Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis

Objectives: We retrospectively reviewed 5 patients with neurosarcoidosis, w ho all presented with central diabetes insipidus and hypogonadism. Design: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. Methods: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluat ion and in response to immunosuppressive therapy. Result: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 y ears. One patient showed deterioration with corticosteroids alone but parti al remission after additional cyclophosphamide. Pituitary dysfunction remai ned unchanged in all patients, despite total clinical and radiological remi ssion in two patients. However, one of these patients died of acute granulo matous meningoencephalitis after two years of follow-up. Conclusion: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiol ogical features is suggestive of neurosarcoidosis. However, there is an inc reasing number of case reports on lymphocytic hypophysitis. Without the bio ptic diagnosis, the differentiation between potentially lethal isolated neu rosarcoidosis and lymphocytic hypophysitis is difficult. These cases demons trate the difficulties in diagnosing neurosarcoidosis and reflect experienc es with follow-up parameters.