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

Abstract

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.

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

“…Meninges of the skull base, hypothalamus and pituitary gland are the most common sites [4][5][6][7][8][9][10][11][12] . The most common neurological symptoms are cranial nerve deficits, headache and seizures 2,7,[10][11][12][13][14][15][16][17] .…”

Diabetes Insipidus from Neurosarcoidosis: Long-term Follow-up for More than Eight Years

“…In patients diagnosed with systemic sarcoidosis and suspected to have intracranial sarcoid manifestation, other neurological disorders should be excluded such as autoimmune inflammatory diseases (e.g. multiple sclerosis, lymphocytic hypophysitis, systemic lupus erythematosus), infectious diseases (i.e., tuberculosis, fungal and bacterial infections) and neoplasms (i.e., lymphoma, meningioma or glioma) (11). However, in the case of isolated neurosarcoidosis in the absence of any systemic involvement, the diagnosis maybe difficult as in our case number 4.…”

Primary non-necrotizing granulomatous hypophysitis mimicking pituitary adenomas

“…Symptoms such as headache, visual disruption and involvement of pituitary gland and stalk, which may result in endocrine dysfunction, usually occur due to the influence of a sellar mass lesion (5).…”

“…due to systemic inflammatory disorders (5,7,12). Primary hypophysitis is classified into five sub-types: lymphocytic, granulamatous, xanthomatous, xanthogranulomatous and necrotizing (38).…”

“…Inflammatory hypophysitis is classified into five sub-types: lymphocytic, granulamatous, xanthomatous, xanthogranulomatous and necrotizing (21). Lymphocytic and granulomatous hypophysitis are the most common types (5). Hypophysitis can also be classified as primary (idiopathic) hypophysitis and secondary hypophysitis.…”