[Study of the hypophyseal-gonadal system in patients with hereditary muscular dystrophy]

Hypophyseal-testicular function was examined in 11 patients with Becker's progressive myodystrophy (PMD), in 9 with Erb-Roth's PMD and in 10 with Landouzy-Dejerine's PMD. The patients' age ranged from 21 to 45 years. Blood serum LH, FSH, prolactin, testosterone and estradiol were measured by RIA. A sexological study was carried out as well. All the patients' groups suffering from PMD were characterized by a significant rise of blood estradiol. Apart from hypoestrogenemia, some of the patients manifested hypotestosteronemia, hyper- and hypoprolactinemia, reduction of the LH level. It is assumed that the identified hormonal alterations, primarily hyperestrogenemia, may play an adaptation role under the conditions of the genetically determined myodystrophic process. On the other hand, however, the neuroendocrine alterations provoked sexual disorders in PMD patients. The patients often manifested arrest of sexual development, libido decrease, objective signs of demasculinization and feminization, subjective complaints of sexual dysfunction whatever the PMD pattern.