Retinitis Pigmentosa with Macular Dystrophy. Report of a Familial Group.

IN an earlier communication (Brit. Ji. Obhthal., Vol. XXIV, p. 469, 1940), attention was drawn to the wide range of appearances seen in macular dystrophy. It was argued that the classical conception of a limited central lesion was not borne out by the actual data. In many cases there is considerable involvement of the peripheral zones, so much so that it was pointed out that to designate these cases as macular dystrophy is like speaking of retinitis pigmentosa as "equatorial degeneration of the retina "-an early and striking change rather than the whole picture would be stressed. The fine graduation of macular dystrophy into generalised dystrophy of the retina was exemplified by recorded cases in the literature showing retinitis pigmentosa with varied and early involvement of the central area, and a case was described (ibid., case 2, p. 485), showing clearly the association of tvpical retinitis pigmentosa with macular dystrophy of the atrophic type. In this particular case no hereditary or familial incidence could be established, so that there was some lack of validity in drawing on this case for an argument on clearly defined familial and hereditarv lesions. The present note supplies the necessary validity, as it concerns a familial group showing the association of the two lesions. Family history: Two of the four sons and one of the two daughters in this sibship are affected. The family is of consanguineous origin, the father being a cousin of the maternal grandfather. There were no miscarriages or early deaths in the sibship.