Summary
Function of the hypothalamic-pituitary axis was investigated in seven patients with myotonic dystrophy (MD).
HGH and ACTH secretion were normal.
TSH response to TRH was impaired in about half the cases, without concomitant thyroid dysfunction. LH and FSH levels were often elevated, with inconsistant response to LH-RH stimulation. Gonadotrophin disturbances in MD have previously been attributed to a primary gonadal lesion, characteristically seen in this disease. High prolactin levels in six of our seven patients however suggest that gonadal failure may also be due to hyperprolactinemia through the direct anti-gonadal effect of prolactin and its interference with hypothalamic-pituitary regulation of gonadotrophin secretion.
Zusammenfassung
Bei sieben Patienten mit Myotonia Dystrophica (MD) wurde die hypothalamische-hypophysäre Funktion untersucht. Die Sekretion von HGH und ACTH waren normal. Die TSH-Produktion nach Stimulation mit TRH war in ungefähr der Hälfte der Fälle gestört, ohne begleitende Schilddrüsendysfunktion. LH- und FSH-Werte waren öfters erhöht mit wechselnder Reaktion auf LHRH-Stimulation.
Die bisher beschriebenen Gonadotrophinstörungen bei der MD wurden bisher immer dem primären Gonadenleiden zugeschrieben. Erhöhte Prolactin-Werte in sechs unserer sieben Patienten sprechen zugunsten der Möglichkeit, daß die Gonadeninsuffizienz auch eine Folge der Hyperprolactinämie sein könnte sowohl durch eine direkte antigonadale Wirkung, wie durch Interferenz mit der hypothalamisch-pituitären Regulation der Gonadotrophin-Sektretion.
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Abbreviations
- ACTH:
-
adrenocorticotrophic hormone
- FSH:
-
follicle stimulating hormone
- HGH:
-
human growth hormone
- LH:
-
luteinizing hormone
- LH-RH:
-
luteinizing hormone-releasing hormone
- PRL:
-
prolactin
- TRH:
-
thyrotrophic releasing hormone
- TSH:
-
thyroid stimulating hormone
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Mahler, C., Parizel, G. Hypothalamic-pituitary function in myotonic dystrophy. J Neurol 226, 233–242 (1982). https://doi.org/10.1007/BF00313396
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DOI: https://doi.org/10.1007/BF00313396