Sarcoidosis of the nervous system: a rare but serious manifestation

Clinically overt granulomatous involvement of the nervous system, or neurosarcoidosis, occurs in up to 5% of patients with sarcoidosis. Diagnosing neurosarcoidosis is often challenging due to its highly heterogeneous and frequently nonspecific clinical presentations, as well as the difficulty in obtaining tissue confirmation. In practice, the diagnosis of neurosarcoidosis is typically made based on supportive findings from ancillary tests - such as magnetic resonance imaging and cerebrospinal fluid analysis - in conjunction with the exclusion of alternative diagnoses in patients with known extraneural sarcoidosis. Current treatment recommendations are largely based on expert opinion and retrospective studies. Several factors should guide the initial treatment strategy, including disease extent, severity, functional impairment, comorbidities, and patient preferences. Glucocorticoids remain the cornerstone of therapy, with steroid-sparing agents and biologics frequently required in more severe cases.