Cerebellar Ataxia And Hypergonadotropic Hypogonadism

Amor et al. (2001) described 2 sisters with onset of progressive cerebellar ataxia at the age of 16 and 32 years, respectively, and secondary amenorrhea due to hypergonadotropic hypogonadism. Sensorineural deafness with vestibular hypofunction and peripheral sensory impairment were also present. Intellect was normal. The authors referred to reports that may represent the same disorder, e.g., that of Skre et al. (1976). Cerebellar ataxia and hypogonadotropic hypogonadism is discussed elsewhere; see 212840.