THE PSEUDO-POSTERIOR LIMITING LAYER SYNDROME - A VITREORETINAL HEREDODEGENERATION WITH AUTOSOMAL-DOMINANT TRANSMISSION

A new vitreoretinal heredodegenerative syndrome with a high incidence (> 40%) of retinal detachment is described. It has been observed in fi ve families (altogether 27 subjects) without consanguinity. The affect ion is autosomal dominant, bilateral, and more often than not (> 70%) coupled with axial myopia exceeding 5 D. Peripheral retinal degenerati ons are found in about 90% of the eyes. The syndrome is characterized by a pathognomonic membrane-like structure in the vitreous cavity, the pseudo-posterior limiting layer. The pseudo-PLL is a purely intravitr eal phenomenon: in contrast to other vitreoretinal syndromes, there ar e no vitreous condensations connected to the retina.