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Gonadal Dysfunction in Wolfram Syndrome: A Prospective Study.

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Gonadal dysfunction, including hypogonadism, is common in Wolfram syndrome (WFS). Early evaluation and management are crucial for patients

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Area of Science:

  • Endocrinology
  • Genetics
  • Reproductive Medicine

Background:

  • Wolfram syndrome (WFS), a rare monogenic neurodegenerative disorder, is characterized by diabetes mellitus, optic atrophy, sensorineural hearing loss, and diabetes insipidus.
  • Gonadal dysfunction, particularly hypogonadism, is an underrecognized but significant clinical feature of WFS, impacting patient quality of life.

Purpose of the Study:

  • To prospectively assess the prevalence and characteristics of gonadal dysfunction in a cohort of genetically confirmed Wolfram syndrome patients.
  • To evaluate hormonal profiles, reproductive parameters, and testicular/ovarian status in men and women with WFS.

Main Methods:

  • Prospective follow-up of 45 WFS patients (25 men, 20 women) with genetically confirmed diagnosis.
  • Men: hormonal evaluations, semen analysis, imaging, and testicular biopsies.
  • Women: assessment of menstrual history, hormonal analyses (including AMH), and imaging.

Main Results:

  • High prevalence of hypogonadism in men (76%), predominantly hypergonadotropic, with testicular damage observed on biopsies.
  • Significant reproductive abnormalities in men, including azoospermia and altered semen parameters.
  • Universal menstrual irregularities in women, with 20% experiencing premature menopause and evidence of early ovarian dysfunction.

Conclusions:

  • Gonadal dysfunction is a prevalent and critical feature of Wolfram syndrome, necessitating systematic screening from puberty onwards.
  • Comprehensive management addressing metabolic, psychological, and sexual health aspects is vital for improving WFS patients' long-term quality of life.
  • Early detection and intervention for gonadal dysfunction can mitigate significant health challenges and support patients' aspirations for relationships and family.