Neurosarcoidosis occurs in 5% of patients with sarcoidosis and can be difficult to diagnose. In this review we discuss the most recent advances in our understanding of the disease, describing clinical characteristics, diagnostic process, treatment, and prognosis.
Clinical presentation is heterogeneous with most patients presenting with cranial nerve palsy, headache, or sensory abnormalities. Patients are classified according to probability of the diagnosis with the Zajicek criteria. In these criteria, histopathological confirmation of noncaseating granulomas in affected tissue outside the nervous system is key. Radiological abnormalities on neuroimaging are nonspecific. No biomarkers have been described that adequately identify patients with sarcoidosis. However, soluble interleukin-2 receptor is a relatively novel biomarker that may be useful. In addition to HRCT scan, 18F-FDG PET-CT scanning can identify occult locations of disease activity and aid in obtaining pathological confirmation. Despite the use of new therapies, still a third of patients remains stable, deteriorate, or die.
Diagnosing and treating patients with neurosarcoidosis remains a challenge. Long-term prospective studies evaluating patients suspected of neurosarcoidosis are needed to assess sensitivity and specificity of ancillary investigations and diagnostic criteria. Furthermore, future studies are needed to evaluate the prognosis and the optimal treatment strategy.
aDepartment of Neurology, Academic Medical Centre, Centre of Infection and Immunity Amsterdam (CINIMA), Amsterdam
bDepartment of Pulmonology, ILD Centre of Excellence, St. Antonius Hospital, Nieuwegein
cDivision of Heart & Lungs, Department of Pulmonology, University Medical Centre, Utrecht
dDepartment of Pharmacology and Toxicology, FHML, University Maastricht, Maastricht, The Netherlands
Correspondence to Matthijs C. Brouwer, Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands. Tel: +31205664042; fax: +0205669374 e-mail: email@example.com