Eur J Endocrinol
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DOI: 10.1530/eje.0.1420365
European Journal of Endocrinology, Vol 142, Issue 4, 365-372
Copyright © 2000 by European Society of Endocrinology
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Case Reports

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

C Bullmann, M Faust, A Hoffmann, C Heppner, F Jockenhovel, D Muller-Wieland, and W Krone

Klinik II und Poliklinik fur Innere Medizin der Universitat zu Koln, Cologne, Germany. ai005@uni-koeln.de

OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who 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 evaluation 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 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous 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 radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters.


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