Introduction:

Several proteinopathies of the nervous system are associated with disturbances in components of the hypothalamic-pituitary-gonadal axis.

Objective:

To assess the relevance of components of the hypothalamic-pituitary-gonadal axis in the pathophysiology of proteinopathies of the nervous system.

Material and Methods:

A literature review was carried out from January to December 2018. Several databases were searched by using descriptors and Boolean operators. Advanced search strategy was used for the selection of articles, taking into account the methodological quality and validity of the studies.

Results:

Disturbances of the normal function of the hypothalamic-pituitary-gonadal axis were identified in proteinopathies of the nervous system. The most frequently reported disturbances were the increase in gonadotropin levels, mainly in luteinizing hormone in Alzheimer´s disease, and the decrease in testosterone levels in Alzheimer´s, Parkinson´s and Huntington´s diseases, and Amyotrophic Lateral Sclerosis, with the resulting worsening of the clinical phenotype. Preliminary evidence was obtained, which was pointing to a possible hypothalamic dysfunction in Spinocerebellar ataxia patients.

Conclusions:

Even when evidences were gathered supporting a link between the pathophysiology of proteinopathies of the nervous system and disturbances in components of the hypothalamic-pituitary-gonadal axis, deeper and more comprehensive studies will be needed to confirm these associations and to characterize the underlying molecular mechanisms.

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