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Ophthalmoplegic retinal degeneration syndrome (barnard-scholz syndrome)


Ophthalmoplegic Retinal Degeneration Syndrome (Barnard-Scholz Syndrome)

General: Onset at all ages (see Kearns-Sayre Syndrome).

Ocular: Unilateral or bilateral progressive weakness of muscles of eyelids, up to severe ptosis; progressive ocular myopathy up to complete ophthalmoplegia; retinitis pigmentosa.



Clinical: Facial, neck, and shoulder muscle weakness; hearing defects; heart block.

Barnard RI, Scholz RO. Ophthalmoplegia and retinal degeneration. Am J Ophthalmol 1944; 27:621.

Geeraets WJ Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Kiloh LG, Nevin S. Progressive dystrophy of the external ocular muscles (ocular myopathy). Brain 1951; 774: 115-l43.




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