Gonadal Function in Males with Myotonic Dystrophy<sup>1</sup>

Harper, Peter S.; Penny, Robert; Foley, Thomas P.; Migeon, Claude J.; Blizzard, Robert M.

doi:10.1210/jcem-35-6-852

Cited by 54 publications

(16 citation statements)

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“…Harper et al (1972) reported that the serum levels of FSH and LH were significantly greater in the male patients with myotonic dystrophy than those observed in normal men. Segal et al (1975) who investigated the gonadotrophin responses to LH-RH injection in 8 men Correlation of serum levels of LH, FSH and testosterone to the histological classification of the testis in 9 patients with myotonic distrophy.…”

Section: Discussionmentioning

confidence: 94%

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Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Takeda

Ueda

1977

Acta Endocrinologica

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Variations in serum levels of LH and FSH after administration of synthetic LH-RH, and basal levels of serum testosterone were studied in 9 male patients with myotonic dystrophy. The degree of testicular damage, as determined histologically on biopsy specimens, was also studied. Results were as follows: 1) it was observed that both the basal and stimulated (maximal) levels of serum LH and FSH were significantly higher than levels found in 9 sex-and age-matched normal controls. 2) The basal level of serum testosterone was consistently lower in the patient group. One patient, however, showed a low normal level, which represented a statistically significant reciprocal relation to both LH and FSH levels as expressed on a logarithmic scale. 3) There was a significant trend which indicated that the higher the serum LH, FSH levels, or the lower the basal levels of serum testosterone, the more extensive was the damage to the seminiferous tubules. From these findings it was concluded that hypogonadism in patients with myotonic dystrophy, is characterized by the development of lesions in the seminiferous tubules.It is well known that hypogonadism is one of the cardinal features in myotonic dystrophy, and is considered to be due to primary testicular damage involving predominantly the seminiferous tubules. Harper et al. (1972) were the first to report the clinical usefulness for determining serum luteinizing hormone (LH) and follicle stimulating hormone (FSH) concentrations which were used as an additional help in the diagnosis of myotonic dystrophy.

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Section: Discussionmentioning

confidence: 94%

“…Harper et al (1972) were the first to report the clinical usefulness for determining serum luteinizing hormone (LH) and follicle stimulating hormone (FSH) concentrations which were used as an additional help in the diagnosis of myotonic dystrophy.…”

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confidence: 99%

Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Takeda

Ueda

1977

Acta Endocrinologica

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“…Histological studies have confirmed that the testicles of DM1 patients are characterized by an increase in the number and size of Leydig cells, as well as by tubular atrophy, hyalinization, and fibrosis of the seminiferous tubules and reduced spermatogenesis [3,19]. These structural gonadic changes correlate with endocrine findings [3,19,20], which point to markedly increased FSH levels, moderately increased or normal LH levels and moderately reduced T levels in male patients [21]. These results reflect findings in patients with generalized testicular failure, such as those with Klinefelter disease (XXY), in whom serum T levels are in the low or low-normal range and serum gonadotropins are subsequently overproduced [22], as well as in aging men, in whom hypogonadism is characterized by increased LH levels and reduced or normal T levels (primary and compensated hypogonadism) [16], along with progressively impaired spermatogenesis.…”

Section: Discussionmentioning

confidence: 95%

Hypogonadism in DM1 and its relationship to erectile dysfunction

et al. 2011

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Myotonic dystrophy type 1 (DM1) is characterized by both a premature appearance of age-related phenotypes and multiple organ involvement, which affects skeletal and smooth muscle as well as the eye, heart, central nervous system, and endocrine system. Although erectile dysfunction (ED) is a frequent complaint in patients with DM1, it has not been investigated in great depth. Hypogonadism, which is reported to be one of the physical causes of ED in the general population, frequently occurs in DM1. We planned this case-control study to evaluate the relationship between hypogonadism, as defined by the sexual hormone profile (FSH, LH, testosterone (T) and prolactin) and ED, as assessed by means of an internationally validated self-administered questionnaire (IIEF). DM1 patients had significantly increased mean levels of both gonadotropins (FSH and LH) (p < 0.0001) and a reduced mean level of T (p < 0.0001) when compared to controls. Twelve patients were eugonadic (normal LH, T, and FSH), while 18 displayed hormonal evidence of hypogonadism, characterized by tubular failure (increased FSH) in all the subjects and associated with interstitial failure in 14 subjects: seven with primary hypogonadism (increased LH and reduced T) and seven with compensated hypogonadism (increased LH and normal T). Patients with hormonal evidence of interstitial failure had a larger CTG expansion (p = 0.008), longer disease duration (p = 0.013), higher grade of disease (p = 0.004) and lower erectile function score (p = 0.02) than eugonadic patients. Impotence occurred in 13/14 hypogonadic patients with interstitial failure and in 5/12 eugonadic patients (p = 0.017, OR = 18.2).

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“…In contrast, the dystrophic hamster uteri were significantly enlarged. We (Harper, Penny, Foley, Migeon & Blizzard, 1972;Febres et al, 1975;Sagel, Distiller, Morley & Isaacs, 1975 ;Takeda & Ueda, 1977 (Howland, 1980). In women, severe weight loss is associated with reduced basal and LHRH-stimulated release of gonadotrophins (see for example Warren, 1977), although more recent studies suggest that submaximal doses of LHRH can give peak responses of FSH which are significantly greater than normal (Vigersky, Loriaux, Andersen, Mecklenburg & Vaitukaitis, 1976;Travaglini et al, 1976 …”

Section: Resultsmentioning

confidence: 99%

Abnormal gonadotrophin release from pituitaries of muscular dystrophic mice and hamsters

Wilkinson¹

1984

Reproduction

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Gonadal Function in Males with Myotonic Dystrophy¹

Cited by 54 publications

References 2 publications

Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Hypogonadism in DM1 and its relationship to erectile dysfunction

Abnormal gonadotrophin release from pituitaries of muscular dystrophic mice and hamsters

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Gonadal Function in Males with Myotonic Dystrophy1

Cited by 54 publications

References 2 publications

Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Pituitary-Gonadal Function in Male Patients With Myotonic Dystrophy - Serum Luteinizing Hormone, Follicle Stimulating Hormone and Testosterone Levels and Histological Damage of the Testis

Hypogonadism in DM1 and its relationship to erectile dysfunction

Abnormal gonadotrophin release from pituitaries of muscular dystrophic mice and hamsters

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Gonadal Function in Males with Myotonic Dystrophy¹