@article{c8a0d9237ab940f49887b1be7e8a9e9a,
title = "Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group",
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..",
author = "Stern, {Barney J.} and Walter Royal and Gelfand, {Jeffrey M.} and Clifford, {David B.} and Jinny Tavee and Siddharama Pawate and Berger, {Joseph R.} and Aksamit, {Allen J.} and Allan Krumholz and Pardo, {Carlos A.} and Moller, {David R.} and Judson, {Marc A.} and Marjolein Drent and Baughman, {Robert P.}",
note = "Funding Information: honoraria for lecturing on neurosarcoidosis, and serving as an expert legal witness for legal proceedings concerning neurosarcoidosis. Dr Royal reported receiving research support from the National Institutes of Health and the Veterans Administration as well as research and/or clinical education grant support from Mallinckrodt, Biogen-Idec, Alexion, EMD-Serono, and MedImmune, and serving as associate editor for the Journal of Neurovirology. Dr Gelfand reported receiving consulting fees from Biogen; research support (to the University of California, San Francisco) from Genentech, MedDay, and Quest Diagnostics; and personal compensation for medical-legal consulting as an expert witness. Dr Gelfand's spouse has received consulting fees from Zosano Pharma Corp, Eli Lilly and Company, and Biohaven Pharmaceutical; honoraria from UpToDate (for authorship) and JAMA Neurology (as an associate editor); consulting payments for work done through the University of California, San Francisco, Pediatric Headache Program from eNeura, Inc; and personal compensation for medical-legal consulting. Dr Clifford reported serving as a consultant, Data and Safety Monitoring Board member, and/or Progressive Multifocal Leukoencephalopathy (PML) adjudication committee member for Amgen, AstraZeneca, Bristol-Myers Squibb, Pfizer, Takeda, Biogen, Merck, Genentech/Roche, Glaxo Smith Kline, Seattle Genetics, Novartis, Protagonist, Dr Reddy, and Merck Serono. He reported being chair of the Data Safety and Monitoring Board for 2 clinical studies involving Huntington disease funded by Wave Life Sciences; he reported no disclosures related to sarcoidosis. Dr Tavee reported being involved in a clinical trial with Araim Pharmaceuticals and receiving speaking fees from Mallinkrodt. Dr Berger reported serving as a consultant and/or on the PML adjudication committees of Amgen, AstraZeneca, Bristol-Myers Squibb, Eisai, Janssen, Parexel, and Pfizer; serving on the Scientific Advisory Board of NeuVir and ExcisionBio; receiving honoraria from Prime Education and the Multiple Sclerosis Foundation for lectures; serving as an associate editor for the Journal of Neurovirology and as an editorial board member of ISRN Education journal, Journal of Neuroscience, World Journal of Rheumatology, and Multiple Sclerosis and Related Disorders journal; receiving publishing royalties for Handbook of Clinical Neurology, Vol 85; serving as a consultant to Alcimed, Amgen, AstraZeneca, Bayer, Biogen, Eisai, EMD Serono, Forward Pharmaceuticals, Genentech/Roche, Genzyme, Inhibikase, Millennium/Takeda, Novartis, Johnson and Johnson, Pfizer, and Sanofi Aventis; receiving research support from Biogen; and participating in legal proceedings for Biogen. Dr Moller reported being chairman and chief technical officer of Sarcoidosis Diagnostic Testing, LLC; receiving funding, including past salary support under the National Heart, Lung, and Blood Institute (NHLBI) Small Business Technology Transfer program (grant R41 HL129728); receiving research support from Endocyte; being a consultant to Merck and a past consultant to Novartis and Dicerna; serving on the Scientific Advisory Board of the Foundation for Sarcoidosis Research; receiving royalties from Hodder Education for editing a book on sarcoidosis; expecting future royalties from Taylor & Francis Group for editing a book on interstitial lung disease; receiving research support, including salary support from NHLBI (grant U01HL112708 [GRADS]); receiving honoraria for lecturing on sarcoidosis; and serving as an expert legal consultant/witness for legal proceedings concerning sarcoidosis. Dr Judson reported serving as a consultant to Biogen and receiving grant support (to his institution) from Novartis and Malinckrodt. Dr Baughman reported serving on the Scientific Advisory Board of the Foundation for Sarcoidosis Research; being a principal investigator on studies of clinical trials of nonneurosarcodiosis studies funded by NHLBI, Mallinkrodt, Celgene, Bayer, Gilead, and Novartis; and being a principal investigator of a registry of advanced sarcoidosis, including neurosarcoidosis. Dr Pawate reported attending an advisory board meeting for Biogen regarding a matter unrelated to sarcoidosis. No other disclosures were reported. Drs Clifford and Stern reported providing modest support, derived from discretionary research funds, for the meetings of the Neurosarcoidosis Consortium Consensus Group. Publisher Copyright: {\textcopyright} 2018 2018 American Medical Association. All rights reserved.",
year = "2018",
month = dec,
doi = "10.1001/jamaneurol.2018.2295",
language = "English (US)",
volume = "75",
pages = "1546--1553",
journal = "JAMA Neurology",
issn = "2168-6149",
publisher = "American Medical Association",
number = "12",
}