Abstract
Marinesco-Sjogren syndrome and hypergonadotropic hypogonadism were observed in 2 kindreds, and occurred together in 9 of 10 affected individuals. The last patient had Marinesco-Sjogren syndrome without manifestations of hypogonadism, and similar findings were observed in 2 affected sisters from a 3rd kindred. On the hypothesis that the concurrence of Marinesco-Sjogren syndrome and hypergonadotropic hypogonadism was caused by linkage, a lod score analysis was conducted. Four sibships in the 2 kindreds were informative with respect to linkage. At the recombination fraction 0.05, the lod score exceeded 3. If linkage causes the concurrence of Marinesco-Sjogren syndrome and hypergonadotropic hypogonadism in these kindreds, the linkage is close. No linkage was observed between the clinical syndromes and 17 marker systems.