AMPA Receptor Encephalitis: A Decade Long Unveiling of Clinical, Radiographic and Serological Findings in an Untreated Patient

had non-hypothalamic-pituitary cranial neuroinflammation.Elevated serum ACE and CSF WBC were present in 5/11 and 10/11 patients, respectively.After a median follow-up of 66 months, the median EDSS was 6.25, all patients with HPA dysfunction (12/14) remained on hormone replacement and/or glucocorticoids, 11/14 patients relapsed, and a median of 2 (range 0-5) steroid-sparing immunosuppressants were utilized. ConclusionsHypothalamic-pituitary neurosarcoidosis presents chronically in association with systemic and non-hypothalamic-pituitary neuroinflammation.Its disease course is characterized by accrual of significant disability with high risk for relapse, and almost all patients require longterm hormone replacement therapy and glucocorticoid treatment.Study Supported By: N/A.