MULTIPLE PITUITARY-HORMONE DEFICIENCIES IN A PATIENT WITH SPINOCEREBELLAR ATAXIA - MAGNETIC-RESONANCE-IMAGING AND HORMONAL STUDIES

Degenerative spinocerebellar ataxia has a rare association with hypogo nadotropic hypogonadism. In this report we present the results of the detailed endocrine evaluation and magnetic resonance imaging in one su ch patient. A 20-year-old male with progressive cerebellar ataxia, hyp ogonadism, and short stature was investigated. Basal testing revealed hypogonadotropic hypogonadism (LH <5 mU/L, FSH <5 mU/L, testosterone 2 .5 nM/L). There was no rise in LH after stimulation with LHRH, peak LH level being <5 mU/L. Insulin hypoglycemia testing was consistent with GH deficiency, with peak GH being 3.2 mU/L. On TRH stimulation, there was no significant rise in prolactin, though the TSH response was nor mal. Magnetic resonance imaging revealed cerebellar atrophy. The anter ior pituitary was atrophic, with a height of 1.4 mm. The posterior pit uitary and the pituitary stalk were normal in size and position. This patient with degenerative spinocerebellar ataxia had multiple pituitar y hormone deficiencies. The results of our endocrine evaluation and MR imaging lead us to believe that these deficits may result from a lesi on at the level of the pituitary gland.