Hypergonadotropic hypogonadism and cerebellar ataxia: an unusual association.

Federal University of São Paulo (UNIFESP), São Paulo SP, Brazil: Department of Neurology and Neurosurgery; Department of Endocrinology; Department of Ophthalmology. The association between cerebellar ataxia and hypogonadism was first described by Gordon Holmes in 1907. It represents a highly heterogenous syndrome with insidious onset. The hypogonadism of most patients with Gordon Holmes syndrome is hypogonadotropic, with a defect in the production or release of gonadotropins by pituitary glands. In contrast, some patients with Holmes type ataxia may have hypergonadotropic hypogonadism, which represents a failure at the level of the gonads rather than at the level of the pituitary.