Zavadenko N N
Zh Nevropatol Psikhiatr Im S S Korsakova. 1990;90(9):25-8.
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.
对11例贝克尔进行性肌营养不良症（PMD）患者、9例埃尔布 - 罗斯型PMD患者和10例兰杜齐 - 德热里纳型PMD患者的垂体 - 睾丸功能进行了检查。患者年龄在21至45岁之间。采用放射免疫分析法测定血清促黄体生成素（LH）、促卵泡生成素（FSH）、催乳素、睾酮和雌二醇。还进行了性学研究。所有患PMD的患者组均表现为血液雌二醇显著升高。除雌激素缺乏外，部分患者还表现为睾酮水平低下、催乳素水平升高或降低、LH水平降低。据推测，所发现的激素改变，主要是雌激素过多，在遗传决定的肌营养不良过程中可能起适应作用。然而，另一方面，神经内分泌改变引发了PMD患者的性功能障碍。无论PMD的类型如何，患者常表现为性发育停滞、性欲减退、去男性化和女性化的客观体征、性功能障碍的主观主诉。
