Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis.
OBJECTIVE
We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Bullmann
C
C
Klinik II und Poliklinik für Innere Medizin der Universität zu Köln, Cologne, Germany. ai005@uni-koeln.de
Faust
M
M
Hoffmann
A
A
Heppner
C
C
Jockenhövel
F
F
Müller-Wieland
D
D
Krone
W
W
D000328:Adult; D001927:Brain Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D020790:Diabetes Insipidus, Neurogenic / Q000209:etiology*; D005260:Female; D006801:Humans; D007006:Hypogonadism / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D007166:Immunosuppressive Agents; D011241:Prednisone