At the Fourth ISCERG Symposium, held in Hakone, Japan, in I966 Straub (I966) reviewed the subject of unilateral retinitis pigmentosa and made a number of important observations. Up to then only 34 cases of unilateral retinitis pigmentosa had been reported. Only one of them was of unilateral disease without pigment (Jacobson and Stephens, I962). Rickers and Domarus (I974) stated that nearly ioo cases of unilateral retinitis pigmentosa had been described since the first report in I865. They thought that the diagnosis could be verified in only about 20 cases.Straub emphasized the clinical value of electroretinography (ERG) in establishing the truly unilateral nature of such cases, indicating that the ERG response must be normal in the unaffected eye. Others have long recognized that many cases of supposed unilateral retinitis pigmentosa were actually instances of highly asymmetric bilateral disease in which the 'normal' eye eventually showed the disorder, but only much later. Franceschetti, Frangois, and Babel (I963) showed that the ERG response in such cases was impaired in both eyes. This significant clinical feature distinguishes genuine unilateral disease from 'pseudounilateral' (that is, asymmetric bilateral) retinitis pigmentosa.Henkes (I964) found that the electro-oculogram was bilaterally disturbed in many instances of presumed unilateral retinitis pigmentosa but normal in the unaffected eye in truly unilateral cases. Thus measuring the resting potential of the eyes as well as the ERG is of value.If unilateral retinitis pigmentosa is uncommon, cases without pigmentation are even rarer. We report here a case of unilateral retinitis pigmentosa sine pigmento. OphthalmoscopyThe fundus of each eye was examined through pupils dilated with i per cent tropicamide and io per cent viscous phenylephrine hydrochloride ophthalmic solutions (Fig. i)
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