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Secondary amenorrhea in two sisters with hypogonadotropic hypogonadism and progressive cerebellar ataxia

Authors
Journal
Brain and Development
0387-7604
Publisher
Elsevier
Publication Date
Volume
11
Issue
6
Identifiers
DOI: 10.1016/s0387-7604(89)80028-2
Keywords
  • Secondary Amenorrhea
  • Hypogonadotropic Hypogonadism
  • Progressive Cerebellar Ataxia
Disciplines
  • Medicine

Abstract

The association of familial hypogonadism with progressive cerebellar ataxia is only rarely encountered. Both primary hypergonadotropic and secondary hypogonadotropic hypogonadism may appear with cerebellar ataxia. However, many of these patients suffer from a variety of neurological and/or somatic malformations. Females, which are relatively rarely affected, display primary amenorrhea. In this report, two sisters presented with secondary amenorrhea prior to the appearance of progressive cerebellar ataxia and were found to have hypogonadotropic hypogonadism. This unique family displays clinical evidence for the presence of a possible common mechanism responsible for progressive hypothalamic and cerebellar impairment of late onset.

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