consisting of pigmentary degeneration of the retina, obesity, mental retardation, hypogenitalism, and polydactyly, is such an example. There also may be other less common abnormalities, such as deafness, convulsions, ophthalmoplegia, cerebellar ataxia, and progeria (premature aging) associated with retinitis pigmentosa.

External ophthalmoplegia is one of the uncommoner abnormalities found associated with pigmentary degeneration of the retina. Barnard and Scholz1 reported four cases of this association and concluded that the association was more than just coincidental. Walsh2 agreed with them and stated that the association is not just fortuitous but rather represents a syndrome. He listed five cases that he had observed,

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