Sarcoidosis has developed an almost legendary mystique among neurologists that is rooted in our limited understanding of the disease. The unknowns are daunting: the precise etiology of sarcoidosis remains unresolved, the factors that drive neurologic organ system involvement remain unclear, the diagnosis can be challenging to confirm in the nervous system, and the potential clinical manifestations of neurosarcoidosis (NS) are often alleged to be protean. Neurosarcoidosis is commonly considered in the differential diagnosis of patients with unexplained neurologic symptoms and clinical deficits, so much so that a tired aphorism is that no differential diagnosis is complete without consideration of sarcoidosis. This situation is not without consequence. Among neuroinflammatory disorders, diagnostic workups for NS account for a disproportionately large share of total inpatient costs,1 and tremendous variability in clinical practice exists regarding application of various proposed diagnostic criteria.2,3 Particularly in the absence of histopathologic findings, this can lead in some cases to premature or unwarranted diagnosis of NS.
Gelfand JM. Demystifying Neurosarcoidosis and Informing Prognosis. JAMA Neurol. 2017;74(11):1296–1298. doi:10.1001/jamaneurol.2017.1473
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