Characteristics and Long-Term Outcome of Neurosarcoidosis: A Population-Based Study from 1976-2013

Abstract

Background/Aims: Neurosarcoidosis is a rare condition with serious health consequences. However, little is known about clinical characteristics and outcome of neurosarcoidosis in the community setting. Methods: Patients with neurosarcoidosis were identified from a previously described cohort of patients with incident sarcoidosis from Olmsted County, Minnesota, United States from 1976 to 2013 using individual medical record review. Data on clinical characteristics, treatment, and outcome were collected. Results: Neurological involvement by sarcoidosis occurred in 11 patients (3% of all patients with sarcoidosis). Cranial neuropathy was the most common type of neurological disease (5 patients; 45%) followed by peripheral neuropathy (3 patients; 27%), and meningitis (3 patients; 27%). Cerebrospinal fluid (CSF) pleocytosis and elevated CSF protein levels were observed in patients with meningitis, intramedullary spinal cord sarcoidosis, intracranial mass lesion and some patients with cranial neuropathy but were normal in patients with peripheral neuropathy. All patients received high-dose glucocorticoids as initial treatment and almost all responded to this therapy. Relapse after glucocorticoid dose reduction necessitated subsequent treatment with steroid-sparing agents in 4 patients. Conclusion: Neurosarcoidosis is an uncommon manifestation of sarcoidosis. Neurosarcoidosis manifestations generally responded well to high-dose glucocorticoids in the majority of patients, but relapse was common.