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Special Communication
December 2018

Definition and Consensus Diagnostic Criteria for NeurosarcoidosisFrom the Neurosarcoidosis Consortium Consensus Group

Author Affiliations
  • 1Department of Neurology, Johns Hopkins University, Baltimore, Maryland
  • 2Department of Neurobiology and the Neuroscience Institute, Morehouse School of Medicine, Atlanta, Georgia
  • 3Department of Neurology, University of California, San Francisco, San Francisco
  • 4Department of Neurology, Washington University in St Louis, St Louis, Missouri
  • 5Department of Neurology, Northwestern University, Chicago, Illinois
  • 6Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee
  • 7Department of Neurology, University of Pennsylvania, Philadelphia
  • 8Department of Neurology, Mayo Clinic, Rochester, Minnesota
  • 9Department of Neurology, University of Maryland, Baltimore
  • 10Department of Medicine, Johns Hopkins University, Baltimore, Maryland
  • 11Department of Medicine, Albany Medical College, Albany, New York
  • 12Department of Pharmacology and Toxicology, Faculty of Health Medicine and Life Sciences, Maastricht University, Maastricht, Interstitial Lung Disease Center of Excellence, St. Antonius Hospital, Nieuwegein, the Netherlands
  • 13Department of Medicine, University of Cincinnati, Cincinnati, Ohio
JAMA Neurol. 2018;75(12):1546-1553. doi:10.1001/jamaneurol.2018.2295
Abstract

Importance  The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease.

Observations  The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis.

Conclusions and Relevance  Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.

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