Isolated neurosarcoidosis--a diagnostic enigma: case report and discussion

Neurosarcoidosis is a rare, but well-recognized cause of hypopituitarism with a predilection for the hypothalamus. We describe a case of panhypopituitarism in a 57-yr-old Asian lady, associated with an infiltrating hypothalamo-hypophyseal lesion, and other intracranial deposits, initially diagnosed as cerebral tuberculomata. Despite antituberculous therapy, the intracranial lesions progressed with significant clinical deterioration. Repeated lumbar puncture, magnetic resonance imaging scans, liver biopsy and Gallium scan were noncontributory, and the diagnosis of isolated neurosarcoidosis was established only following biopsy of an intracranial lesion. The lesion regressed on steroid and azathioprine therapy. Isolated neurosarcoidosis poses a considerable management problem. We review recent advances in the investigation, diagnosis, and treatment of this condition.