Many faces of neurosarcoidosis: from chronic meningitis to myelopathy

Daan Fritz, Mareye Voortman, Diederik van de Beek, Marjolein Drent, Matthijs C. Brouwer*

*Corresponding author for this work

Research output: Contribution to journal(Systematic) Review article peer-review

Abstract

Purpose of review

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.

Recent findings

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, F-18-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.

Summary

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.

Original languageEnglish
Pages (from-to)439-446
Number of pages8
JournalCurrent Opinion in Pulmonary Medicine
Volume23
Issue number5
DOIs
Publication statusPublished - Sept 2017

Keywords

  • autoimmune disorders
  • clinical neurology
  • granulomatous inflammation
  • neurosarcoidosis
  • STEROID-REFRACTORY NEUROSARCOIDOSIS
  • ANGIOTENSIN-CONVERTING ENZYME
  • NERVOUS-SYSTEM SARCOIDOSIS
  • ANTI-TNF THERAPY
  • IGG4-RELATED DISEASE
  • PULMONARY SARCOIDOSIS
  • DRAMATIC RESPONSE
  • INFLIXIMAB
  • MANAGEMENT
  • DIAGNOSIS

Cite this