THE ASSOCIATION of retinal degeneration and neurologic disorders is well known. Prominent among these is cerebromacular degeneration, or amaurotic familial idiocy. The association of pigmentary degeneration of the retina with neurologic disorders is best known in the Laurence-Moon-Biedl syndrome, which includes mental retardation. Still other heredodegenerative diseases of the nervous system reported to be associated with pigmentary degeneration of the retina are Friedreich's ataxia,1 progressive dementia,2 the cerebellopyramidal syndrome3 and spastic4 and flaccid5 paraplegias.
In 1944, Barnard and Scholz6 reported 4 cases of ophthalmoplegia with pigmentary degeneration of the retina and concluded that they all represented a single syndrome with a common etiologic factor.
Walsh7 concurred in this opinion and described 4 cases of a similar disorder. His case 224 appears to be the same as case 4 of Barnard and Scholz; thus a total of 7 such cases have been reported.