Neurosarcoidosis: diagnosis and management

Semin Respir Crit Care Med. 2010 Aug;31(4):419-27. doi: 10.1055/s-0030-1262210. Epub 2010 Jul 27.

Abstract

Sarcoidosis is a multisystemic inflammatory granulomatous disease that affects both the central and peripheral nervous system. The neurological manifestations depend on the areas of the neuroaxis affected. In the brain, patients with neurosarcoidosis have leptomeningeal and intraparenchymal infiltration of granulomas that leads to, for example, cranial nerve palsies, basal meningitis, and endocrine dysfunction. It can cause peripheral neuropathies such as mononeuritis multiplex and sensorimotor polyneuropathy as well as radiculopathy and myopathy. Diagnosis and management of patients with neurosarcoidosis are challenging given that the gold standard is tissue-proven biopsy, which, in most cases of neurological illness, is difficult to obtain. Treatment strategies have not been rigorously evaluated but corticosteroids are considered the drug of choice. Other immunosuppressant agents such as cyclophosphamide, mycophenolate mofetil, and infliximab are efficacious in the treatment of neurosarcoidosis.

Publication types

  • Review

MeSH terms

  • Biopsy / methods
  • Brain / physiopathology
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / drug therapy*
  • Central Nervous System Diseases / physiopathology
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / drug therapy*
  • Peripheral Nervous System Diseases / physiopathology
  • Sarcoidosis / diagnosis
  • Sarcoidosis / drug therapy*
  • Sarcoidosis / physiopathology

Substances

  • Glucocorticoids
  • Immunosuppressive Agents